craniovertebral junction

92
Craniovertebral Craniovertebral junction junction Introduction Introduction Embryology Embryology Anatomy Anatomy Radiology Radiology Anomalies of cvj. Anomalies of cvj.

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Page 1: Craniovertebral junction

Craniovertebral junction Craniovertebral junction Introduction Introduction EmbryologyEmbryology

AnatomyAnatomyRadiologyRadiology

Anomalies of cvjAnomalies of cvj

Cvj collective term that refers to Cvj collective term that refers to occiputatlasaxisamp supporting occiputatlasaxisamp supporting ligamentsligaments

Its transition between mobile Its transition between mobile cranium amp rigid spinal columncranium amp rigid spinal column

It encloses soft tissue structures It encloses soft tissue structures of the cervicomedullary of the cervicomedullary jn(medulla spinal cordamp lower jn(medulla spinal cordamp lower cranial nerves) cranial nerves)

Embryology and development Embryology and development of cvjof cvj

Development of the cartilaginous cranium amp the adjacent structures begins during the early weeks of intrauterine life

2ndGestational week Mesoderm cells condense in the midline to form notochordalprocess

3rdGestational week

-notochordalprocess invaginatesin bw ectoamp endoderm to form notochord

-dorsal ectoderm thickens to form neural groove which folds fuses amp becomes neural tube

Eampd contdhellipEampd contdhellip

Between 3rdamp 5thweek

-part of mesoderm which lies on either side of notochord (Paraxial mesoderm) gives rise to somites(Segmentation)

-total 42 somitesform at 4thweek -ventromedialportion of somiteis ka

sclerotomewhich forms the vertebral bodies -each sclerotomedifferentiates into a

cranial loosely arranged portion and a caudal compact portion by a fissure ka ldquoFissure

Eampd contdhellipEampd contdhellip

Mesenchymalcells of the fissure condense around the notochord to form the intervertebraldisc

Notochord disappears at the vertebral bodies but persist asnucleus pulposusat disc

The first four sclerotomesdo not follow this course amp fuse to form the occipital bone amp postportionof FM

This membraneousstage is fbstages of chondrificationamp ossification

Out of 4 occipital sclerotomesthe first 2 form basiocciput the III Jugular tuberclesand the IV (Proatlas) form parts of foramen magnum atlas and

Eampd contdhellipEampd contdhellip

PROATLAS divided into the hypocentrum centrumamp the neural arches

-hypocentrumforms the vestigial condylustertiusor anterior tubercle of the clivus

-centrumforms the apex of the dens also forms the apical ligament (AL) of dens (AL may contain notochordaltissuesoka rudimentary IVdisc)

-ventral part of the neural arch forms the ant margin of FM 2 occipital condylesamp the alaramp cruciateligaments

-dorsal part forms paired rostralarticularfacets lateral masses of C1 amp superior portion of the post arch of the atlas

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 2: Craniovertebral junction

Cvj collective term that refers to Cvj collective term that refers to occiputatlasaxisamp supporting occiputatlasaxisamp supporting ligamentsligaments

Its transition between mobile Its transition between mobile cranium amp rigid spinal columncranium amp rigid spinal column

It encloses soft tissue structures It encloses soft tissue structures of the cervicomedullary of the cervicomedullary jn(medulla spinal cordamp lower jn(medulla spinal cordamp lower cranial nerves) cranial nerves)

Embryology and development Embryology and development of cvjof cvj

Development of the cartilaginous cranium amp the adjacent structures begins during the early weeks of intrauterine life

2ndGestational week Mesoderm cells condense in the midline to form notochordalprocess

3rdGestational week

-notochordalprocess invaginatesin bw ectoamp endoderm to form notochord

-dorsal ectoderm thickens to form neural groove which folds fuses amp becomes neural tube

Eampd contdhellipEampd contdhellip

Between 3rdamp 5thweek

-part of mesoderm which lies on either side of notochord (Paraxial mesoderm) gives rise to somites(Segmentation)

-total 42 somitesform at 4thweek -ventromedialportion of somiteis ka

sclerotomewhich forms the vertebral bodies -each sclerotomedifferentiates into a

cranial loosely arranged portion and a caudal compact portion by a fissure ka ldquoFissure

Eampd contdhellipEampd contdhellip

Mesenchymalcells of the fissure condense around the notochord to form the intervertebraldisc

Notochord disappears at the vertebral bodies but persist asnucleus pulposusat disc

The first four sclerotomesdo not follow this course amp fuse to form the occipital bone amp postportionof FM

This membraneousstage is fbstages of chondrificationamp ossification

Out of 4 occipital sclerotomesthe first 2 form basiocciput the III Jugular tuberclesand the IV (Proatlas) form parts of foramen magnum atlas and

Eampd contdhellipEampd contdhellip

PROATLAS divided into the hypocentrum centrumamp the neural arches

-hypocentrumforms the vestigial condylustertiusor anterior tubercle of the clivus

-centrumforms the apex of the dens also forms the apical ligament (AL) of dens (AL may contain notochordaltissuesoka rudimentary IVdisc)

-ventral part of the neural arch forms the ant margin of FM 2 occipital condylesamp the alaramp cruciateligaments

-dorsal part forms paired rostralarticularfacets lateral masses of C1 amp superior portion of the post arch of the atlas

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 3: Craniovertebral junction

Embryology and development Embryology and development of cvjof cvj

Development of the cartilaginous cranium amp the adjacent structures begins during the early weeks of intrauterine life

2ndGestational week Mesoderm cells condense in the midline to form notochordalprocess

3rdGestational week

-notochordalprocess invaginatesin bw ectoamp endoderm to form notochord

-dorsal ectoderm thickens to form neural groove which folds fuses amp becomes neural tube

Eampd contdhellipEampd contdhellip

Between 3rdamp 5thweek

-part of mesoderm which lies on either side of notochord (Paraxial mesoderm) gives rise to somites(Segmentation)

