By: Dr. Kush vyas

Moderator: Dr. sanjeev Reddy

32 days: first evidence of limb buds

appearance

3rd wk: limb makes their appearance as a

small elevation as buds at side of the trunk

4th-5th wk: axial part of the mesoderm of the

limb bud becomes condensed converted into

cartilaginous skeletal and ossification center

of the limb formed

6th wk: upper furrow: arm, forearm hand

:Lower furrow: thigh leg foot

Medial side of the leg

After femur the longest bone to be formed

into body

1 body

2 extremities from where tibia bone going to be

formed

– upper extremity

lower extremity

Large and expanded into two eminences,

medial

lateral

Borders

Medial border: smooth ,rounded above and

below and prominent in centre

Lateral border: a/k/a interossous crest , thin

and prominent where interossous memb.

attaches

Surfaces:

Medial surface: smooth concave and broader

Lateral surface:

narrower than medial

upper 2/3rd:shalow grove for origin of the tibialis ant.

Lower 1/3rd : smooth and concave curves gradually

forward to ant aspect of the bone covered by

tendons of tibialis ant. ,EHL , EDL

Post. surface:

prominent ridge will be popliteal line

MIDDLE 1/3rd : divided into two part by vertical ridge

Medial and broader part: will give origin to FDL

Lateral and narrow part: tibialis post.

remaining post. Part is smooth and covered by

tibialis post. ,FDL and FHL

Much smaller than upper

surfaces:

Ant surface:

above- smooth and rounded covered by tendon of

extensors

Lower margins : rough and transverse depression

where articular capsule of ankle joint attaches

Post surface:

transverse shallow grove

Post surface of talus

Serving passage for tendon of FHL

Lateral surface: triangular rough depression,

inferior interoosoeus ligament connecting with

fibula

Medial surface: medial malleolus

Specific type of the non union

Either well established or incompletely

developed

characterized by osseous dysplasia and

segmental weakness of bone resulting in

anterolateral angulations of tibia with

pathological fracture with lack of bone union

Define: congenital condition of unknown

origin in which discontinuity of the bone at

the junction of middle and distal 1/3rd or

beyond is present at the birth or develops

during growth period and permitting

persisting abnormal mobility and creating

illusion of false joint

Incident: 1 in 250,000 live birth

1891:Paget one of the first describe a case

of CPT

1903: codivilla performed a lengthening

through a femoral osteotomy followed by

gradually calcaneal traction and casting

1921: Putti lengthened the femur using

distal traction and proximal counter traction

followed by nail inserted in the femur

1937: ducroquet and cottard describe

association of CPT with neurofibromatosis

1951: Mc farland suggeted bone bypass graft

1961: G.A. Ilizarow discovered that slow

distraction of a corticotomy through process

through process called distraction

osteogenesis could produce new bone in

widening distraction gap

1956: charney treated CPT with intra

meddulary nail

1982: boyd classified CPT

1985: pho et all describes efficiency of

vascularised fibular graft

Unknown

40 % of patient present with typical of

neurofibromatosis or von Recklinghausen’s

disease which is inherited as an autosomal

dominant trait with variable penetrance and

high rate of mutation

A possible relationship of neurofibromatosis,

fibrous dysplasia and CPT is suggested

The fact the fibrous constriction lesion is

universally present which suggests primary

pathological lesion is in the periosteal

structures found in tibia

the defect leads to fracture and non union,

mechanical forces accentuate the problem

Tibia is the most commonly affected site

associated with congenital anterolateral

bowing in neurofibromatosis which

progresses to neurofibromatosis

CPT can also occur in fibula, radius, ulna,

femur and clavicle

1.Complete defect in bone

2.Congenital bone cyst

3.Congenital bowing of tibia

typical pseudoarthosis found at birth.

