Developmental Disturbances ofThe Oral Cavity IMPORTANT TERMINOLOGIES

 Developmental DisorderIt is the abnormality, defect or disturbance which occurs during the developmental stages of the tissues or organs.

 Congenital DisorderIt is the abnormality, defect or disturbance which occurs during the developmental stages of the tissues or organs in the intra-uterine stages.

 The manifestation may be visible at the time of birth; OrIn the later stages during growth of the affected tissues.

 Inherited DisorderThe abnormality, defect or disturbance which is transmitted through genes to off springs in the subsequent generation.

 Genetic DisorderThe abnormality, defect or disturbance which is caused by gene abnormality or mutation.

 Craniofacial Anomalies (CFA)•    Anomaly         : Irregularity/different from normal.

•    CFA       : Deformities in the growth of the head & neck. Factors responsible are:  1) Combination of genes  2) Environmental  3) Deficiencies (e.g. Folic Acid)

 Common types of CFA

         Cleft Lip, Cleft Palate Or CLEFT LIP & PALATE (Unilateral/Bilateral)

          Craniosynostosis

Sutures in skull close too early.     Causes problem in brain & skull growth.     May also cause pressure inside of the head to increase.     The skull & facial bones to change from a normal, symmetrical appearance.

          Hemifacial Microsomia/ Goldenhar

syndrome/   Brachial Arch syndrome Tissues on one side of the face are underdeveloped.Affecting primarily the ear, mouth & jaw areas.Sometimes both sides may be affected & may involve skull as well as face.

 

         Vascular MalformationA birthmark or a growth, present at birth which is composed of blood vessels that can cause functional or esthetic problems.May involve multiple body systems.Named after type of Blood Vessels involved.Types:   Lymphangiomas

                             Arteriovenous malformations                             Vascular gigantism

         HemangiomaA type of birthmark,Most common benign tumor of skin,May be present at birth (faint red mark),Or appear in 1st months after birth,Types:   Port wine stain,

Salmon patch,Strawberry Hemangioma.

          Deformational Plagiocephaly

Plagiocephaly : Means oblique headAsymmetrical shape of head from repeated pressure to the same area of the head.

          Teratogenic Agents causing CFA

Cause birth defects when present in the fetal environment.Can be drugs, chemicals & infectious physical & metabolic agents.May adversely affect the intrauterine environment of the developing fetus.Extent of defect depends on dose, time of development, susceptibility & interactions.

  CHAPTER OUTLINE•    DD of the Jaws•    DD of the Lips & Palate•    DD of the Oral Mucosa•    DD of the Gingiva

•    DD of the Salivary Glands•    DD of the Tongue•    DD of the Teeth

•    Developmental / Fissural Cysts of the Oral & ParaOral regions

DEVELOPMENTAL DISTURBANCES OF THE JAWS

•      Agnathia•      Micrognathia•      Macrognathia•      Facial Hemihypertrophy•      Facial Hemiatrophy

AGNATHIA     (Otocephaly, holoprosencephaly agnathia)

•      Hypoplasia or absence of the  mandible.•      Abnormally placed ears.•      Autosomal recessive trait.•      Only a portion of jaw is missing, commonly.•      In maxilla, either one maxillary process or even

premaxilla may be involved.•      If mandible is involved:     Entire mandible, or condyle or ramus of one side.     Rarely bilateral agenesis of condyles or of ramus

also seen.

•      Due to failure of migration of neural crest mesenchyme  into the maxillary prominence at the 4th to 5th week of gestation.

•      Prognosis is very poor.•      Considered lethal.

MICROGNATHIA•      It means - Small jaw,•      Either maxilla or mandible ,•      Abnormal positioning or abnormal relation of one

jaw with other may  give the illusion of a smaller jaw,

•      True can be acquired or congenital.•      Congenital is associated with anomalies like     -   Congenital heart disease,     -   Pierre Robin syndrome,•      Follows hereditary pattern•      In maxilla, it occurs due to deficiency in the

premaxillary area & pts. have middle 1/3rd of the face retracted.

•      Mouth breathing could cause micrognathia although it is possible that micrognathia may lead to mouth breathing due to maldevelopment of nasal & nasopharyngeal structures.

If it affects the mandible, there is:•      Retrusion of chin,•      Other reasons of this Retrusion can be:

     - Posterior positioning of mandible or     - Steep mandibular angle,•      Agenesis of condyles

Acquired is b’coz of postnatal reasons, which mainly results from disturbance in the area of TMJ.

e.g.  Ankylosis of TMJ, due to trauma or by infection of mastoid, of middle ear or of joint itself.

Clinically seen as:   1.  Severe retrusion of chin.   2.  Steep mandibular angle.   3.  Deficient chin button.

OTHER POSSIBLE CAUSES:-        Congenital Syphilis-        Turner’s Syndrome-        Treacher-Collins Syndrome

MACROGNATHIA•      Abnormally large jaws,•      Increase in size proportional to generalized

increase in the size of entire skeleton like in pituitary gigantism,

Other conditions are:•      Paget’s disease of bone, where overgrowth of

cranium & maxilla,

•      Acromegaly, mandibular enlargement owing to hyperpituitarism,

•      Leontiasis ossea, a form of fibrous dysplasia where enlargement of maxilla ,

Etiology:

•      Sometimes due to disparity in the size of maxilla in relation to the mandible.

•      Angle between ramus & body also influence the relation of mandible to maxilla, as does the actual height of the ramus.

•      Long rami with less steep angle with body.•      Excessive condylar growth predisposes to

mandibular prognathism,•      Factors favoring mandibular prognathism,      > Increase in height of ramus      > Increased mandibular body length      > Increased gonial angle      > Anterior positioning of glenoid fossa,      > Decreased maxillary length,      > Posterior positioning of maxilla in relation to

cranium,      > Prominent chin button      > Varying soft tissue contours

Treatment•      Surgical correction is possible.•      Ostectomy or•      Resection of a portion of the mandible to decrease

its length.

FACIAL HEMIHYPERTROPHY•      Also termed as Facial Hemihyperplasia,•      Rare developmental anomaly,•      Asymmetric overgrowth of 1 or more body parts,•      Can be isolated or associated with other

malformation syndromes,•      In the true sense, it is caused by hyperplasia of

tissues than hypertrophy.•      Associated syndromes are     > Beckwith-Wiedemann syndrome     > Neurofibromatosis     > Epidermal nevus syndrome     > Multiple exostoses syndrome     > Klippel-Trenaunay-Weber syndrome     > McCune-Albright syndrome

Etiology:-      Hormonal Imbalance-      Chromosomal Abnormalities-      Intrauterine Localised Alterations-      Vascular / Neurogenic

ClassificationBy Hoyme et al

•      Complex hemihyperplasia :     Involving half of the body  (atleast 1 arm & 1 leg)

                                                   They can be ipsilateral or contralateral

•      Simple :  Involvement of single limb•      Hemifacial hyperplasia:  Involvement of one side

of face.

Clinical Features•      Enlargement confined to one side of body.•      Unilateral macroglossia.•      Premature development & eruption.•      Increased size of dentition.•      Females > males 63:37.•      Equal involvement of right & left sides.•      Familial occurrence seen sometimes.

Oral Manifestations•      Dentition : crown size, root size & shape & rate of

development may be affected.•      Mainly permanent teeth affected.•      Teeth involved are enlarged.•      Mainly cuspids, premolars & 1st molar.•      Roots may be enlarged or even short.•      Permanent teeth on the affected side develop

more rapidly.•      Erupt before their counterparts on the uninvolved

side.•      Premature shedding of deciduous teeth.•      Bone of maxilla & mandible also enlarged.•      Wider & thicker, with altered trabecular pattern

sometimes.

Tongue :

•      Enlarged lingual papilla,•      Unilateral enlargement,•      Contralateral displacement,

Buccal mucosa :•      Appears velvety,•      May seem to hang in soft, pendulous folds on the

affected side.

Histologic Features•      Generally uninformative.•      True muscular hypertrophy was not found.

Differential Diagnosis•      Neurofibromatosis.•      Fibrous dysplasia of the jaws.

Treatment & Prognosis•      No specific treatment,•      Attempts at cosmetic repair,•      Periodical abdominal ultrasound /MRI is

recommended to rule out tumors.

FACIAL HEMIATROPHY

Other names :          Parry-Romberg syndrome,          Romberg-Parry syndrome,          Progressive facial hemiatrophy,          Progressive hemifacial atrophy

•      1st reported by Romberg in 1846,•      Slow progressive atrophy of the soft tissues of

essentially half the face char. by progressive wasting of subcutaneous fat, sometimes accompanied by atrophy of skin, cartilage, bone, muscle.

•      Although the atrophy is usually confined to one side of the face & cranium, it may occasionally spread to the neck & one side of the body & it is accompanied usually by contralateral jacksonian epilepsy,trigeminal neuralgia, and changes in the eyes & ear.

Etiology•      Cerebral disturbance leading to increased &

unregulated activity ofsympathetic nervous system, which in turn produced the localized atrophy through its trophic functions conducted by way of sensory trunks of the trigeminal nerve.

Other possible etiologies include:•Peripheral Trigeminal Neuritis•A type of Scleroderma•Trauma / Infection / Heredity.

Clinical Features•      Appears in 1st or 2nd decade of life.•      Progresses over a period of 2 to 10 years.•      Female : Male is 3:2•      Slight predilection for the left side.

•      Early sign : Painless cleft, line or furrow which is white in colour near the midline of the face or forehead.

•      This marks the boundary between normal & atrophic tissue.