-total 42 somitesform at 4thweek -ventromedialportion of somiteis ka

sclerotomewhich forms the vertebral bodies -each sclerotomedifferentiates into a

cranial loosely arranged portion and a caudal compact portion by a fissure ka ldquoFissure

Eampd contdhellipEampd contdhellip

Mesenchymalcells of the fissure condense around the notochord to form the intervertebraldisc

Notochord disappears at the vertebral bodies but persist asnucleus pulposusat disc

The first four sclerotomesdo not follow this course amp fuse to form the occipital bone amp postportionof FM

This membraneousstage is fbstages of chondrificationamp ossification

Out of 4 occipital sclerotomesthe first 2 form basiocciput the III Jugular tuberclesand the IV (Proatlas) form parts of foramen magnum atlas and

Eampd contdhellipEampd contdhellip

PROATLAS divided into the hypocentrum centrumamp the neural arches

-hypocentrumforms the vestigial condylustertiusor anterior tubercle of the clivus

-centrumforms the apex of the dens also forms the apical ligament (AL) of dens (AL may contain notochordaltissuesoka rudimentary IVdisc)

-ventral part of the neural arch forms the ant margin of FM 2 occipital condylesamp the alaramp cruciateligaments

-dorsal part forms paired rostralarticularfacets lateral masses of C1 amp superior portion of the post arch of the atlas

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 4: Craniovertebral junction

Eampd contdhellipEampd contdhellip

Between 3rdamp 5thweek

-part of mesoderm which lies on either side of notochord (Paraxial mesoderm) gives rise to somites(Segmentation)

-total 42 somitesform at 4thweek -ventromedialportion of somiteis ka

sclerotomewhich forms the vertebral bodies -each sclerotomedifferentiates into a

cranial loosely arranged portion and a caudal compact portion by a fissure ka ldquoFissure

Eampd contdhellipEampd contdhellip

Mesenchymalcells of the fissure condense around the notochord to form the intervertebraldisc

Notochord disappears at the vertebral bodies but persist asnucleus pulposusat disc

The first four sclerotomesdo not follow this course amp fuse to form the occipital bone amp postportionof FM

This membraneousstage is fbstages of chondrificationamp ossification

Out of 4 occipital sclerotomesthe first 2 form basiocciput the III Jugular tuberclesand the IV (Proatlas) form parts of foramen magnum atlas and

Eampd contdhellipEampd contdhellip

PROATLAS divided into the hypocentrum centrumamp the neural arches

-hypocentrumforms the vestigial condylustertiusor anterior tubercle of the clivus

-centrumforms the apex of the dens also forms the apical ligament (AL) of dens (AL may contain notochordaltissuesoka rudimentary IVdisc)

-ventral part of the neural arch forms the ant margin of FM 2 occipital condylesamp the alaramp cruciateligaments

-dorsal part forms paired rostralarticularfacets lateral masses of C1 amp superior portion of the post arch of the atlas

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 5: Craniovertebral junction

Eampd contdhellipEampd contdhellip

Mesenchymalcells of the fissure condense around the notochord to form the intervertebraldisc

Notochord disappears at the vertebral bodies but persist asnucleus pulposusat disc

The first four sclerotomesdo not follow this course amp fuse to form the occipital bone amp postportionof FM

This membraneousstage is fbstages of chondrificationamp ossification

Out of 4 occipital sclerotomesthe first 2 form basiocciput the III Jugular tuberclesand the IV (Proatlas) form parts of foramen magnum atlas and

Eampd contdhellipEampd contdhellip

PROATLAS divided into the hypocentrum centrumamp the neural arches

-hypocentrumforms the vestigial condylustertiusor anterior tubercle of the clivus

-centrumforms the apex of the dens also forms the apical ligament (AL) of dens (AL may contain notochordaltissuesoka rudimentary IVdisc)

-ventral part of the neural arch forms the ant margin of FM 2 occipital condylesamp the alaramp cruciateligaments

-dorsal part forms paired rostralarticularfacets lateral masses of C1 amp superior portion of the post arch of the atlas

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 6: Craniovertebral junction

Eampd contdhellipEampd contdhellip

PROATLAS divided into the hypocentrum centrumamp the neural arches

-hypocentrumforms the vestigial condylustertiusor anterior tubercle of the clivus

-centrumforms the apex of the dens also forms the apical ligament (AL) of dens (AL may contain notochordaltissuesoka rudimentary IVdisc)

-ventral part of the neural arch forms the ant margin of FM 2 occipital condylesamp the alaramp cruciateligaments

-dorsal part forms paired rostralarticularfacets lateral masses of C1 amp superior portion of the post arch of the atlas

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 7: Craniovertebral junction

Eampd contdhellipEampd contdhellip

ATLAS no vertebral body amp no IV disc

-major portion formed by first spinal sclerotome -trasitionalvertebra as centrumof sclerotomeis

separated to fuse with the axis body forming the odontoidprocess

-hypocentrumof 1stspinal sclerotomeforms the anterior arch of the atlas

-neural arch of the first spinal sclerotomeforms the inferior portion of the posterior arch of atlas

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 8: Craniovertebral junction

Eampd contdhellipEampd contdhellip

AXIS develops from 2ndspinal sclerotome

-hypocentrumof 2ndspinal sclerotomedisappears during embryogenesis

-centrumforms the body of the axis vertebra amp neural arch develops into the facets amp the posterior arch of the axis

-At birth odontoidbase is separate from the body of axis by a cartilage which persists until the age of 8 later the center gets ossifiedormay remain separate asOs-odontoidium

-The apical segment is not ossified until 3 years of age at 12 years if fuses with odontoidto form normal odontoid failure leads to Os termin

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 9: Craniovertebral junction

AnatomyAnatomy

Articular Articular LigamentousLigamentous MusclesMuscles NeuralNeural LymphaticsLymphatics ArterialArterial venousvenous

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 10: Craniovertebral junction

articulararticular

Upper surfaces of C1 lateral masses are cup-like or concave which fit into the ball amp socket configuration united by articularcapsules surr the AO joint amp by the ant amp post AO membranes