The upper and lower segment represent variable diaphysis, ranging from proximal

segment representing 2/3rd to 3/4th of the bone

a shorter distal fragment ranging from 1/3rd to very small portion of the bone

Nearing the defect the diaphysial portion become progressively tapered, their ends sclerotic and medullary canal obligated

The interval between the bone ends are

occupied by very cellular fibrous tissue which

continue with periosteum

When the fibers are arranged in whorls and

contain an occasional fine bone trabeculum,

it resembles the histological picture of

fibrous dysplasia

occurs in the lower third of tibia microscopic

picture closely resembles that seen in fibrous

dysplasia

Diameter of tibia neither narrowed nor

appreciably expanded

fracture invariably takes place through the

weaned shaft

Complicated by fracture will results in

pseudoarthosis

Anterolateral bowing is usually predisposed to

this complication

Postero medial bowing rarely undergoes a

pathological fracture

Boyd classification:

Type1:

Ant. Bowing and defect in tibia present at birth

Other congenital deformities also present which

affect the ultimate management of the

pseudoarthorosis

Type2:

Most common

Often associated with NF

Worst prognosis

Ant bowing and hourglass constriction of tibia

present at birth

Spontaneous fracture or fracture following minor

trauma commonly occurs before 2 yr of age

‘’HIGH RISK TIBIA’’

Tibia is tapered and sclerotic and the medullary

canal is obliterated

Type3:

Develops in congenital cyst usually near the

junction of the middle and distal third of the

tibia

Ant bowing may precede or follow the

development of fracture

Recurrence of the fracture after Rx is less

common than type 2

excellent results after only one operation noted

Type 4:

Originates in the sclerotic segments of the bone

in classic location without narrowing of tibia

Medullary canal is partially or completely

obliterated

Insufficiency or stress fracture develops in the

cortex of the tibia and gradually extends through

the sclerotic bone

With completion of the fracture, healing fails to

occur and fracture widens and pseudoarthosis

occur

Prognosis: good to better

Type5:

Pseudoarthosis of tibia with dysplastic fibula

May be both bone involved

Prognosis: good if lesion confined to fibula

If progresses to tibia mostly prognosis and progression

resembles type 2

Type6:

Occurs in interaosseous neaurofibroma or

schwanoma that results in pseudoarthosis

rarest

Type 1 : ANTEROLATERAL BOWING OF TIBIA

Type 2: anterolateral bowing

increased cortical thickness

narrow medullary canal

tubular defect

Type 3: cystic lesion

Type 4: presence of fracture, a cyst or frank

pseudoarthosis

—7-month-old boy with neurofibromatosis and congenital pseudoarthrosis of the tibia

(Crawford type I).

7-month-old boy with neurofibromatosis and

congenital pseudoarthorosis of the tibia

(Crawford type I).

shows congenital pseudoarthorosis of the

tibia as hyperintense lesion

Note diffuse edema of soft tissue ventral to

the tibia

—7-month-old boy with neurofibromatosis and congenital pseudoarthorosis of the tibia

(Crawford type I).

7-month-old boy with neurofibromatosis and

congenital pseudoarthorosis of the tibia (Crawford

type I)

T1-weighted MR image depicts anterolateral

bowing and circumscribed cortical thickening of

the tibia Medullary canal is preserved

Bone marrow in region of anterolateral bowing of

tibia shows slight hypointensity

—1-month-old male neonate with congenital pseudoarthorosis of tibia (Crawford type III)

without neurofibromatosis.

ty

—1-month-old male neonate with congenital

pseudoarthrosis of tibia (Crawford type III)

without neurofibromatosis

Lateral radiograph of calf depicts characteristic

cystic lesion in distal part of tibia

—1-month-old male neonate with congenital pseudoarthrosis of tibia (Crawford type

III) without neurofibromatosis.

y

—1-month-old male neonate with congenital

pseudoarthorosis of tibia (Crawford type III)

without neurofibromatosis

—1-month-old male neonate with congenital pseudoarthrosis of tibia (Crawford type

III) without neurofibromatosis.

—1-month-old male neonate with congenital pseudoarthrosis of tibia

(Crawford type III) without neurofibromatosis.

—5-year-old boy with neurofibromatosis and congenital tibial pseudoarthrosis

(Crawford type IV).

5-year-old boy with neurofibromatosis and

congenital tibial pseudoarthorosis (Crawford

type IV)

Lateral radiograph of right leg depicts

congenital tibial pseudoarthorosis type IV

with frank pseudoarthorosis of distal tibia.

—5-year-old boy with neurofibromatosis and congenital tibial

pseudoarthrosis (Crawford type IV).