•      This mark is termed as “COUP DE SABRE”. It appears like the scar of a fencing sword.

•      A bluish hue may appear in the skin overlying atrophic fat.

•      Atrophy follows the distribution of one or more divisions of the trigeminal nerve,

•      Facial flattening may be mistaken for Bell’s palsy.

•      Affected area extends progressively with the atrophy of the skin, subcutaneous tissue, the muscles, bone, cartilages, alveolar bone & soft palate on that side of the face.

•      Ipsilateral wasting of the salivary glands & hemiatrophy of the tongue.

•      Hollowing of the cheek may be seen.•      Eyes appear depressed in the socket.•      Unilateral involvement of the ear, larynx,

esophagus, diaphragm, kidney & brain.•      It starts in 1st decade & lasts for about 3 yrs before

it becomes quiescent,•      Final deformity varies widely, burning itself out in

some patients with minimal atrophy, while in others progressing to marked atrophy.

•      Rarely one half of the body may be affected,

•      Pigmentation disorders, vitiligo, pigmented facial naevi, contralateral jacksonian epilepsy, contralateral trigeminal neuralgia & ocular complications.

Oral Manifestations•      Dental : Incomplete root formation.•      Delayed eruption.•      Severe facial asymmetry, resulting in facial

deformation.•      Difficulty in mastication.•      Hemiatrophy of the lips & tongue.•      Eruption of teeth on affected side may also be

retarded.

Differential Diagnosis•      Post –traumatic fat atrophy,•      Hemifacial microsomia     (1st & 2nd brachial arch syndrome),•      Goldenhar syndrome,•      Partial lipodystrophy

Treatment & Prognosis•      No specific treatment.•      Disease is progressive for a period of several yrs

& then remains unchanged for remainder of the patient’s life.

ABNORMALITIES OF DENTAL ARCH RELATIONSDisparity of the relation of one jaw to the other.

Most accepted classification – Angle (1899)

Class I       -        Arches in normal mesiodistal relations.

Class II      -        Mandibular arch is distally placed in relation to the maxillary arch.•                           Div 1-        Bilaterally distal, protruding maxillary incisors•                           Div 2-        Bilaterally distal, retruding maxillary incisors

Class III-   Mandibular arch mesially placed in relation to maxillary arch•                           Div.: Bilaterally mesial.•                           Subdiv:      Unilaterally mesial

DEVELOPMENTAL DISTURBANCES OF THE LIPS & PALATE

•      Congenital Lips & Commissural Pits & Fistulas,•      Van Der Woude syndrome,

•      Double Lip•      Cleft lip & Cleft palate,•      Cheilitis Glandularis,•      Cheilitis Granulomatosa,•      Hereditary Intestinal Polyposis syndrome,•      Labial & Oral Melanotic Macule

CONGENITAL LIP & COMMISSURAL PITS & FISTULAS

•      Malformations often following a hereditary pattern.•      May occur alone or in associated with other

developmental anomalies.•      Like cleft lip or cleft palate or both.

Etiology•      Pits may result from notching of the lip at an early

stage of development.•      With fixation of the tissues at the base of the notch

or  From failure of complete union of the embryonic lateral sulci of the lip.

•      These persist & develop into the typical pits.

Clinical Features•      Unilateral/bilateral depression that occurs on

the vermilion surface of either lip.•      Mainly on the lower lip.•      A sparse mucous secretion may exude from the

base of this pit.•      Lip appears swollen, accentuating the appearance

of the pits.

•      If fistula present, fluid may be expressed.

Treatment•Surgical excision•If asymptomatic, there is no need of any treatment.

VAN DER WOUDE SYNDROME(cleft lip syndrome, lip pit syndrome, dimpled papillae of the lip)

•Autosomal dominant syndrome.•Cleft lip, cleft palate & distinctive pits of lower lip.•Variations like lip pits alone, absent teeth or isolated cleft lip & palate also seen.•Orofacial anomalies mainly due to abnormal fusion of palate & lips.•Occurs at 30-50 days post conception.Clinical Features

•      Male = female,•      Variations in severity occurs,•      Even within families,•      Hallmark :  Cleft lip &/or palate + lower lip pit.•      Clefts may be unilateral/bilateral,•      Submucous cleft palate is common, which may be

missed on examination.•      Hypernasal voice & cleft or bifid uvula helps in

such diagnosis,•      Pits here are usually medial, on vermilion portion

of the lower lip,

•      Centered on small elevations in infancy, but are simple depressions in adults,

•      Pits often associated with accessory Salivary Glands, that empty into pits, (Sometimes with visible discharge)

•      Maxillary hypodontia, (missing maxillary incisors or premolars )

•      Syngnathia (congenital adhesion of jaws),•      Narrow, high arched palate,•      Ankyloglossia (Short glossal frenulum or tongue-

tie )

Extra oral manifestations•      Limb anomalies.•      Popliteal webs.•      Brain abnormalities.•      Accessory nipples.•      Congenital heart defects.•      Hirschsprung disease.

Treatment•      Thorough orofacial examination.•      Thorough general physical examination.•      Examination & genetic counseling by a pediatric

geneticist for families having inheritance for Van Der Woude syndrome.

•      Surgical repair of cleft lip & palate,•      Surgical excision of lip pits.

DOUBLE LIP

•      Rare anomaly characterized by redundant fold of tissue on the mucosal side of lip,

•      Congenital or Acquired,•      Congenital - 2nd to 3rd month of gestation,•      Acquired is called Ascher’s syndrome : results

from trauma or oral habits,

Clinical Features•      Upper lip > Lower lip.•      At times both lips may be involved.•      Observed when pt. smiles or when lips are tense.•      It cannot be observed when lips are at rest.•      When the upper lip is tensed, the double lip

resembles a “CUPID’s BOW”.

Acquired:Ascher ‘s syndrome is characterised by a triad of:–Double Lip ,–Blepharochalasis *,–Non –toxic thyroid enlargement (*- sagging of upper eyelid due to edema at the outer canthus, which causes interference with vision.)

Histologic Features•Epithelium is usually normal but abundance of minor Salivary Glands,

•Blepharochalasis shows hyperplasia of lacrimal glands.

Treatment•Mild cases require no treatment,•Severe ,simple surgical excision of excess tissue for esthetic purposes.

CLEFT LIP & PALATE

•      Common congenital malformations,•      2 types are:          -Cleft lip with or without cleft palate          -Isolated cleft palate•      Incidence varies depending upon geographic

origin, racial & ethnic backgrounds & socioeconomic status.

•      Failure in the fusion of nasal & maxillary processes leads to the cleft of the primary palate.

•      Unilateral / Bilateral,•      Slight notch on lip to complete cleft,•      Cleft of secondary palate is medial, bifid uvula to

complete cleft palate up to the incisive foramen.

Etiology•      Heredity ,•      Environmental factors are also important,•      Mode of transmission,     Single mutant gene, or

     No. of genes (polygenic inheritance)

Polygenic :•      Added effect of many genes from both  the

parents & showing effect if a certain threshold level of genetic liability is reached.

  Low risk type

Monogenic/Syndromic :•      (Associated with other congenital abnormality )    High risk type

Other causes:•      Defective vascular supply to the area,•      Mechanical disturbance where size of tongue may

prevent the union,•      Circulating substances, such as alcohol, certain

drugs & toxins,•      Infections, &•      Lack of inherent developmental forces.

Classification

DAVIS & RITCHIE CLASSIFICATION:   (1922) Group I      :        Pre-Alveolar Clefts                   (Clefts of the Lip alone)                             Unilateral / Bilateral / MedianGroup II    :        Post Alveolar Clefts                   (Different degrees of hard & Soft palate clefts that extend into the alveolar ridge)Group III:  Alveolar Clefts

                             (Complete Clefts involving the palate, Alveolar ridge & the lip)                             Unilateral / Bilateral / Median

VEAU classification   (1931)

Group I   (A):     Defects of soft palate only,Group II  (B):     Clefts involving  hard & soft palate extending upto incisive foramen.Group III  (C):    Complete Unilateral Clefts involving soft palate, hard palate, lip & alveolar ridge.Group IV (D):    Complete bilateral clefts affecting soft palate, hard palate, lip & alveolar ridge.

FOGH ANDERSEN CLASSIFICATION : (1942)

Group I      :        Clefts of the Lip.                   Single:       Unilateral / Median                   Double:     Bilateral CleftsGroup II    :        Clefts of the Lip & Palate.                   Single:       Unilateral                   Double:      Bilateral CleftsGroup III:           Clefts of Palate extending upto incisive foramen.

SCHUCHARDT & Pfeiffer's SYMBOLIC CLASSIFICATION :

•      This classification makes use of a chart made up of a vertical block

•      of three pairs of rectangles with an inverted triangle at the bottom

•      Inverted triangle – Soft Palate.•      Rectangles - Lip, Alveolus and Hard Palate.•      Areas affected by clefts are shaded in the chart.

KERNAHEN’s STRIPED “Y” CLASSIFICATION:

•      Symbolic classification•      This uses a stripped Y having numbered blocks.•      Each block represents specific areas of the oral

cavity.•      Block 1 and 4 –       Lip•      Block 2 and 5 –       Alvelous•      Block 3 and 6 –       Hard palate anterior to incisive

foramen•      Block 7 and 8 –       Hard palate posterior to

incisive foramen•      Block 9 –                 Soft Palate•      Boxes are shaded in areas where cleft has

occurred.