10487084 synovialjoints bw atlas amp axis ndash

2 median ndashfront amp back of dens (Pivot variety) 2 lateral ndashbw opposing articularfacets(Planevariety)

1048708Each joint has its own capsule amp synovialcavity

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 11: Craniovertebral junction

LigamentousLigamentous

atlanto-occipital ligatlanto-occipital lig anterior a-o membraneanterior a-o membrane Posterior a-o membranePosterior a-o membrane Lateral a-o ligLateral a-o ligAtlanto axial ligAtlanto axial lig Ant a-a ligAnt a-a lig Post a-a ligPost a-a lig Transverse lig of atlasTransverse lig of atlas

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 12: Craniovertebral junction

Axis-occipital ligAxis-occipital lig Tectorial membTectorial memb Alar ligAlar lig Apical ligApical lig

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 13: Craniovertebral junction

musclesmuscles

Have minor role related to Have minor role related to stabilizationamp do not limit stabilizationamp do not limit movements movements

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 14: Craniovertebral junction

neuralneural

Neural structures related to cvj Neural structures related to cvj caudal portion of brainstcaudal portion of brainstem Cerebellum 4th ventricle amprostral part of

spinal cord Upper cranial amplower cervical

nerves

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 15: Craniovertebral junction

Neural contdhellipNeural contdhellip

Lower 4 cn are closely related to cvjLower 4 cn are closely related to cvj 9amp109amp10thth cn arise from medulla(ion)ampinf cn arise from medulla(ion)ampinf

cerebellar peduncle seperated by cerebellar peduncle seperated by dural seathdural seath

Acessory n is only nerve that passes Acessory n is only nerve that passes through foramen magnum has two through foramen magnum has two parts ramus internus amp ramus parts ramus internus amp ramus externusexternus

The C1 C2 and C3 nerves distal to the ganglion divide into dorsal and ventral rami

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 16: Craniovertebral junction

lymphaticslymphatics

The lymphatic drainage of the O-A-A joints is primarily into the retropharyngeal LN amp then into the deep cervical chain

These LNrsquosalso drain the nasopharynxamp hence retrograde infection may affect the synoviallining of the CVJ complex with resultant neck stiffness amp instability

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 17: Craniovertebral junction

vascularvascular

The major arteries related to CVJ are vertebral posteroinferiorcerebellararteries (PICA) and the meningealbranches of the vertebral and external and internal carotid arteries

The venous structures in the region of the FM are divided into three groups

-Extraduralveins(extraspinalamp intraspinalpart) -Intradural(neural) veins amp

-Duralvenous sinuses( superior petrosal marginal amp occipital)

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 18: Craniovertebral junction

kineticskinetics

3 major articulations determine stability and 3 major articulations determine stability and movements at the skull basemovements at the skull base

Nodding or ldquoyes-yesrdquo movements occur at Nodding or ldquoyes-yesrdquo movements occur at atlanto occipital joints atlanto occipital joints

Rotary or no ndashno movements occur at Rotary or no ndashno movements occur at atlanto-axial joints Both atlanto occipital atlanto-axial joints Both atlanto occipital and atlanto axial articulations are involved and atlanto axial articulations are involved in flexion motion Flexion at occipito atlantal in flexion motion Flexion at occipito atlantal joint is 13-15joint is 13-1500 and an additional 10 and an additional 1000 of of motion occurs at atlas articulationmotion occurs at atlas articulation

Rotation of the craniovertebral complex Rotation of the craniovertebral complex occurs only at atlas ndash axis jointoccurs only at atlas ndash axis joint

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 19: Craniovertebral junction

Kinetics contdhellipKinetics contdhellip

The articular surfaces of atlanto- axial The articular surfaces of atlanto- axial complex are convex with horizontal complex are convex with horizontal orientationorientation

Allows maximum mobility at the cost of Allows maximum mobility at the cost of stability Thus the stabilizing effect of stability Thus the stabilizing effect of upper is essential upper is essential

Cervical musculature not fully developed Cervical musculature not fully developed until age 8 allowing for abnormal until age 8 allowing for abnormal atlanto- axial rotations atlanto- axial rotations

Atlanto-axial rotation is maximum at 45Atlanto-axial rotation is maximum at 4500 Beyond this an interlocking of the lateral Beyond this an interlocking of the lateral mass of the atlas over the superior mass of the atlas over the superior surface of the axis vertebra occurs surface of the axis vertebra occurs

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 20: Craniovertebral junction

Kinetics contdhellipKinetics contdhellip

Lateral rotation of neck possible is Lateral rotation of neck possible is 909000 half of which occurs above C2 half of which occurs above C2 level and the remainder in lower level and the remainder in lower cervical spine cervical spine

Odontoid process is of central Odontoid process is of central importance in the radiology of importance in the radiology of craniovertebral lesions Atlanto- axial craniovertebral lesions Atlanto- axial joints allow less flexion-extension joints allow less flexion-extension motion than rotationmotion than rotation

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 21: Craniovertebral junction

Radiological evaluation of cvjRadiological evaluation of cvj

Conventional digital Conventional digital radiography radiography

Lateral viewLateral view Translateral view of skull Translateral view of skull

including cervical spine with full including cervical spine with full flexion and extension flexion and extension

Open mouth viewOpen mouth view Conventional tomographyConventional tomography

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 22: Craniovertebral junction

Ct(ncctamp3dct)Ct(ncctamp3dct) Mriampdynamic mriMriampdynamic mri Dynamic MRI uses sagittal Dynamic MRI uses sagittal

sections in active flexion and sections in active flexion and extension- better evaluation of extension- better evaluation of AAD and ligamental changes in AAD and ligamental changes in AAD AAD

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 23: Craniovertebral junction

craniometrycraniometry

Craniometryof the CVJ uses a series of lines planes amp angles to define the normal anatomic relationships of the CVJ