5-year-old boy with

neurofibromatosis and congenital

tibial pseudoarthrosis (Crawford

type IV).

pseudoarthrosis is revealed as tibial

non-union with increased signal

intensity (arrowheads) and

hyperintense tissue ventral to

nonunion (arrows).

—5-year-old boy with neurofibromatosis and congenital tibial

pseudarthrosis (Crawford type IV).

y

—5-year-old boy with neurofibromatosis and

congenital tibial pseudoarthorosis (Crawford

type IV)

show a hypo intense pseudoarthorosis area

with hypo intense soft tissue ventral to

pseudoarthorosis

—5-year-old boy with neurofibromatosis and congenital tibial pseudoarthorosis

(Crawford type IV).

y

5-year-old boy with neurofibromatosis and

congenital tibial pseudoarthorosis (Crawford

type IV). Pseudoarthorosis and soft tissue

(arrows)

show marked contrast enhancement after

administration of gadolinium

frequently noted at birth

The deformity is evident from the apical

prominence laterally in the leg with the foot

inverted or at least medially displaced in

relation to the lower leg

If neonatal fracture has occurred, mobility at

pseuarthrosis sight will be evident

Vast majority of anterolateral deformity are

unilaterally, the shortening and angulations

is easily appreciated when the affected leg is

compared with the normal leg

If signs f neurofibromatosis are present , diagnosis is readily apparent

A milder deformity unaccompanied by signs of neurofibromatosis may not come to medical attention until much later when a limp due to deformity or an impending fracture draws attention to the leg

the foot and ankle may be normal or slightly normal or slightly smaller than the contra lateral side

Presence of cutaneous manifestations of neurofibromatosis clarifies the diagnosis

The late form of pseudoarthosis presents as fracture in an older child will probably be companied by of this findings

x ray is the diagnostic tool

A dysplasia of the mesodermal and neuroactodermal tissue ,neurofibromatosis is a hereditary dysplasia that may involve almost every organ system of the body .

Incidences: 1 in 3000 live births .

50% are autosomal dominant and

50% are sporadic from spontaneous mutatations

Neurofibromatiosis is divided into two types

1.NF1 or von Recklinghausen disease

2.NF2 or bilateral acoustic neuroma

frequency of 9:1NF1 to NF2

Chromosome 17 is defective in NF1 and skin manifestation are prominent ,such sessile or pedunculated skin lesions (mollusca fibrosa) and café au lait spots

Haemartomas,gliomas, and alignment nerve sheath tumor are also seen in NF1along with spiral neaurofibroma.

Chromosome 22 is defective in NF2 and there are minimal skin manifestation . Bilateral acoustic neuromas meningiomas ,

schwanoma are seen in NF2 along with spinal schwanoma

Only NF1 manifests skeletal finding with radiographic features

At least 50% of patients demonstrate bony changes most commonly pit like cortical erosions resulting from

direct pressure from adjacent nuerofibromas ,

commonly in long ones and ribs .

The ribs may also appear twisted or ribbon like

The long bones may demonstrate overgrowth, cortical defects or, bowling and sclerosis.

Pseudoarthosis formation , most commonly within tibia

The long bones are the sight of fibrous cortical defects and nonossifying fibromas associated with neurofibromatosis

AP AND LATERAL VIEW SHOULD BE TAKEN

Initially,

bowing of the tibia convex anteriorely or

anterolaterally with narrowing of the tibial shaft

and sclerosis encroaching on the medullary cavity

at apex

The apex of the of the curve is typically at

junction of middle and distal third of the

shaft of the tibia

Sometimes, shows pointed distal fragment

and cupped proximal fragment which fit

together to form a false joint

There is often associated tibial shortening

50% cases, associated With NF will show lytic

lesion within the tibia

1.Fibrious dysplasia

2.Rickets

3.osseous syphilis

Early congenital syphilis

Late congenital syphilis

4.infantile scurvy

5.Weisman Netter Stuhl syndrome

6.osteogenesis Imperfecta

7.Nonunion of fracture

8.blounts disease(infantile tibia vara)

depends on age and type

A true congenital pseuarthrosis of the tibia

will not heal when treated by casting alone .

treatment categorized as

1. prophylactic

2. directed towards pre pseudoarthosis lesions

3.active directed towards fully developed

congenital pseudoarthorosis

Patients with anterolateral bowing of the tibia at the junction of the middle

and distal 3rd of the diaphysis

accompanied by narrowing and sclerosis

lose of definition of medullary canal

considered at a risk for developing pathological fracture and non union .