LAHSHAL CLASSIFICATION•Simple classification put forth by Okriens in 1987.•It is a paraphrase of the anatomic areas affected.•L-lip, A-Alvelous, H-Hard Palate, S- Soft Palate•Classification based on the fact that lips, alveolus and hard palate can be bilateral while soft palate are unilateral.•Denoted by indicating the alphabet standing for areas involving cleft.•E.g  L_ _ S_ _ _  - Denotes Cleft of right lip and soft palate.

Clinical Features•      CLP: More in males, more severe as well.•      Isolated cleft palate: More in females.•      Nonsyndromic & Syndromic forms.•      Syndromic form is with additional birth defects like

lip pits etc.•      Nonsyndromic are the ones with no physical &

developmental anomalies .

Cleft Lip•      Unilateral / Bilateral,•      Line of cleft starts on the lateral part of the upper

lip & continues through the philtrum to the alveolus between the lateral incisor & the canine tooth.

•      Clefting anterior to incisive foramen (lip & alveolus) : cleft of anterior palate,

•      May vary from a simple notch to more severely bilateral cleft lip &alveolus that separates the philtrum of the upper lip & premaxilla from the rest of the maxillary arch.

Cleft Lip Palate (CLP)•      When cleft lip continues from the incisive foramen

further through the palatal suture in the middle of the palate, it is termed as cleft lip with palate,

•      Unilateral / Bilateral,•      Cleft line may be interrupted by skin or mucosa

bridges, hard (bone) bridges or both.•      Isolated cleft palate is different from CLP,•      Both etiologically & embryologically,•      Several subtypes are :     > Cleft of uvula (mild form)     > Cleft of soft palate (severe form)     > Complete cleft palate : cleft of hard palate,

soft palate & cleft uvula

Clinical Significance•      Physical & psychologic effects.•      Difficulty in eating & drinking.

•      Speech problems.

Treatment•      Coordinated care of  Paediatric surgeons, Dentist

(Oral Surgeon, Orthodontist / Pedodontist & Esthetic Dentist), Speech therapists & Psychologists.

•      Treatment is challenging, lengthy, costly & dependent on the skills & experience of a medical team.

CHEILITIS GLANDULARIS(actinic cheilitis)•      Inflammatory disorder of the lip.•      Progressive enlargement & eversion of the

lower labial mucosa.•      Resulting in obliteration of the mucosal-vermilion

interface.•      Chronic environmental exposure causes erosion,

ulceration & crusting.•      Susceptibility to actinic damage also increases,•      A potential predisposing factor for the

development of actinic cheilitis & Squamous Cell Carcinoma.

Etiology•      In Response to chronic irritation,

•      Lip enlargement due to inflammation, hyperemia, edema & fibrosis.

•      Long standing actinic exposure leads to surface keratosis, erosion & crusting,

•      Other chronic aggravating factors are self inflicted biting, excessive wetting, from compulsive licking, drying associated with mouth breathing.

Clinical Features•      Clinically distinct, deeply suppurative, chronic

inflammatory condition of the lower lip,•      Characterised by mucopurulent exudate from

ductal orifices of labial minor Salivary Glands (SGs),

•      Evidence of SG hyperplasia, Chronic progressive condition.

•      Patient complains of pain ,enlargement & loss of elasticity of the lips.

•      Initially asymptomatic swelling occurs with clear viscous secretion from ductal openings.

•      Periods of quiescence interrupted by transient painful episodes with suppurative discharge.

•      Burning discomfort or sensation of rawness associated with vermilion border,

•      Prolonged exposure to external environment results in desiccation & disruption of labial mucosa.

•      Although uncommon this lesion has heightened risk for developing Squamous Cell Carcinoma,

•      Dysplastic surface epithelial change is evident,•      Males > females,•      4th to 7th decade of life,•      Fair skinned individuals.

Classification3 types:•Simple :  Multiple painless papules with central depressions & dilated canals•Superficial (suppurative) :  Baelz’s disease  Painless indurated swelling with shallow ulcerations & crusting•Deep Suppurative :          Deep seated infection with formation of abscess, sinus tracts, pits & fistulas,& causes scarring.

Histologic Features•      Biopsy should include lip tissue (Surface

epithelium) & adequate depth to include submucosal Salivary Glands,

•      No consistent pathognomic features,•      Minor Salivary Glands may be normal or non

specific sialadenitis, it may include atrophy, Chronic inflammation,& interstitial fibrosis,

•      Bacterial infection may be present in cases with suppuration.

Differential Diagnosis

•      Actinic keratosis,•      Atopic dermatitis,•      Cheilitis Granulomatosa,•      Sarcoidosis ,•      Squamous Cell Carcinoma.

Treatment•      Antibiotic therapy for suppurative cases.

CHEILITIS GRANULOMATOSA(Miescher-Melkersson Rosenthal Syndrome)

•      Chronic swelling of lip due to granulomatous inflammation,

•      Miescher syndrome is exclusively for granulomatous changes in lip,

•      Melkersson Rosenthal is when cheilitis is associated with facial palsy & plicated tongue (Fissured Tongue) as a manifestation of Crohn’s disease.

Etiology•      Unknown,•      Genetic predisposition,•      Dietary or other antigens (contact Ag)

Clinical Features•      Non tender swelling,•      Enlargement of one or both lips,•      First episode of edema subsides within hours.•      Recurrent attacks, swelling persists, slowly

increases & becomes permanent.

•      Recurrence ranges from days to years.•      Early manifestations are sudden diffuse or

occasionally nodular swellings of lip or face (cheeks).

•      Less commonly involved are forehead & eyelids.•      Upper lip > lower lip.•      Soft firm nodular on palpation.•      Chronic – lip appears cracked ,fissured with

reddish brown discoloration & scaling.•      Gradually becomes painful & acquires consistency

of firm rubber,•      Regresses after some yrs,•      Regional lymph node enlargement,•      May be associated with fissured tongue,•      Decreased Salivary Gland secretion,•      Facial palsy (lower motor neuron type),•      Non caseating granulomas may form.

Differential Diagnosis•Insect bites,•Sarcoidosis,

Histologic Features•      Chronic inflammatory cell infiltrate,•      Peri & para vascular aggregations of lymphocytes,

plasma cells & histiocytes,•      Focal non caseating granuloma form with

epithelioid cells & Langhan’s type of giant cells.

Treatment

•      Patch test to be done, for reactions to metals food or other antigens.

•      If positive avoid particular allergen.•      Intralesional corticosteroids injections.•      Non steroidal anti inflammatory drugs.•      Mast cell stabilizers.•      Chlofazimine & Tetracycline.•      Surgery & radiation.

HEREDITARY INTESTINAL POLYPOSIS SYNDROME

Other names are:Peutz - Jeghers syndromeIntestinal hamartomatous polyps in association with mucocutaneous melanocytic macules.

•Autosomal dominantly inherited disorder,•Characterised by intestinal hamartomatous polyps in asso with mucocutaneous melanocytic macules.•15 fold increased risk of developing cancer of GI tract, including the pancreas & luminal organs, and of the female & male reproductive tracts & the lung,•It was named after Peutz, who noted a relationship b/n intestinal polyps & the mucocutaneous macules in 1921,

•And after Jeghers, who is credited with the definitive descriptive reports in 1944 & later in 1949.

Etiology•Germline mutation of the STK11 (serine threonine kinase 11) gene 

Clinical Features•      Described in all races,•      Males = females,•      Average age at diagnosis is men: 23, women: 26,•      Positive family history of Peutz-Jeghers syndrome

usually,•      Principal cause of morbidity: Intestinal location of

polyps (SI, colon, stomach)

Morbidity includes

•      Small intestinal obstruction•      Abdominal pain•      Hematochezia•      Prolapse of a colonic polyp•      These typically occur in the 2nd & 3rd decades of

life.

 Complaints include•      Repeated bouts of abdominal pain in patients

younger than 25yrs.•      Unexplained intestinal bleeding.•      Menstrual irregularities in females.

•      Cutaneous pigmentation (1-5 mm macules) of the perioral region crossing the vermilion border & perinasal areas is seen.

•      Pigmentation may be present on the fingers & toes,

•      On the dorsal aspects of hands & feet, and around anus & genitalia,

•      Pigmentation may fade after puberty,•      Mucosal pigmentation affects mainly  buccal

mucosa & rarely intestinal mucosa.

Histologic Features•      Excessive smooth muscle arborization throughout

the polyp,•      With the appearance of pseudoinvasion because

some of the epithelial cells usually from benign glands are surrounded by the smooth muscle.

Treatment•      Surgical treatment of the polyps.

DEVELOPMENTAL DISTURBANCES OF THE ORAL MUCOSA

 Fordyce’s Granules Focal Epithelial Hyperplasia

FORDYCE’S GRANULES (FORDYCE’S DISEASE)Developmental anomaly characterized by heterotrophic collections of sebaceous glands at various sites in the oral cavity.

EtiologyMay result from

 Inclusion into oral cavity of ectoderm having some potential of skin during development of maxillary & mandibular processes.

CLINICAL FEATURES: Small yellow spots  discretely separated or

forming large plaques. Often projects slightly above the surface. Bilaterally symmetrical :-

   1) on mucosa opposite Molars,   2) inner surfaces of lips,   3) retromolar region,   4) occasionally on tongue, gingiva, palate & frenum.

 Ectopic sebaceous glands occur besides oral cavity, in the esophagus, female & male genitalia, the palms & soles, the parotid gland, larynx & orbit,  

 Seen more in adults since sebaceous gland & hair do not reach maximal development until puberty

Histologic Features

 The heterotrophic collection is histologically same as that seen in skin normally but unassociated with hair follicles

 The glands are superficial, consistency of lobules, grouped around one or more ducts which open on surface of mucosa

 Ducts show “keratin plugging” at times.