These measurements can be taken on plain Xrays3DCT or on MRI

No single measurement is helpful

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 24: Craniovertebral junction

LATERAL PROJECTION OF SKULL X-RAY

bull Palatondashoccipital (Chamberlainrsquos line) (gt5mm) bull Palatondashsuboccipitalline (McGregor line) ( gt 7mm) bull Foramen magnum line (McRae line) (Tip of dens below this line)

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 25: Craniovertebral junction

Chamberlanrsquos line(palato-Chamberlanrsquos line(palato-occipital)occipital)

Line joining posterior tip of hard palate to the posterior rim of foramen magnum Odontoid tip usually within 5 mm above this line Disadvantage - variability of posterior rim of foramen magnum

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 26: Craniovertebral junction

Mcgregors line(palato-Mcgregors line(palato-suboccipital line)suboccipital line)

Line joining lowest point of occipital bone to the posterior tip of hard palate Odontoid tip should be within 7 mm above this line It appears to be most accurate and reproducible

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 27: Craniovertebral junction

Wakenheimclival line(basilar)Wakenheimclival line(basilar)Line representing the downward prolongation of clivus along its posterior surface Dens should lie anterior and caudal to this tangent If clivus concave convex baseline by connecting a point just below the posterior clinoid process to basion Basilar line forms an angle with a line along the posterior surface of axis ranging from 150-1800 (in flexion and extension ) Canal compromise is present at angle lt 1500

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 28: Craniovertebral junction

Kalus index(ht of posterior Kalus index(ht of posterior cranial fossa)cranial fossa) Perpendicular line drawn from Perpendicular line drawn from

tip of odontoid process plane of tip of odontoid process plane of foramen magnum to Twinningrsquos foramen magnum to Twinningrsquos lineline

average - 35mm lt 30 mm is average - 35mm lt 30 mm is basilar invagination (BI) basilar invagination (BI)

Twinning line- joins internal Twinning line- joins internal occipital protuberance to occipital protuberance to tuberculum sellae)tuberculum sellae)

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 29: Craniovertebral junction

Twinningrsquos Point- Falls normally in Twinningrsquos Point- Falls normally in center of 4th ventricle Intersection center of 4th ventricle Intersection of Twinnings line amp Klausrsquo vertical is of Twinnings line amp Klausrsquo vertical is situated upto 10 mm anterior to the situated upto 10 mm anterior to the midpoint of Twinningrsquos linemidpoint of Twinningrsquos line

Klausrsquo index is concerned only with Klausrsquo index is concerned only with structures in midline of skull structures in midline of skull

Possesses specific value in the Possesses specific value in the diagnosis of basilar hypoplasia the diagnosis of basilar hypoplasia the main abnormality in basilar main abnormality in basilar invaginationinvagination

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 30: Craniovertebral junction

Mcray line(plane of foramen Mcray line(plane of foramen magnum)magnum)

line from basion to opisthion Normal 33 mm Symptomatic disease associated with value below 20 mm Stenosis widening of foramen magnum is a diagnosis that depends on the ratio of sagittal diagmeter of foramen magnum to that at the level of atlas

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 31: Craniovertebral junction

Basal angleBasal angle

Angle between the lines drawn from nasion to tuberculum sellae and tuberculum sellae to basion Normal 124 -140 any widening is called platybasia

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 32: Craniovertebral junction

Boogards line amp angleBoogards line amp angle

Boogardrsquos line connects nasion to Boogardrsquos line connects nasion to opisthion Boogardrsquos angle opisthion Boogardrsquos angle McRaersquos line drawn first Second McRaersquos line drawn first Second line drawn from dorsum sellae to line drawn from dorsum sellae to basion along the plane of clivus basion along the plane of clivus Angle formed between these 2 lines Angle formed between these 2 lines is measured Average 122is measured Average 12200 both both measurement will be altered in measurement will be altered in basilar impression- the basion will basilar impression- the basion will be above Boogardrsquos line and angle be above Boogardrsquos line and angle will be greater than 135will be greater than 13500

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 33: Craniovertebral junction

Atlanto temporomandibular Atlanto temporomandibular indexindex

(Normal 30 (Normal 30 ++ mm lt 10 mm mm lt 10 mm in BI in BI

Distance between superior Distance between superior edge of anterior arch of edge of anterior arch of atlas and the highest point atlas and the highest point of temporomandibular of temporomandibular interspace Regarded as interspace Regarded as one of the best criteria of one of the best criteria of BI since it defines the BI since it defines the position of foramen position of foramen magnum relative to a magnum relative to a lateral lying structure which lateral lying structure which is not involved in the is not involved in the invagination invagination

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 34: Craniovertebral junction

Spino lamellarlineSpino lamellarline

Line drawn from interoccipital Line drawn from interoccipital ridge above and down along the ridge above and down along the fused spinous process of C2 fused spinous process of C2 and C3 Normally it should and C3 Normally it should intersect posterior arch of atlas intersect posterior arch of atlas If atlas is fused post arch is If atlas is fused post arch is anterior to the line posterior anterior to the line posterior compression of spinal cord may compression of spinal cord may occur occur

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 35: Craniovertebral junction

Frontal projectionFrontal projection

Bidigastric line (FischgoldBidigastric line (Fischgold Line joining tips of mastoid Line joining tips of mastoid

processes and normally passes processes and normally passes through atlanto occipital interspace through atlanto occipital interspace Tip of odontoid will be with in 1 cm Tip of odontoid will be with in 1 cm above this line Disadvantage ndash above this line Disadvantage ndash Mastoid tips vary greatly in Mastoid tips vary greatly in development if mastoid tips are development if mastoid tips are well defined odontoid process is well defined odontoid process is blurred and vice versa blurred and vice versa

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 36: Craniovertebral junction

Condylar angle (Schmidt ndash Condylar angle (Schmidt ndash Fisher angleFisher angle angle formed by intersection of angle formed by intersection of

2 lines drawn of atlanto- 2 lines drawn of atlanto- condylar interspace Normal condylar interspace Normal 135135+ + 50 angle wider in condylar 50 angle wider in condylar hypoplasiahypoplasia