The extremity must be protected by an orthotic and the child guarded against excessive activity

As longitudinal growth and remodeling take place, there is a tendency towards spontaneous correction of the deformity and restoration of normal architecture

The threat of fracture is present until skeletal maturity is reached

If fracture and pseudoarthosis is eminent particularly when the bone structure is inadequate and especially in presence of a cyst ,child’s activity cannot be controlled

The weakened region of the tibia is by passed by a cortical or rib transplant

Since congenital cyst in this region invariably fractures

This is treated by bypass procedure and later curette the defect, fill it with bone transplant and immobilize the leg in a cast till the defect is obliterated and the tibia of the adequate size is obtained

for an established pseudoarthorosis ,surgical treatment is necessary

older the patient more likely success of union so, age of puberty would seem to be appropriate but by this time , the leg is under developed, deformed and short and amputation frequency preferred

aggressive surgical interventions is undertaken early in childhood, because sufficient time reminds for growth factors to operate so that when skeletal maturity is reached , a well-developed even normal extremity results

if the initial surgery fails , the procedure may be repeated

surgery can be attempted as a early as four years of age

If surgery is to be postponed , deformity should be prevented by an orthotic

The principles of treatment are as follows :

1.Stabilization

a.dual on lay bone grafts (Boyd)

provides both stability and induction of

osteogenesis

Tapered slender bones will not permit its use

B . Intramedullary rod (charnley)

Disadvantages :

Permits the rotary forces which can be controlled

with a thigh length cast with the knee flexed at

45

Advantage:

Permits osteogensis inducing axial compressive

forces; controls a small distal fragment by trans-

tarsal insertion of the rod

C. External skeletal fixation

Useful for maintaining length ,when the defect is

inadequate and when poor soft tissue of the

pseuarthrosis will not permit local fixation device

2.stimulation of osteogenesis

A . Bone transplants

autogeneous cancellous bone is preferred

B. Electrical stimulation :

used along or as an adjunctive measure with

stabilization and bone transplant

this will increase the rate of bony union.

3.maintainace of cast immobilization

This is accomplished until bony bridging is

adequate and the structure and size of the tibia

are sufficient to withstand the possibility of a

pathlogigal fracture.

The cast must be applied to the upper thigh,

and the knee must be kept flexed at 45 degree to

prevent gravity induced tensile force

4.compression versus non compression stress

Proponents of intramaddulary rodding advocate

immediate wait bearing to encourage

compression stress that induce osteogenesis

Two tibial cortical grafts and the supply of

cancellous bone chips are removed from a

donor or obtained from a bone bank

The pseudoarthorosis is exposed, and the

fibrous tissue , which may surprisingly extend

into the surrounding soft tissue, is

completely excised .

The eburnated bone is removed from the

bone ends and the medullary canal is opened

up by drilling

To correct bowing and the equines deformity an Achilles tenotomy and a fibular osteotomy are required if possible ,the fibula should be preserved to aid postoperative immobilization

The lateral surface of the tibia above and below are shaved down flat , and the tibial cortical transplant are affixed ,one medullary and other laterally .

A space is left between the ends of tibial fragments into which are packed the cancelous bone chips .

Only skin and subcutaneous tissue are closed .

A cast is applied and immobilization is continued until union is apparent .

Fracture and non union are postoperative complications ,and proyectio of the leg by a letherlacer brace or a plastic orthosis and until skeleton maturity is mandatory

Fixation by an interaamadulary rode eliminate an angulatory strain while permitting beneficial axial compressive forces desperate the criticism that is still allows rotational stresses.

rodding of the tibia by the transtrasal approach is the most commonly used method of fixation

it can be used alone with external plaster support ,but generally autogeneous cancelousbone or osteo-perosteal bone transplant are added fixation tarsus will prevent nail from cutting of short distal segment of the tibia before introducing the nail

Achilles tenotomy will overcome the bowing action that prevents correction of deformity

mcElvenny first called attention to the heavy

cuff of tissue surrounding the bone at

pseudoarthorosis and he reasoned the

presence of these tissues , whether

congenital or a result of a fracture , may

decrease bone production and consequently

healing .