Treatment•         Usually needs no treatment.•          It may rarely change to benign  sebaceous gland

oedema or develop into keratin filled pseudocysts.

FOCAL EPITHELIAL HYPERPLASIA (Heck’s disease)

•         Most contagious oral papillary lesion,•         HPV induced epithelial proliferation,•         HPV-13 & HPV-32,•         Endemic among children,•         Rarely residual lesions seen in adults,•         Hence it disappear on their own,•         May be an oral manifestation of AIDS,

Clinical features•         Different from other HPV infections,•         As it produces extreme acanthosis or hyperplasia

of the prickle cell layer of the epithelium with minimal production of surface projections or induction of C.T. proliferation,

•         Mucosa may be 8-10 times thicker than normal

•         Mainly in children,•         May occur in young & middle aged adults as well,•         No gender predilection,•         Sites :•         Labial, buccal & lingual mucosa,•         Gingiva & tonsillar area•         Lesions are broad based or slightly elevated so

as to present as well demarcated plaques,•         Frequently Papillary in nature but smooth

surfaced & flat topped lesions are more commonly seen

•         Papules & plaques are of normal color of mucosa, but may be pale or white as well,

•         Hyperplastic lesions are small, discrete & well demarcated but they frequently cluster so closely together that entire mucosal area takes on a cobblestone or fissured appearance.

Histologic Features•         Focal acanthosis of the oral epithelium,•         Thickened mucosa extends upward & not down

into underlying C.T.,•         Hence lesional rete ridges are at the same depth

as the adjacent normal rete ridges,•         Ridges are widened,often confluent & sometimes

club shaped,•         They are not long & thin as in psoriasis,

Lesion easily identified  b’coz of ,•         Lack of pronounced surface projections,•         Presence of mitosoid cells,&

•         Lack of CT cores in surface projections, when present

Treatment•         Conservative excisional biopsy,•         No further treatment required,•         Surgery for esthetic reasons might be done,•         Rare in adults as subside on its own after months

or yrs.

DEVELOPMENTAL DISTURBANCES OF THE GINGIVA

 Fibromatosis Gingivae Retrocuspid Papilla 

FIBROMATOSIS GINGIVAE (Elephantiasis gingivae, Hereditary gingival fibromatosis, Congenital macrogingivae)

•         Diffuse fibrous overgrowth of the gingival tissues,•         Benign idiopathic condition affecting both the

arches,•         Hereditary,•         Autosomal dominant,•         Males = Females,

Clinical Features•         Dense, diffuse, smooth or nodular overgrowth of

the gingival tissues of one or both  arches,

•         Usually appearing about the time of eruption of the permanent incisors,

•         However seen in even very young children, & rarely at birth.

•         The tissue not inflamed but is of normal or pale color,

•         Often so dense & firm it prevent the normal eruption,

•         Not painful, No tendency for hemorrhage,•         Crowns are nearly hidden  even after full eruption

with respect to the alveolar bone

Histologic Features•         Fibrous hyperplasia,•         Epithelium somewhat thickened with elongated

rete pegs,•         Bulk of tissue is dense fibrous C.T.,•         Bundles of collagen fibers are coarse & show few

interspersed fibroblasts & blood vessels.

Treatment:          When tooth eruption is affected, surgical removal

of the excessive tissue is indicated.          Surgery is also done for cosmetic purposes.          The fibrous tissue has a tendency to recur for

which only tooth extraction can prevent recurrences.

DEVELOPMENTAL DISTURBANCES OF THE TONGUE

  Aglossia & Microglossia Syndrome  Macroglossia  Ankyloglossia or Tongue-Tie  Cleft Tongue  Fissured Tongue  Median Rhomboid Glossitis  Benign Migratory Glossitis  Hairy  Tongue  Lingual Varices

AGLOSSIA & MICROGLOSSIA SYNDROME •         Rare congenital anamoly•         Almost always associated to malformations in the

extremities, like hands & feet, cleft palate & dental agenesis,

•         Aglossia Syndrome :     Microglossia + extreme glossoptosis•         Rudimentary small tongue,•         No gender predilection & no genetic implication•         As a consequence of the lack of muscular

stimulus b/n the alveolar arches, these do not develop transversely & the mandible does not grow in an anterior direction, producing a severe dentoskeletal malocclusion,

•         Fetal cell trauma in 1st few weeks of gestation may be responsible.

•         Associated Syndrome:       ORO-MANDIBULAR-LIMB Hypogenesis Syndrome

       Hypodactylia (Absence of fingers)

       Hypomelia     (Hypoplasia of a part or all of the limb)

       Hypoplasia of Mandible with Missing Lateral Incisors.

MACROGLOSSIA(Tongue hypertrophy, Prolapse of the tongue)

Congenital or AcquiredCongenital•         Vascular Malformations – Lymphangiomas /

Hemangiomas•         Muscular hypertrophy•         Down syndromeAcquired•         Metabolic/Endocrine                 –   Cretinism,

Diabetes, Hypothyroidism•         Inflammatory/Infection   -    Syphilis, TB,

Actinomycosis, Scurvy•         Systemic /medical            -    Myxedema,

Acromegaly, Neurofibromatosis•         Traumatic                          -    Surgery,

Haemorrhage, Radiation•         Neoplastic                         -    Carcinoma,

Plasmacytoma•         Infiltrative                         -    Amyloidosis,

Sarcoidosis

CLINICAL FEATURES Common in children,

 In growing stages, may lead to displacement of teeth or malocclusion,Crenation or scalloping at lateral borders of the tongue,

 Mandibular prognathism may be observed. If constantly protruding, may ulcerate or get

secondarily infected & necrosed. The surface appearance as well as histological

appearance depends on the underlying cause or condition.

 Complication  AIRWAY OBSTRUCTION

Associated Syndromes: Beckwith-Wiedemann Syndrome

(Visceromegaly, Gigantism, Neonatal hypoglycaemia, Omphalocele (Umblical Hernia))

TREATMENT :Surgical intervention for protection of the airway, mastication & deglutition mainly.

ANKYLOGLOSSIA(Tongue Tie)

It is a condition where the tongue mobility is restricted due to

1)   Short, Thick Lingual Frenum or2)   High attachment of the frenum to the tip of the

tongue or high muco-gingival attachment.

2 Types:    1) Complete        – Rarely seen; fusion of the tongue to the floor of the mouth.

          2) Partial    -  More commonly seen; termed as Tongue Tie.

Clinical Features Difficulty in speech specially certain consonants

like l,r,t,d,n,th,sh,z, Anterior open bite. Feeding difficulty in infants. May lead to periodontal problems if high muco-

gingival attachment is present. This may cause persistent gap b/n mandible

incisors.

Treatment Frenulectomy ( Frenectomy) In children- self correction

CLEFT TONGUE Condition due to lack of fusion of lateral lingual

swellings during development. Condition may be Complete or Partial, Seen as a deep groove in the midline of the dorsal

surface , Partial cleft results b’coz of incomplete merging &

failure of groove obliteration by underlying mesenchymal proliferation,

 Associated with ORO FACIAL DIGITAL SYNDROME Thick fibrous bands in the lower Anterior

Mucobuccal Fold eliminating the sulcus.

 Clefting of hypoplastic mandibleible

FISSURED TONGUE(Scrotal Tongue, Lingua Plicata, Plicated Tongue)

Characterised by grooves of varying depth along dorsal & lateral aspects of tongue which cause no adverse consequences other than being a collection site for food debris and colonization site for Candida Albicans.

ASSOCIATED SYNDROMES :MELKERSSON - ROSENTHAL SYNDROME,DOWN SYNDROMEBenign Migratory Glossitis (Rarely)

Clinical Features•         Benign condition,•         No racial predilection,•         Slightly male predilection,•         Diagnosed mainly in adulthood,•         Prominence increases with age, Condition Hereditary, trauma, or Vitamin

deficiency. Suspected etiology : a polygenic or autosomal

dominant mode•         Multiple grooves or fissures on the dorsum &

often to lateral borders, usually 2 to 6 mm in depth.

 Asymptomatic unless debris is entrapped within the fissure

 Either central fissure in the midline with radiating small fissures or numerous fissures on the dorsal surface which divide the tongue into “multiple islands”.

 May be interconnected, separating dorsum into several lobules, 2-6mm in depth

CLASSIFICATION  of the Pattern of Fissures: (Outdated concept)

1.    Transverse2.    Cerebriform3.    Foliaceous

Histologic Features Hyperplasia of rete ridges, Loss of keratin on the surface of filiform papillae Neutrophilic micro-abscesses within the epithelium, Increase in thickness of lamina propria, Mixed inflammatory infiltrate in the lamina propria.

TREATMENT No specific treatment, Extra care should be taken to clean the fissures

using gauze and tooth brush.

MEDIAN RHOMBOID GLOSSITIS(Central papillary atrophy of the Tongue)

 Congenital abnormality Tongue is formed by fusion of 2 lateral processes

in midline & fusing above a central structure from the 1st & 2nd branchial arches, the tuberculum impar,

 Failure of tuberculum impar to retract before the fusion of lateral halves of the tongue

 Rhomboid shaped smooth erythematous mucosa lacking in papillae or taste buds,

 A focal area of susceptibility to recurring or chronic atrophic candidiasis,

 So  posterior midline atrophic candidiasis Present  on dorsum just anterior to circumvallate

papillae, Long axis of red patch is in anteroposterior

direction,  Male : Female is 3:1,  < 2mm, & show a smooth, Flat surface,

sometimes lobulated, Devoid of filiform papillae. Usually erythematous but sometimes show excess

keratin production & thus show white surface change,

 Infected cases may also demonstrate a midline soft palate erythema in the area of routine contact with the underlying tongue involvement ; referred to as kissing lesion.