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 37: Craniovertebral junction

Classification of abnormalities Classification of abnormalities of of CVJCVJ Congenital Anomalies and Congenital Anomalies and

Malformations Malformations Malformations of occipital bone Malformations of occipital bone Manifestations of occipital vertebra Manifestations of occipital vertebra

Clivus segmentations Clivus segmentations Remnants around foramen magnum Remnants around foramen magnum Atlas variant Atlas variant

Basilar invagination Basilar invagination Condylar hypoplasia Condylar hypoplasia Assimilation of atlasAssimilation of atlas

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 38: Craniovertebral junction

IIII Malfomations of atlas Malfomations of atlas Assimilation Of Atlas Assimilation Of Atlas Atlanto axial fusion Atlanto axial fusion Aplasia of atlas archesAplasia of atlas arches

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 39: Craniovertebral junction

IIIIII Malfomations of axis Malfomations of axis Irregular atlantoaxial segmentation Irregular atlantoaxial segmentation Dens dysplasias Dens dysplasias Ossiculum terminale persistans Ossiculum terminale persistans Os Odontoideum Os Odontoideum Hypoplasia ndash Aplasia Hypoplasia ndash Aplasia Segmentaion failure of C2- C3Segmentaion failure of C2- C3

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 40: Craniovertebral junction

Abnormalities of foramen Abnormalities of foramen magnum magnum Secondary basilar invagination ndash Secondary basilar invagination ndash

eg Pagetrsquos disease eg Pagetrsquos disease Osteomalacia Rheumatoid Osteomalacia Rheumatoid cranial settling renal resistance cranial settling renal resistance rickets rickets

Foraminal stenosis ndash eg Foraminal stenosis ndash eg Achondroplasia Achondroplasia

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 41: Craniovertebral junction

Atlanto axial instability Atlanto axial instability Traumatic occipitoatlantal and atlanto Traumatic occipitoatlantal and atlanto

axial dislocations Os odontoideum axial dislocations Os odontoideum Downrsquos syndrome Downrsquos syndrome Infections ndash eg Griselrsquos syndromeInfections ndash eg Griselrsquos syndrome Inflammatory ndash eg rheumatoid arthritis Inflammatory ndash eg rheumatoid arthritis Tumors- eg Neurofibromatosis Tumors- eg Neurofibromatosis

Syringomyelia Syringomyelia Errors of metabolism ndash eg Morquios Errors of metabolism ndash eg Morquios

syndromesyndrome

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 42: Craniovertebral junction

Multiple anomalies frequently Multiple anomalies frequently seen Common combination seen Common combination were AAD with occipitalisation of were AAD with occipitalisation of atlas BI with chiari atlas BI with chiari malformation and BI with malformation and BI with occipitalisation of atlasoccipitalisation of atlas Miscellaneous ndash eg Fatal Miscellaneous ndash eg Fatal

Warfarin syndrome Convadi Warfarin syndrome Convadi syndromesyndrome

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 43: Craniovertebral junction

Anomalies of occiputAnomalies of occiput

Condylar hypoplasiaCondylar hypoplasia In condylar hypoplasia the occipital condyles are underdeveloped and have a flattened appearance leading to BI (violation of the Chamberlain line) and widening of the atlantooccipitaljoint axis angle

The lateral masses of the atlas may be fused to the hypoplasticcondyles further accentuating the BI

Condylarhypoplasialimits or may even abolish movements at the A-O joint

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 44: Craniovertebral junction

Kippel feil syndromeKippel feil syndrome

Due to failure of the normal segmentation of cervical somitesduring the third and eighth weeks of gestation

1048708Classic triad of low posterior hair line short neck and limited neck ROM found in less than 50 of cases

1048708The most consistent finding is limitation of neck motion

1048708Generally flexion and extension are better preserved than side-bending and rotation

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 45: Craniovertebral junction

Type 1Cervical spine fusion in which elements of many vertebrae are incorporated into a single block

Type 2Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantalfusion

Type 3Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 46: Craniovertebral junction

Scoliosis-Up to 60 have gt15 degrees curve Genito-urinary-up to 65Most common is absence

of kidney Sprengelsdeformity-approx 35 Cardio-pulmonary-5-15 most commonly VSD Deafness-30 all types MC mixed Sykinesis-Mirror motions have been described in up

to 20 of patients under the age of 5 Cranio-cervical abnormalities (25)-Includes C1-

C2 hypermobilityand instability BIChiariI malformation diastematomyelia amp syringomyelia

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 47: Craniovertebral junction

Anomalies of occiput contdhellipAnomalies of occiput contdhellip

Terms basilar invagination Terms basilar invagination basilar impression and basilar impression and platybasia are not synonymous platybasia are not synonymous and require definitionand require definition

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 48: Craniovertebral junction

Basilar invaginationBasilar invagination

Basilar invaginationBasilar invagination ndash is a ndash is a developmental defect implying prolapse developmental defect implying prolapse of vertebral column into the skull base It of vertebral column into the skull base It is frequently associated with such is frequently associated with such developmental bony anomalies of the developmental bony anomalies of the region as occipitalization of atlas region as occipitalization of atlas defective fusion of arch and block defective fusion of arch and block vertebral malformationsvertebral malformations

There are two types of basilar invagination primary invagination which is developmental and more common and secondary invagination which is acquired

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 49: Craniovertebral junction

Topographic types of BI Anterior BI hypoplasiaof the basilar

process of the occipital bone BI of the occipital

condyles(ParamedianBI)ndashCondylarhypoplasia

BI in the lateral condylararea Posterior BI posterior margin of the

FM is invaginated Unilateral BI GeneralisedBI

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 50: Craniovertebral junction