any operation for congenital

pseudoarthorosis should include complete

excision of this tissue

the operation for prophylactic treatment of

an imminent fracture and pseudoarthorosis a

congenital angulation of the tibia

a single graft is placed posteriorly so as to

span the pseudoarthorosis except and the

upper and lower sites of implantation the

transplant is entirely exta-periosteal

The operation is also used preliminary to

curettage and bone grafting of a congenital

cyst pre-pseudoarthrotic lesion

Sofield’s multiple osteotomies with internal

fixation by medullary rods:

Fragmentation

Reversal of fragments

realignment

Micro vascular transfer of the contrlateral or

ipsilateral fibula

Pre operative arteriography is must to determine

anastomosis sight and rule out anomalies

Disadvantage:

Non union at one end of the tibia

Stress fracture

Morbidity of donor leg

Principle: correct all angular deformity and

maximizes the cross sectional area of union

of pseudoarthorosis

Therapeutic consideration includes:

Type of bone graft

Type of fixation

Whether to resects the pseudoarthorosis or not

Electrical stimulation has been used

primary goal: union of the site

Most imp thing to be kept in mind:

Correct associated problems

Leg length discrepancy

Multilevel multi directional tibia deformity

Proximal migration of fibula

Fibular non union

Ankle mortise valgus

Ankle joint dorsiflexion

Cavo valgus deformity

Valgus contracture

Principle of distraction osteogenesis:

Same as limb lengthening principle i.e distraction

of a bone segment after low energy periosteal

osteotomy by mechanically applied tension force

is primary method of limb lengthening

For skeletally immature patient:

Epiphysiolysis of epiphyseal distraction

The site of bone regeneration constitutes the

centre for stimulating the surrounding

musculo-facial neurovascular and cutaneous

tissue

The degenerated bone undergoes maturation

or consolidation phase at the end of the

lengthening until the new bone is

morphologically and functionally in

distinguishable form

1 mm/day is the critical rate and critical

rhythm in limb lengthening

(0.25/6hour is the standard format should be

followed clinically)

Lengthening should be performed without

interruption function of the limb including partial

wt bearing in the leg is actively encouraged

Indication: Limb lengthening

Angular or multiplanar deformities

Foot deformities including lengthening of the metatarsal

Congenital anomaly of the lower limb( tibial and fibular hemimelia)

Joint contracture

Non union and infected non union

Acute fracture

Fusion of the joint

In case of dwarfism

1.when to lengthen??

2.how much bone can be lengthened??

3.which external fixator should be used??

4.what are the stage of the lengthening

proess??

5.How long should be external fixator used??

Amount of the discrepancy and age of the

patient

Discrepancy; >5 cm or

expected discrepancy at the age of skeletal

maturity i.e boys 16 yrs and girls 14yrs

No strict age demarcation is there but the

idea behind eligibility is child should bear

weight and co operate with the excersise

regimen

If angular deformity is present and

discrepancy is even less than 5 cm:

Should be corrected surgically and using external

fixator

Yet to be determined

20 to 25% of original length gived good

results

More than 25% lengthening lead to higher

incidence of all types of complications

Most of the time is surgeon’s choice

Ilizarow circular fixator for tibial lengthening

Uniplanar fixator for femoral lenghtening

Half pins for uniplanar fixator

K wire for ilizarow

4 stages

Distraction

Consolidation

Removal of frame

rehabiliation

Distraction:

5-7 days post operatively

Optimum distraction 1mm/day (0.25mm/6 hour)

Example: 5 cm lengthening will require

approximately 50 days

Consolidation:

External fixator kept on the patient until the

bone formed consolidates and becomes strong

enough to allow safe removal

Duration: amount of the lengthening

Aprox:1 month for 1cm

Removal of the frame:

Evaluate quality and strength of the new bone

Plain radiograph should show

New bone formation

Should completely bridges and lengthened the site

New cortex formation

Followed bt brace or cast support for 6 wks

Rehabilitation:

After removal of cast and brace

Physiotherapy

ROM

As explained, for every 1 cm 1 month to 2

month required

Depends on discrepancy