Histologic Features

 Smooth or nodular surface covered by atrophic stratified squamous epithelium overlying a fibrosed stroma with somewhat dilated capillaries,

 Loss of fungiform & filiform papillae, Elongated rete ridges which branch & anastomose

with premature keratin production in individual cells.

 Chronic inflammatory cell infiltrate within subepithelial & deeper fibrovascular tissues, 

 Silver staining for fungus will reveal candida hyphae & spores in the superficial layers of the epithelium,

 The tangential cutting of specimen may result in the artifactual appearance of cut reteridges as unconnected islands of stratified squamous epithelium ,leading to mistaken diagnosis of Well differentiated Squamous Cell Carcinoma,

 For this reason pt. should be treated with topical antifungal prior to biopsy.

Treatment Amphoterecin B or nystatin may be given

BENIGN MIGRATORY GLOSSITIS(Geographic Tongue / Wandering Rash of

Tongue)

•         Psoriasiform mucositis of dorsum of tongue,•         Like psoriasis- etiology unknown,•         More prominent during stress,

•         Frequency increases in persons with psoriasis.

Clinical features•         Characterised by constantly changing pattern of

serpiginous white lines surrounding areas of smooth, depapillated mucosa,

•         The lesion usually has a Central portion which shows inflammation and the borders are formed by a thin, yellowish, band or line.

•         Depapillation of filiform papillae occurs and the fungiform papillae appear as small, elevated dots, with loss of taste sensation.

•         The name given is due to desquamation in one location for a short time which heals and re-appears in another location; similar to migration

•         ECTOPIC GEOGRAPHIC TONGUE: This is a similar lesion which is seen on the buccal mucosa, gingiva, lips, palate (other than the tongue).

Histologic Features•         Biopsy to be taken from prominent part at the

periphery of a depapillated patch,•         Hyperkeratosis with Acanthosis is seen on the

margins.•         A thickened layer of keratin is infiltrated with

neutrophils,•         These inflammatory cells often produce small

microabcesses, Monro’s abscess, in the eratinized & spinous layers,

•         Rete ridges thin & elongated with only a thin layer of epithelium overlying CT papillae.

•         Similar to Psoriasis; thus it is classified under Psoriasiform lesion.

HAIRY TONGUE(Lingua nigra/villosa , Black hairy tongue)

 Not a developmental disturbance Defective desquamation of the filiform papillae, Marked accumulation of keratin in filiform

papillae  hair like appearance, It may appear brown, white, green, pink or any

color depending upon specific etiology & secondary factors.

Etiology•         Hypertrophy of filiform papillae on dorsum of

tongue usually due to lack of mechanical stimulation & debridement.

•         Heavy smokers•         Antibiotic therapy•         Poor oral hygiene•         Radiation therapy•         Overgrowth of fungi / bacteria•         Use of oxidizing mouthwashes•         Systemic diseases: e.g Anemia.

Clinical Features•         More in males,•         Associated with HIV infected pts,

•         Elongated filiform papilla which measures upto 15mm in length (normal 1mm) which gives a thick matted appearance

•         The colour depends on the pigment produced by the bacteria.

•         Rarely symptomatic unless infected with candida leading to glossopyrosis,

•         Tickling sensation in soft palate & oral pharynx during swallowing,

•         Gagging in more severe cases,•         Halitosis

Differential Diagnosis:-   Oral Hairy Leukoplakia

This is a lesion caused by Epstein-Barr virus which is seen in HIV positive patients and seen more on the lateral borders of the tongue.

(Can be differentiated by biopsy)-   Candidiasis/Leukoplakia/Oral Lichen Planus.

Histologic Features Elongated filiform papillae with mild hyperkeratosis, Accumulated debris intermingled with papillae &

candidal pseudohyphae.

Treatment:-         Eliminating predisposing factors (tobacco,

antibiotics, mouthwash, etc.) and/or brushing the tongue with a medium or heavy brush or tongue scraper.

-         Surgical removal if other methods are ineffective.

LINGUAL VARICES A varix is abnormally dilated, tortuous vein

mainly which is subjected to increased hydrostatic pressure but poorly supported by surrounding tissues.

 Rare in children and common in adults.

Clinical Features:Sublingual Varix in lingual ranine veins,

o   Most common•         Red or purple shot like clusters of vessels on the

ventral & lateral surface of the tongue & in floor of the mouth,

•         AsymptomaticSolitary Varices:

•         Also in upper & lower lip, buccal mucosa & buccal commissures.

•         Early varicosity indicates premature aging.

Histological Features:•         Dilated veins•         Lines of Zahn – Concentric layered zones of

platelets & erythrocytes (Secondary thrombosis)•         May undergo recanalisation•         May lead to phlebolith.

LINGUAL THYROID NODULE:

-        During the 3rd to 4th week of IntraUterine life, the thyroid gland begins to form from the floor of the pharyngeal gut as an endodermal invagination.

-        The tongue forms at the same time and is associated with the thyroid gland by the thyroglossal duct which later becomes the lingual remnant termed as Foramen Caecum.

-        If the primitive gland does not “migrate” to it’s normal position, the thyroid tissue remains on the surface of the tongue.

Etiology:-         The nodule enlarges during functional insufficiency of the

thyroid gland specially due to goiter.

Clinical features:-   Seen more in females during the adolescent

age, and may be due to hormonal influence.-   The enlargement is benign and

asymptomatic.-   May lead to dysphagia, dyspnoea, dysphonia.-   Seen near the base of the tongue as a

smooth nodular mass around 2-3cm in diameter.-   It may appear vascular and may be painful.

Histologic Features:-         Resembles normal thyroid tissue or the

fetal/embryonal type or goiter or colloid degeneration.

-         Accessory salivary glands should be ruled out in the same location.

-         Both may give rise to adenomas or adenocarcinomas.

DEVELOPMENTAL DISTURBANCES OF THE SALIVARY GLANDS

1.    Aplasia2.    Xerostomia3.    Hyperplasia of Palatal Glands4.    Atresia5.    Aberrancy6.    Developmental Lingual Mandibular Salivary

Gland Depression7.    Anterior Lingual Depression

APLASIA     (Agenesis) •         Absence of Salivary Glands, Any 1 or group of

Salivary Glands,•         Uni / Bilateral,•         It’s a diagnosis of exclusion,•         CT Scan / MRI shows absence of glands &

replacement by fat & fibrous tissue.•         Scintiscanning with a radioisotope will confirm the

diagnosis,•         Absence of salivary duct orifice / papilla –clue to

diagnosis.•         Isolated or associated with –

 Hemifacial microsomia LADD Syndrome Mandibulofacial Dysostosis

LADD SyndromeL >

•         Lacrimal apparatus shows occlusion of the lacrimal puncta,

•         nasolacrimal duct obstruction with overflow of tears (epiphora),

•         lacrimal sac inflammation (dacrocystitis) &•         lacrimal gland aplasia

A >•         Auricles deformed with Cup – Shaped   appearance

with some Hearing Loss.D >•         Dental – Peg Shaped Teeth, Hypodontia &

Enamel Hypoplasia, SG Agenesis & XerostomiaD >•         Digital Deformities – Deviation  of the fingers

medially or laterally

Clinical Features:1.    Xerostomia,2.    Increased caries, Rampant caries or similar to

radiation caries.3.    Burning sensation,4.    Oral Infections,5.    Taste Aberrations,6.    Difficulty with denture retention.

7.    Cracking, Fissuring of lips and corner of the mouth seen.

8.    Dry, smooth oral mucosa which makes it appear pebbly.

Treatment•         Supportive,•         Aim at relieving Xerostomia & its effects,•         Salivary Substitutes,•         Mouthwashes•         Comprehensive Dental Care,•         Flouride Therapy,•         Good Oral Hygiene.

XEROSTOMIA(Dry Mouth)•         Not a disease rather a symptom of Salivary Gland

Dysfuntion.•         Have serious negative effects on patient’s quality

of life•         Affecting dietary habits, nutritional status,

Speech, Taste, Tolerance to dental prosthesis, & Increased susceptibility to dental caries

EtiologyTemporary•         Psychological•         Duct Calculi•         Sialoadenitis

•         Drug Therapy      (Zyban – Anti smoking)Permanent1.    SG Aplasia2.    Sjogrens Syndrome3.    Radiotherapy4.    Surgical Desalivation5.    Other systemic conditions like DM, Parkinson’s

disease, Cystic fibrosis & Sarcoidosis

C/Fs•         Assess if Dryness is continual or intermittent,•         Accompanied by pain or swelling,•         Uni / Bilateral,•         Any relative history of anxiety, stress or

depression, systemic disorder, irradiation, trauma, surgery or medication.

•         Patients occupation, diet & domestic situation is relevant.

•         If Duct Calculi – Unilateral dryness with pain or discomfort & swelling in affected gland on stimulation.

•         If Sjogren’s Syndrome – Bilateral swelling, often constant, accompanied by Dry Eyes & Rheumatoid Arthritis & Lymph Node Enlargement.

•         In Postmenopausal women a more typical case of non specific xerostomia is seen.

•         Atrophy of oral mucosa due to hormonal changes & a mild chronic candidiasis & reduced

salivary flow due to age & depression (on medication), marginally iron deficiency anemia.