SIGNS SYMPTOMS usually occur in 2ndor 3rddecade Short neck(78)torticollis (68) ssof associated ACM (cerebellaramp vestibular

disturbances) amp syringomyelia(25 to 35) Motor amp sensory disturbances (85) Lower cranial nerves involvement Headache amp pain in the nape of neck (greater occipital N) ssof raised ICP (HCP) due to posterior encroachment

which causes blockage of aqueduct of sylvius Compression of cerebellum amp vestibular apparatus

leading to vertical or lateral nystagmus(65) (not due to direct pressure from post rim of FM but rather due to a thickened band of dura)

Vertebral artery insufficiency ss

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 51: Craniovertebral junction

Basilar impression(sec basillar Basilar impression(sec basillar invagination)invagination) Basilar impression refers to

secondary or acquired forms of BI amp is due to softening of the bone amp is seen in conditions such as rickets hyperparathyroidism osteogenesis imperfecta Paget disease neurofibromatosis skeletal dysplasias and RA amp infection producing bone destruction with or without ligamentous laxity

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 52: Craniovertebral junction

Assimilation of atlas or Assimilation of atlas or occipitalizationoccipitalizationThe failure of segmentation between the skull

and first cervical vertebra results in assimilation of the atlas

The assimilation may be complete or partial

It invariably results in basilar invagination Wackenheimclivusbaseline may be normal

but the clivus-canal angle may be decreased

1048708When incompletely assimilated the atlas arches appear too high on the lateral plain radiograph or when completely assimilated are not visible at all

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 53: Craniovertebral junction

TOPOGRAPHIC FORMS (WACKENHEIM) 1048708Type I Occipitalization(generally subtotal)

associated with BI 1048708Type II Occipitalization(generally subtotal)

associated with BI amp fusion of axis amp 3rdcervical vertebrae

1048708Type III Total or subtotal occipitalizationwith BI amp maldevelopmentof the transverse ligament

1048708Type III may be associated with various malformations like C2-C3 fusion hemivertebra dens aplasia tertiary condyle

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 54: Craniovertebral junction

Anomalies of atlasAnomalies of atlas

With the exception of the various atlantooccipitalassimilations most atlas anomalies when isolated produce no abnormal CVJ relationships and are not associated with basilar invagination

The vast majority of anomalies consist of various arch clefts aplasias and hypoplasias

Arch anomalies are frequently mistaken for fractures in the evaluation of plain radiographs of patients with a history of cervical spine trauma

Differentiated from fracture Differentiated from fracture Developmental cleft margins are smooth with intact Developmental cleft margins are smooth with intact

cortical edge amp no soft tissue swelling cortical edge amp no soft tissue swelling Fracture Fracture jagged edges communicate with soft jagged edges communicate with soft

tissue welling displacement of lateral masses seen tissue welling displacement of lateral masses seen with axial compression fracturewith axial compression fracture

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 55: Craniovertebral junction

Ponticulus Ponticulus posticuskimmerlersquos deformityposticuskimmerlersquos deformity

it is a bony ridge projecting posteriorlyfrom the articularedge of the atlas superior articularfacet

The bony projection may be only a few mm long or may elongate to unite with the adjacent neural arch of the atlas to produce an ldquoARCUATE CANALrdquothrough which the vertebral artery passes

This is due to ossification of a portion of the oblique A-O ligament

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 56: Craniovertebral junction

Posterior arch anomalies(mc)Posterior arch anomalies(mc)

Total or partial aplasia of the posterior atlas arch is rare Although absence of the posterior arch when isolated is

usually asymptomatic but may be associated with anterior atlantoaxialsubluxation

Bilateral atlantoaxialsubluxationmay be associated with both total and partial aplasias simulating the Jefferson fracture

The majority of posterior atlas clefts (97) are midline whereas lateral clefts through the sulcusof the vertebral artery account for the remaining 3

Posterior arch rachischisismay be superimposed on the odontoidprocess or the axis body on the open-mouth odontoidview simulating a fracture

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 57: Craniovertebral junction

Split atlasSplit atlas

It is typically encountered in association with posterior rachischisis-ldquosplit atlasrdquo

1048708Normally on a lateral radiograph the anterior arch of the atlas appears crescenticor half-moon-shaped with dense cortical bone surrounding the medullarycavity and a well-defined predentalspace

1048708In anterior arch rachischisis the anterior arch appears fat or plump and rounded in configuration appearing to lsquolsquooverlaprsquolsquothe odontoidprocess (making identification of the predentalspace impossible) the arch may have unsharp duplicated anterior margins

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 58: Craniovertebral junction

Abnormalities of axisAbnormalities of axis

Stability dependent on integrity Stability dependent on integrity of osseous ligamentous and of osseous ligamentous and muscle complex Instability of muscle complex Instability of atlantoaxial joint is documented atlantoaxial joint is documented by lateral cervical radiographs in by lateral cervical radiographs in the fixed and extended neck the fixed and extended neck positionposition

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 59: Craniovertebral junction

Group I Group I In combination with In combination with occipitalization of atlas frequent fusion of occipitalization of atlas frequent fusion of C2 amp C3 vertebrae Odontoid displaced C2 amp C3 vertebrae Odontoid displaced posteriorlyposteriorly

Group II Group II Incompetence of odontoid Incompetence of odontoid process due to its maldevelopment process due to its maldevelopment

Group III Group III No occipitalization and No occipitalization and odontoid normal Odontoid seen to be odontoid normal Odontoid seen to be merely dislocated backward and clearly merely dislocated backward and clearly seen in flexion seen in flexion

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 60: Craniovertebral junction

Anomalies of axisAnomalies of axis

Atlanto-axial segementation Atlanto-axial segementation defectsdefects

Type I Os odontoideum Type II Ossiculum terminale Type III Agenesis of odontoid base Type IV Agenesis of apical segment Type V Agenesis of odontoid

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 61: Craniovertebral junction

Os odontoideumOs odontoideum

an independent osseous structure lying cranial to the axis body in the place of dens

The anterior arch of the atlas is rounded and hypertrophic but the posterior arch is hypoplastic

As the gap between the os odondtum and the axis body usually extends above the level of the superior articular facet of the axis cruciate ligament incompetence and A-A instability are common