•         Degree of severity varies,•         Dry or burning sensation but mucosa normal in

appearance, complete lack of saliva,•         Chronic xerostomia predisposes to rampant

caries & frequent loss of teeth,•         Difficulty with artificial dentures.•         Mucosa – Dry, atrophic sometimes inflammed or

more often pale & translucent,•         Tongue – Atrophy  of papilla, inflammation,

fissuring or cracking & severe denudation, soreness, burning & pain of Oral Mucosa & tongue.

Treatment•         Eliminate etiology•         Promote salivary stimulation by chewing sugar

free chew gums•         Salivary substitutes.

ATRESIA•         Congenital occlusion or absence of one or more

of the major salivary gland ducts,•         Rare,•         Result in formation of a retention cyst, or•         Produce relatively severe xerostomia.

ABERRANCY•         When Salivary Gland are present in a location

which is farther from their normal situation,

•         Difficult to define due to widespread distribution of accessory SGs,

•         No significant clinical relevance,•         Occasionally found within the body of the

mandible.

LINGUAL MANDIBULAR SALIVARY GLAND DEPRESSION Other names are :1.    Static bone cyst/ cavity or defect of the

mandible,2.    Lingual mandibular bone cavity,,3.    Latent bone cyst,4.    Stafne cyst or defect.

•         Recognised by Stafne in 1942.•         Unusual form of aberrant Salivary Gland tissue,•         Wherein a developmental inclusion of glandular

tissue is found within or adjacent to the lingual surface of the body of mandible, within a deep & well circumscribed depression,

•         Males > Females,•         Considered as developmental rather than

pathologic defect.

R/F•         It appears as an ovoid radiolucency with a

sclerotic border located b/n the inferior alveolar canal & the inferior border of the mandible in the region of the 2nd or 3rd  molars.

•         It may be unilateral or bilateral

•         Differentiated from traumatic bone cyst by location, which almost invariably lies superior to the inferior alveolar canal.

H/F•         Normal Submandibular gland tissue.•         The cyst may contain muscle, fat, blood vessels,

connective tissue or lymphoid  tissue.

ANTERIOR LINGUAL DEPRESSION

•         Similar to Stafne cyst,•         An asymptomatic round or ovoid radiolucency

may occur in the anterior segment of the mandible,•         Poorly circumscribed, between Central Incisor &

1st Premolar area,•         Development of a true central Salivary Gland

neoplasm may occur rarely.

Treatment:

No treatment is necessary as the lesion is asymptomatic.

DEVELOPMENTAL DISTURBANCES OF THE TEETH

 Size of teeth Shape of teeth Number of teeth Structure of teeth Growth / Eruption of teeth

DEVELOPMENTAL DISTURBANCES IN THE SIZE OF THE TEETH

 Microdontia Macrodontia

MICRODONTIA •         Teeth smaller than normal

TRUE GENERALIZED All teeth smaller Teeth well formed, merely small May be seen in hormonal disturbances like Pituitary Dwarfism   RELATIVE GENERALIZED Teeth Normal,  slightly small More of illusion as Jaws are larger

Involving one tooth commonly-   Maxillary Lateral Incisor & 3rd Molars followed

by Supernumerary teeth-         Common form, ‘Peg Laterals’  of Maxillary Lateral

Incisor,•         Mesial & distal sides taper incisally forming a peg or cone shaped crown,•         Root is also shorter here.

MACRODONTIA .    Teeth are larger than normal.    Can be of the following types:1)   True Generalized (Seen in Pituitary Gigantism)2)   Relative Generalized (Small jaw, normal tooth)3)   Single tooth.

DEVELOPMENTAL DISTURBANCES IN SHAPE OF TEETH

 Gemination Fusion Concrescence Dilaceration Talon cusp Dens in dente Dens evaginatus Taurodontism Supernumerary rootsGEMINATION

 Division of tooth germ by an invagination  incomplete formation of 2 teeth,

 Formation of completely or incompletely separated crowns with 1 root & 1 canal,

 Permanent and deciduous dentition Hereditary tendency, Twinning formation of 2 equivalent teeth,1 normal

& 1 supernumerary tooth,

FUSION  Union of two normally separated tooth germs, Depending upon the stage of development it can

be  Complete  Incomplete 

 If before calcification a single large tooth is formed, If after crown formation then fusion of roots only. Dentin is always confluent in cases of true fusion, More common in deciduous though affects both the

dentition, Possible dental problems are Spacing &

Periodontal Problems.

In the case of gemination, there is an extra tooth present in the dentition, while one tooth less in fusion.

CONCRESCENCE  Form of Fusion which occurs after root formation, Teeth united by cementum only, Suspected Etiology :1)Traumatic injury

 Crowding Of Teeth with resorption of interdental bone,

 2 teeth are involved generally, A rare case of 3 has been also seen, Extraction with caution 

DILACERATION  Angulation/sharp bend/curve in root or crown of a

formed tooth, Trauma during tooth formation or secondary to

adjacent cyst, tumour, odontomes  calcified position of tooth changed  so remaining tooth form at an angle,

 Often following trauma to deciduous predecessor where tooth is driven apically into the jaws,

•         Mainly seen in root, and specially in permanent Maxillary or Mandibular Central Incisors.

•         Bend may occur anywhere along the length of the tooth, cervical, middle or at the apex depending upon the amount of root formed at the time of injury,

•         Extraction & RCT with caution.

TALON CUSP

 Resembles Eagle’s talon, Lingual projection from cingulum of maxillary or

mandibular permanent incisor Cusp blends with lingual surface but there is a

deep developmental groove,

 Composed of normal enamel, dentin & a horn of pulp tissue,

 Problems :Caries, Esthetic, Occlusal Accommodation

o       Associated with:-   STURGE-WEBER SYNDROME

     -   RUBINSTEIN - TAYBI SYNDROME Developmental retardation, Broad thumbs & great toes, Characteristic facial features, Delayed or incomplete descent of testes in males Stature, Head Circumference & Bone Age below 50th percentile.o        May be associated with some Somatic & Odontogenic anomalies.

DENS IN DENTE (Dens Invaginatus/ dilated composite odontome)

 Dens in dente - tooth within a tooth  Misnomer Called an Odontome as the imagination causes

dilation which disturbs the formation of the tooth.

 Invagination in crown surface before calcification Suspected Etiology : Increased localized external pressure, Focal growth retardation, & Focal growth stimulation in certain areas of the tooth bud,

 Frequently seen in permanent Maxillary Lateral Incisor,

 When Maxillary Central Incisors involved, it is frequently bilateral.

 Rarely posteriors & roots of teeth also involved. This radicular invagination usually results from an

infolding of Hertwig ’s sheath & takes its origin within the root after development is complete,

 Appears as an accentuation of the lingual pit, The Radiographic finding is usually a PEAR

SHAPED INVAGINATION of enamel & dentin. Mild form—deep invaginations in the lingual pit

area, which  may not be clinically evident, pear shaped invagination of enamel and dentin with a narrow constriction at the opening on the surface of the tooth & closely approx. pulp in its depth.

 Food debris  caries & infection, Severe form – invagination till root apex, bizzare

R/F appearance, Restore the tooth prophylactically to prevent caries

& infection as the pit causes lodging of food debris.

DENS EVAGINATUS (Leong’s premolar/Occlusal Enamel Pearl) Accessory cusp, or Enamel globule on occlusal surface b/n buccal &

lingual cusps of premolars which may contain only enamel or dentin & pulp at times.

 Unilateral /Bilateral,

 Occurrence : Mongoloid Ancestry, Chinese, Japanese, Filipinos, Eskimos & American Indians,

 Disadvantages :       a) Incomplete eruption                                      b) Teeth displacement                             c)  Pulp exposure

Pathogenesis : Proliferation & evagination of an area of inner

enamel epithelium & subjacent odontogenic mesenchyme into the dental organ during early tooth development.

 So considered as Antithesis of dens invaginatus,

TAURODONTISM •         By Sir Arthur Keith in 1913,•         Tooth is enlarged at expense of the roots, due to

failure of H.E.R.S to invaginate in the horizontal level

•         Means ‘bull –like’ teeth,Classified as :

  Mild - Hypotaurodont  Moderate - Mesotaurodont  Severe – Hypertaurodont – (furcation near the

apices)Possible causes :

1)   Specialized or retrograde character,2)   Primitive pattern,3)   Mendelian recessive trait,4)   Atavistic feature,

5)   A mutation resulting from odontoblastic deficiency during dentinogenesis of the roots,

Clinical Features•         Deciduous / Permanent Dentition,•         Almost invariably molars are involved,•         Single or sometime many teeth are involved,•         Unilateral / Bilateral,•         Any quadrant combination may be involved.

R/F•         Teeth are rectangular in shape rather than taper

towards roots,•         Pulp chambers extremely enlarged,•         Greater apico-occlusal height than normal,•         Pulp lacks usual constriction at cervical part,•         Roots extremely short,•         Furcation may be just a few mm above apices,

Associated Syndromes:-                     Klinefelter’s Syndrome-                     Down Syndrome-                     A variant of Amelogenesis Imperfecta.

SUPERNUMERARY ROOTS •         Very  common,•         May  involve  any  teeth,•         Normal  single-rooted  teeth  show 2 or

many  roots, like  mandible canine & premolars,•         Even  molars  also  show  extra  roots,

specially  3rd  molars,

•         The supernumerary roots are usually smaller than the normal.R/Fs 

- Help in diagnosis.- At times roots may be superimposed on

other roots.Surgical Importance:- May be retained in the alveolus after extraction which may lead to infection.

- Failure of endodontic treatment if root is missed out.