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 62: Craniovertebral junction

Two types Two types Orthotopic Os lies in place of Orthotopic Os lies in place of

dens and moves in conjuction dens and moves in conjuction with atlas and axis (Transverse with atlas and axis (Transverse ligament is functional) ligament is functional)

Dystopic Os lies base or Dystopic Os lies base or occipital bone and moves with occipital bone and moves with the clivus with which it may the clivus with which it may fuse fuse

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 63: Craniovertebral junction

Os odontoideumOs odontoideum Dens fractureDens fracture

Convex upper Convex upper margin of axis margin of axis bodybody

Flattened sharp Flattened sharp uncorticated upper uncorticated upper marginmargin

Hypertrophy of Hypertrophy of ant Arch of atlas ant Arch of atlas

Normal half moon Normal half moon shape shape Appearance of ant Appearance of ant Arch Arch

Convex post Convex post border and border and increased cortical increased cortical thickness thickness

Gap between Gap between fragments is fragments is characteristically characteristically narrow amp irregularnarrow amp irregular

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 64: Craniovertebral junction

Persistent ossium terminalePersistent ossium terminale

(Bergmanrsquos ossicle)- failure of (Bergmanrsquos ossicle)- failure of fusion of terminal ossicle to the fusion of terminal ossicle to the remainder of odontoid process remainder of odontoid process (usually unites by 10-12 yrs ) (usually unites by 10-12 yrs ) Absolute differentiation with type Absolute differentiation with type 1 odontoid fracture may be 1 odontoid fracture may be difficult at times Whether difficult at times Whether congenital or traumatic in origin congenital or traumatic in origin this anomaly is stable when this anomaly is stable when isolatedisolated

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 65: Craniovertebral junction

Odontoid aplasiaOdontoid aplasia

Total aplasiaof the odontoidprocess is extremely rare

A true aplasiais associated with an excavation defect into the body of axis

may simulate osodontoideum as the osfragment may be perfectly projected over the atlas arch on the open mouth odontoidview

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 66: Craniovertebral junction

Traumatic lesions of cvjTraumatic lesions of cvj

MC clinical features are LOC or cranial nerve damage

Classification (Anderson amp Montesano) Type I impacted ndashdue to axial loading Type II Basilar skull -due to direct blow

to skull Type III Avulsion Type I amp II are stable injuries while type III

is potentially unstable

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 67: Craniovertebral junction

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare 1048708 Jefferson s burst of atlas 1stdescribed by

Geoffrey jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 68: Craniovertebral junction

OCCIPITO-ATLANTAL INSTABILITY Traumatic non traumatic Traumatic usually fatal 8 incidence in RTA Seen with cardiorespiratoryarrest quadriplegia loss

of autonomic function VA insufficiency etc

Traynelisclassification

Type I anterior displacement of occiputon atlas Type II vertical displacement bw occiputamp cervical

spine Type III posterior displacement of occiputon atlas

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 69: Craniovertebral junction

OF ATLAS Posterior arch 23rdof all occur at the junction of

posterior arch amp lateral mass (hyperextension injury) Anterior arch rare Jefferson s burst of atlas 1stdescribed by Geoffrey

jeffersonin 1920Jeffersonrsquos

Axial loading ndashdownward displacement of condyleswith separation of lateral mass of C1

Classically 4 part -2 each in ant amp post arch neck pain amp stiffness Cervical collar Halo immobilization

Non union ndashocciputto C2 fusion

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 70: Craniovertebral junction

HANGMANrsquoS ( TRAUMATIC SPONDYLOLISTHESIS OF AXIS )

ldquoJudicial Hangingrdquo-submentalknots causes dislocation of neural arch of axis

Today majority due to RTA Bl passing downward through the neural arch of

axis with resultant anterior displacement of C2 on C3

Two basic mechanisms

Hyperextension amp distraction Hyperextension amp compression

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 71: Craniovertebral junction

Type I are either non-displaced or have no angulationamp lt3mm of displacement (stable injury with uncommon neurological deficits)

Type II with significant angulationamp translation of anterior fragment

Type III with severe angulationamp displacement along with concomitant UL or BL facet dislocation

Neck pain but neurological deficits less Surgical Rx seldom required due to high chances of

spontaneous interbodyfusion amp healing Most managed by reduction amp external

immobilization

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 72: Craniovertebral junction

Hangmans x-rayHangmans x-ray

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 73: Craniovertebral junction

Hangman ctHangman ct

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 74: Craniovertebral junction

ODONTOID Constitute about 7 ndash14 of cervical spine Flexion is the MC mechanism of injury causing

anterior displacement of C1 on C2

Anderson amp DrsquoAlonzoclassificationndash Type I oblique avulsion through the upper part of

the odontoidprocess at the point of alarligament attachment

Type II occur at the junction of the odontoidprocess amp the body of axis

Type III extend down in to the body of axis

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 75: Craniovertebral junction

Type I are stable amp heal well if immobilisedin a collar or brace

Type III are usually stable skull traction fbhalo or brace for 3 ndash4 months results in fusion

Type II are prone to non union with a failure rate of 30 -60 with conservative measures

Indications for Sxndashdisplacement gt= 5mm nonunion age gt7 years disruption of the TL

Odontoidcompression screws (acute type II ) C1-2 arthrodesis(wiring fusion transarticularscrews

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 76: Craniovertebral junction

Cvj anomalies in downs Cvj anomalies in downs syndromesyndrome

Characterized by increased ligamentouslaxity and abnormal joint and bony anatomy which predispose to instability

1048708Incidence of radiologicAA instability ranges from 7 to 40 although lt 1 of patients are symptomatic

1048708The incidence of bony anomalies involving the occipital condyle C1 ring and odontoidis also increased in Down syndrome

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 77: Craniovertebral junction

Chiari malformationsChiari malformations

The Chiarimalformations are a group of hindbrain herniationsyndromes initially described by Austrian pathologist Hans Chiariin 1891