DEVELOPMENTAL DISTURBANCES IN NUMBER OF TEETH  Anodontia Supernumerary teeth Pre decidous dentition Post permanent dentition

ANODONTIA – (ABSENCE OF TEETH)

2 types :1) True, &2) False/Pseudo    True further divided into     Total – all teeth missing,     Partial – some teeth missing,

True Total Anodontia Congenital absence of teeth, All teeth are missing, True failure of odontogenesis,  Rare, Deciduous / Permanent, Frequently associated with Hereditary

Ectodermal Dysplasia,

True Partial Anodontia Hypodontia (lack of 1 or more teeth)

or Oligodontia (Lack of more than six teeth). One or more teeth, Common condition, Commonly  involved teeth are 3rd Molars, Maxillary

Lateral Incisor, Maxillary or Mandibular 2nd Premolar,

 Sometimes bilateral 3rd molar missing evolutionary trend Hereditary Ectodermal Dysplasia

•         Congenitally missing deciduous teeth are uncommon,

•         If missing : Maxillary LI > Mandible LI > mandible C,

•         X-ray radiation of face at an early age teeth of one or both quadrant on same side missing,

•         Tooth buds are extremely sensitive to X-Ray radiation & may be destroyed completely by even small dosages,

•         Teeth already forming & partially calcified may be stunted by x- rays,

•         Induced or false anodontia – due to extraction.

•         Pseudo-anodontia –   Multiple unerupted teeth

Other Associated Syndromes:     Turner Syndrome     Down Syndrome     Gorlin Syndrome

SUPERNUMERARY TEETH

•         Closely resemble the teeth of the group to which it belongs like molars, anteriors etc,

•         It is believed that these develop from•         3rd tooth bud arising from the dental lamina near

the permanent tooth bud, or•         From splitting of permanent bud itself,•         Hyperactivity theory :these are formed as a result

of local, independent, conditioned hyperactivity of dental lamina.

•         Hereditary in some cases•         Etiology unknown,•         Deciduous & Permanent Dentition,•         Maxilla / Mandible,•         Single / Multiple,•         Uni / Bilateral,•         Erupted or Impacted,

•         Multiple supernumerary teeth are seen to be associated with :

      Cleft Lip & Palate,      Cleidocranial Dysplasia,      Gardner Syndrome,•         Males > Females = 2 : 1 in case of permanent

dentition,

Classification•         Acc to Morphology & Location :•         Deciduous : Normal or Conical•         Permanent : Conical Tuberculate Supplemental Odontome

Types:MESIODENS:  Between Central Incisors                   May be Paired or Single                   Erupted or Impacted                   Conical with short roots.

DISTOMOLARS/DISTODENS:                   Usually observed as 4th Molar placed

Distally to the 3rd molar.                   Maxillary jaw is affected more

commonly.

PARAMOLARS:

                   Seen in between molars – Interproximally, buccally or lingually.

Associated Syndromes:-   CleidoCranial dysplasia-   Sturge-Weber Syndrome-   Gardner’s Syndrome     1) Multiple Polyposis of the Large Intestine     2) Osteomas of the Bones, including long

bones, skull & jaws     3) Multiple Epidermoid or Sebaceous Cysts of

the skin, scalp & back     4) Occasionally Desmoid tumors,     5) Impacted multiple Supernumerary &

Permanent Teeth.

PREDECIDUOUS DENTITION (premature eruption, natal teeth, neonatal teeth)

•         Structures which appear to be erupted teeth seen when an infant is born,

•         Present usually in mandible incisor area,•         These thought to arise either from an accessory

bud, or from bud of an accessory dental lamina.•         Natal Teeth : teeth erupted by the time of

birth.Prematurely erupted true deciduous teeth not to

be extracted•         NeoNatal Teeth: Erupts within 30 days.

Hornified epithelial structures without roots, occuring on the gingiva over the crest of the ridge, which may be easily removed.

•         Some believe these structures present are only the Dental Lamina Cyst of the Newborn,

•         This cyst commonly project above the crest of the ridge, is white in color & is packed within keratin, so that it appears ‘hornified’ & can be easily removed.

POST PERMANENT DENTITION

•         Eruption of several teeth after extraction of all the teeth,

•         Seen pert after the insertion of a full denture,•         Classified as multiple supernumerary unerupted

teeth, increased they probably develop from a bud of the dental lamina beyond the permanent tooth germ,

•         Mostly it is due to delayed eruption of retained or embedded teeth.

DEVELOPMENTAL DISTURBANCES IN THE STRUCTURE OF THE TEETH

Involving Enamel•         Amelogenesis Imperfecta (Hereditary Enamel

Hypoplasia)

•         Environmental Enamel Hypoplasia

Involving Dentin•         Dentinogenesis Imperfecta•         Dentin Dysplasia•         Regional Odontodysplasia•         Dentin Hypocalcification

ENAMEL HYPOPLASIA  Incomplete or defective formation of the organic

enamel matrix of teeth. 2 types :1.    Hereditary (Amelogenesis Imperfecta),2.    Environmental Enamel Hypoplasia.       In hereditary type both the dentitions are

affected.

AMELOGENESIS IMPERFECTA (Hereditary enamel dysplasia, Hereditary brown

opalescent teeth) 

•         Structural defect of tooth enamel,•         Entirely ectodermal defect,•         Defective Gene – locus DXS85 at Xp22.•         Site for amelogenin – principal protein in

developing enamel.

Enamel development occurs in 3 stages :1.    Formative       – deposition of organic matrix2.    Calcification – matrix is mineralized3.    Maturation     – crystals enlarge & mature

Classification - Depending on the mode of inheritance, as well as the clinical appearance of the enamel all are further subdivided.By Witkop & Sauk – Clinical, Histologic & Genetic Criteria. (1989)Type I                 Hypoplastic IA     Hypoplastic, Pitted Autosomal DominantIB     Hypoplastic, Local Autosomal DominantIC     Hypoplastic, Local Autosomal RecessiveID     Hypoplastic, Smooth, Autosomal DominantIE      Hypoplastic, Smooth X linked DominantIF      Hypoplastic, rough Autosomal DominantIG     Enamel Agenesis, Autosomal recessive.

Type II               Hypomaturative IIA    Hypomaturation, pigmented Autosomal recessive

IIB              Hypomaturation, X-linked recessiveIIC              Snow Capped teeth, Autosomal

dominant

Type III           Hypocalcified IIIA            Autosomal dominantIIIB            Autosomal recessive

Type IV     Hypomaturation-Hypoplastic with Taurodontism

IVA            Hypomaturation-Hypoplastic with taurodontism, Autosomal dominant

IVB            Hypoplastic-Hypomaturation with taurodontism, Autosomal dominant

Clinical Features:•         HP         -  Enamel not formed to full normal

thickness on newly erupted developing teeth,•         HC -   Enamel is soft,

•         HM-      Enamel can be pierced by an explorer point under firm pressure & can be lost by chipping away from underlying normal appearing dentin.

•         All teeth of both dentition are affected to some degree,

•         May or may not show discoloration,•         Discoloration if present may vary from yellow to

dark brown,•         Enamel may have a chalky texture or even a

cheesy consistency or may be hard.•         Sometimes enamel is smooth or it may have

numerous parallel vertical wrinkles or grooves.•         May be chipped or show depressions in the base

of which dentin may be exposed,•         Contact points are often open,•         Occlusal surface & incisal edges are frequently

severely abraded.

R/Fs Overall shape may or may not be normal,

depending upon the amount of enamel present on the tooth & the amount of occlusal & incisal wear,

 Enamel may appear totally absent,

 If present, very thin layer mainly over cusp tips & interproximal areas,

 In some cases it resembles dentin due to hypocalcification.

H/Fs HP – defect in matrix formation or total absence of

matrix, disturbance in the differentiation or viability of ameloblasts.

 HC – defects of matrix structure & of mineral deposition,

 HM – alterations in enamel rods & rod sheath structure. 

HYPOPLASTIC : Inadequate deposition of matrix Enamel is not fully developed in full thickness Pitted variety Enamel b/w pitscalcified and coloured Localized linear depression

HYPOCALCIFIED :•         Enamel is soft and easily lost•         Eruption-yellow brown to orange but becomes

brown to black•         Coronal enamel is removed over the years

HYPOMATURITIVE :•         Defective maturation therefore chips of easily

from dentin.       Brown-yellow, mottled appearance

•         Can be pierced with a dental explorer point.•         Snow capped patternszone of white enamel on

incisal and occlusal surfaces

ENVIRONMENTAL ENAMEL HYPOPLASIA  Either dentition may be affected, Even a single tooth may be involved, Usually both enamel & dentin are affected though

to varying degree.

Different factors capable of producing injury to ameloblasts may be:

 Nutritional deficiency Exanthematous diseases Congenital syphilis Hypocalcaemia Birth injury, premature birth, Rh hemolytic disease Local infection/trauma Ingestion of chemicals Idiopathic causes

C/Fs Mild cases : only a few small grooves, pits or

fissures on enamel surface. Severe cases : rows of deep pits arranged

horizontally across the surface, May be a single row of such pits or several rows,

indicating a series of injuries.

 Hypoplasia occurs only if the injury occurs during the time, teeth are developing or during formative stage of enamel development

 Once the enamel is calcified, no such defect is produced,

 So with the chronological knowledge of development of deciduous & permanent teeth time of injury can be identified from the location of the defect on the teeth.