1048708Types of Chiari malformations Type I Caudal descent of cerebellartonsils in

cervical spine Osseous anomalies of posterior skull base and spine

It presents in early adulthood rather than at birth Associated with syringomyeliain 50 to 70

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 78: Craniovertebral junction

Type II Caudal descent of cerebellarvermisand brain stem into cervical spine

Open spinal dysraphism Hydrocephalus Multiple neuroaxisanomalies

Type III Craniocervicalencephalocelecontaining portions of cerebellum and brain stem

Hydrocephalus Type IV (Controversial not commonly accepted as a

Chiarimalformation)

Aplasiahypoplasiacerebellum

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 79: Craniovertebral junction

Non traumatic non developmental Non traumatic non developmental conditions associated with cvjconditions associated with cvj

Griselsyndrome Atlantoaxialsubluxationassociated with inflammation of adjacent soft tissues of the neck

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 80: Craniovertebral junction

Rheumatoid arthritis From laxity of the ligaments and

destruction of the articularcartilage Osteogenesisimperfecta Neurofibromatosis Morquiosyndrome Secondary to odontoidhypoplasiaor aplasia Other arthridities(PsoriasisLupus

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 81: Craniovertebral junction

Rheumatoid arthritisRheumatoid arthritis

AAD is due to loss of tensile strength amp stretching of TL due to destructive inflammatory changes as well as secondary degenerative changes in tissues from vasculitis

1048708Similar changes occur in the median amp lateral joints which result in erosive changes in adjacent bone amp formation of granulation tissue in the synovialjoints

1048708Odontoidprocess ndashosteoporosis angulation

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 82: Craniovertebral junction

Osteophyteformation (stabilizing effect) does not occur secondary to deficient osteogenesis(characteristic of RA)

BI occurs secondary to loss of bone in lateral mass of the atlas with resultant rostralmigration of axis vertebra

The lateral mass of atlas may with lateral displacement of bone fragment

Later on erosion of occipital condylesoccur Excessive granulation tissue along with

invaginatedodontoidproduces ventral cervicomedullarycompression

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 83: Craniovertebral junction

Tb Tb

lt1 of all cases of spinal TB 1048708Local pain restriction of neck movements amp acute

tenderness of upper C-spine ndashCardinal features 1048708Compression of CMJ could be due to granulation

tissue cold abscess or bony instability amp displacement

1048708Lower cranial Nerves paralysis + 1048708Waxing amp waning picture due to acute

compression of ASA on flexion which gets releivedon extension

1048708Ligaments are extensively infiltrated by the disease process amp give way

1048708Hyperaemicdecalcification occurs

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 84: Craniovertebral junction

Radiological findings in 3 stagesndash

Stage I Retropharyngeal abscess with ligamentouslaxity + bony architecture of C1-C2 preserved

Stage II Ligamentousdisruption with AAD minimal bone destruction amp retropharyngeal mass +

Stage III marked destruction of bone complete obliteration of anterior arch of C1 amp complete loss of odontoidprocess marked AAD amp O-A instability

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 85: Craniovertebral junction

neoplasmsneoplasms

Unusual Metastaticmalignancies such as carcinoma of the

breast lung prostate kidney and thyroid in adults and neuroblastoma Ewingrsquos tumor leukemia hepatomaand retinoblastoma in children are most common

Primary malignancies involving the craniocervicaljunction are rare(multiplemyeloma)

Benign tumors are very rare

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you
Page 86: Craniovertebral junction

Thank youThank you

DrsameeraDrsameera

  • Craniovertebral junction
  • Slide 2
  • Embryology and development of cvj
  • Eampd contdhellip
  • Slide 5
  • Eampd contdhellip
  • Slide 7
  • Slide 8
  • Slide 9
  • Anatomy
  • articular
  • Ligamentous
  • Slide 13
  • muscles
  • neural
  • Neural contdhellip
  • lymphatics
  • vascular
  • kinetics
  • Kinetics contdhellip
  • Slide 21
  • Radiological evaluation of cvj
  • Slide 23
  • craniometry
  • Slide 25
  • Chamberlanrsquos line(palato-occipital)
  • Mcgregors line(palato-suboccipital line)
  • Wakenheimclival line(basilar)
  • Kalus index(ht of posterior cranial fossa)
  • Slide 30
  • Mcray line(plane of foramen magnum)
  • Basal angle
  • Boogards line amp angle
  • Atlanto temporomandibular index
  • Spino lamellarline
  • Frontal projection
  • Condylar angle (Schmidt ndash Fisher angle
  • Slide 38
  • Classification of abnormalities of CVJ
  • Slide 40
  • Slide 41
  • Slide 42
  • Slide 43
  • Slide 44
  • Anomalies of occiput
  • Kippel feil syndrome
  • Slide 47
  • Slide 48
  • Anomalies of occiput contdhellip
  • Basilar invagination
  • Slide 51
  • Slide 52
  • Basilar impression(sec basillar invagination)
  • Assimilation of atlas or occipitalization
  • Slide 55
  • Anomalies of atlas
  • Ponticulus posticuskimmerlersquos deformity
  • Posterior arch anomalies(mc)
  • Split atlas
  • Abnormalities of axis
  • Slide 61
  • Anomalies of axis
  • Os odontoideum
  • Slide 64
  • Slide 65
  • Persistent ossium terminale
  • Odontoid aplasia
  • Traumatic lesions of cvj
  • Slide 69
  • Slide 70
  • Slide 71
  • Slide 72
  • Slide 73
  • Slide 74
  • Slide 75
  • Slide 76
  • Hangmans x-ray
  • Hangman ct
  • Slide 79
  • Slide 80
  • Slide 81
  • Cvj anomalies in downs syndrome
  • Chiari malformations
  • Slide 84
  • Non traumatic non developmental conditions associated with cvj
  • Slide 86
  • Rheumatoid arthritis
  • Slide 88
  • Tb
  • Slide 90
  • neoplasms
  • Thank you