Nutritional deficiency & Exanthematous diseases : Rickets – most common cause, Def of Vit A & C, Measles, Chicken Pox & Scarlet Fever, Ameloblasts are most sensitive group of cells in the

body in terms of metabolic function. Hypoplasia is of pitting variety, pits tend to stain so

teeth may be very unsightly. Teeth involved are the once which form within 1st

yr after birth like CI & LI, cuspids & 1st molars. Premolars, 2nd  & 3rd molars are seldom affected,

since their formation does not begin until about age of 3yrs or later.

Congenital Syphilis : Not of pitting type, characteristic & pathognomic, Hutchinson’s Teeth – Maxillary & Mandible

Permanent Incisors,

 Maxillary CI – Screw driver shaped, mesial & distal surfaces of crowns are tapering toward the incisal edges & incisal edges usually notched.

 Mandibular CI & LI are similarly involved though Maxillary LI may be unaffected.

 Tapering or notching is b’coz of absence of central tubercle or calcified centre.

 Mulberry / Moon’s / Fournier’s Molars – crowns of 1st molars are irregular,

 Enamel of occlusal surface & occlusal 3rd of tooth appears to be arranged in an agglomerate mass of globules rather than in well formed cusps,

 Crown narrower at occlusal than cervical. Not all pts. with congenital syphilis have dental

findings & vice – versa.

Hypocalcaemia: Serum calcium level fall as low as 6-8 mg/100 ml,

(normal : 9-11mg/100ml), This level produces hypoplasia in developing teeth, It’s of pitting type similar to in case of nutritional def

& exanthematous dis type.

Birth Injury, Premature Birth & Rh Hemolytic disease :

 Neonatal line or ring present in deciduous teeth & 1st perm molars may be thought as Enamel Hypoplasia.

 Produced in dentin as well,

 Disturbance indicative of the trauma or change of environment at the time of birth.

 In traumatic births the formation of enamel may cease at this time.

 E hypo more common in prematurely born children, In children suffering from Rh hemolytic disease, Seen in both pre & postnatal enamel, In some cases GI dist or some other illness in

mother may also be responsible.

Local infection or trauma : Only a single tooth is involved, Permanent maxillary incisor or maxillary or

mandibular premolar, Severity depends upon :1.    Severity of infection,2.    Degree of tissue involvement,3.    Stage of permanent tooth formation during

infection.

 Any degree of hypoplasia – from mild, brownish discoloration of the enamel to a severe pitting & irregularity of crown,

 These referred to as ‘Turner’s Teeth’, & condition as ‘Turner’s Hypoplasia’,

 Deciduous caries – bacterial infection – periapical tissue of deciduous – disturb Ameloblastic layer of developing perm tooth – hypoplastic crown

 Another type is trauma to deciduous teeth specially when tooth is driven into alveolus & has disturbed

permanent tooth bud – yellowish or brownish stain or pigmentation of enamel usually on labial surface or as true hypoplastic pitting defect or deformity,

 Disturbance in either matrix formation or in calcification can occur, depending upon the stage of tooth formation at the time of injury.

Mottled Enamel : By GV Black & Frederick S McKay in 1916 Ingestion of fluoride containing drinking water

during tooth formation, Severity increases with increase of fluoride

content  > 1ppm, Disturbance to ameloblasts during formative stage Enamel matrix is defective or deficient, With higher levels of fluorine – interference with the

calcification process of the matrix. Occasionally white flecking or spotting of enamel Mild changes – White  opaque areas involving

more of tooth surface area, Moderate & severe changes showing pitting &

brownish staining of the surface, Corroded appearance of the teeth, Tendency for wear & even fractures. In Europe, Africa, Asia & United States, Treatment – Bleaching. 

DENTINOGENESIS IMPERFECTA

 Autosomal dominant condition, Gene maps to chromosome no 4 It encodes protein dentinsialophosphoprotein

(DSPP) It constitutes about 50% of non collagenous

component of dentin matrix. Both deciduous & permanent teeth, Gray to yellowish brown, Broad crown with constriction of the cervical area

resulting in a tulip shape, R/Fs teeth appear solid, lacking pulp chambers &

root canals.- - enamel easily broken leading to exposure of

dentin that undergoes accelerated attrition.

CLASSIFICATION – By SheildsTYPE I May be associated with Osteogenesis ImperfectaTYPE II Autosomal dominant trait, Not associated with

Osteogenesis ImperfectaTYPE III Brandywine type Dentinogenesis Imperfecta

Seen as a racial isolate in Maryland, USA.

Revised classification DI Type I –  without Osteogenesis imperfecta

(opalascent dentin), DI Type II – Bradywine type / Shell Teeth.

DENTINOGENESIS IMPERFECTA - TYPE I(Capdepont teeth, Opalescent dentin)

 Mutation in the DSPP gene (locus 4q21.3 encoding dentin phosphoprotein & dentin sialoprotein)

 Distinct from Osteogenesis Imperfecta & affects only the teeth,

 No increased frequency of bone fractures. Teeth are blue – gray or amber brown &

opalescent.On R/Fs – Bulbous crowns, Roots narrower than normal, Pulp chamber & root canals narrower than normal

or completely obliterated, Enamel split from dentin under occlusal stress.

DENTINOGENESIS IMPERFECTA - TYPE II(Brandywine type)

 Found in Bradywine tri-racial isolate in southern Maryland,

 This type is not associated with Osteogenesis Imperfecta.

 Separate mutation from Dentinogenesis Imperfecta Type I

 Crowns of deciduous & permanent teeth wear rapidly after eruption,

 Multiple pulp exposures may occur, Dentin is amber & smooth.

R/Fs – Deciduous dentition show very large pulp

chambers & root canals during the 1st few yrs though may reduce in size with age.

 Permanent teeth have pulpal spaces that are either smaller than normal or completely obliterated.

 Classic ‘ Shell Teeth’   appearance.

H/Fs Purely a mesodermal disturbance, Appearance of enamel is normal except its peculiar

shade due to dentinal disturbance Dentin – irregular tubules with large areas of

uncalcified matrix, Tubules larger in diameter so less numerous in

given area, In some areas complete absence of tubules Cellular inclusions like odontoblasts are common, Pulp chamber almost obliterated by continuous

deposition of dentin, Odontoblasts have limited ability to form well

organized matrix, they degenerate rapidly becoming entrapped in the matrix.

Chemical & Physical Features

 DI 1 – water content is greatly increased (60 % above normal),

 Inorganic content less than normal, So density, X-ray resorption & hardness of dentin

is low, Micro-hardness of dentin here closely resembles

that of cementum which explains rapid wear,

Treatment

 It is directed at preventing loss of enamel & subsequent loss of dentin through attrition,

 Cast metal crowns on posterior teeth. Jacket crowns on anterior teeth. Care should be taken as roots of these teeth too

are easily fractured.

DENTIN DYSPLASIA(Rootless teeth)•         Rare disturbance of dentin characterized by

normal enamel but atypical dentin formation with abnormal pulp pathology.

•         1st reported by Ballschmiede – spontaneous exfoliation of multiple teeth in 7 children of 1 family – Rootless  Teeth.

•         1st concise description – Rushton – Dentin Dysplasia

 Etiology – Hereditary Autosomal dominant

 Shields Classification – Type  I – Dentin Dysplasia

 Type II – Anomalous Dysplasia of Dentin Witkop suggested classification as – Type  I – Radicular Dentin Dysplasia Type II – Coronal Dentin Dysplasia

C/FsTYPE I - Radicular Both dentitions affected Teeth clinically appear normal in morphology &

color Occasionally slight amber translucency, Generally normal eruption pattern, though delayed

eruption is also noticed Exhibit extreme mobility & exfoliate prematurely or

even after minor trauma.

TYPE II - Coronal Both dentitions affected, but Involvement of both dentitions is different clinically,

r/f & h/p. Deciduous if affected yellow, brown or bluish –

gray  opalescent appearance Permanent appears normal clinically.  R/FsType I Roots are short, blunt, conical or similarly

malformed, In deciduous – pulp chambers & root canals are

usually completely obliterated,

 In permanent – a crescent shaped pulpal remnant may still be seen in chamber,

 Obliteration here occurs pre-eruptively Periapical radiolucency representing granulomas,

cysts or abscesses involving apparently otherwise intact teeth.   

Type II Deciduous – pulp chambers obliterated, This does not occur before eruption, Permanent – abnormally large pulp chamber in the

coronal portion“ THISTLE TUBE”  & within this area radiopaque foci resembling pulp stones may be found.

H/PType I Coronal dentin normal, Pulp is obliterated by calcified tubular dentin,

osteodentin & fused denticles, Normal dentinal tubule formation is blocked so new

dentin forms around obstacles & appears as “Lava flowing around boulders”

 This “cascades of dentin” results from repetitive attempts to form root structure

Type II Deciduous teeth exhibit amorphous and atubular

dentin in radicular portion, coronal dentin is relatively normal.

 In permanent teeth also relatively normal coronal dentin but the pulp has multiple pulp stones or denticles.

REGIONAL ODONTODYSPLASIA(Ghost teeth)

Other names are :1.    Odontodysplasia2.    Odontogenic Dysplasia3.    Odontogenesis Imperfecta

Suspected etiology•         Somatic mutation•         Latent virus residing in the odontogenic

epithelium which become active during the development of tooth,

•         Local vascular defects ( due to associated with vascular facial nevi)

C/Fs•         One or more teeth in a localized area are affected

in an unusual manner,•         Maxilla > Mandible,•         Teeth frequently involved are : Maxillary permanent CI, LI & Cuspids, Mandible- same teeth are affected, Deciduous as well as permanent teeth. Delay or total failure in eruption, Irregular  shape with defective mineralization

R/Fs•         Marked reduction in radiodensity so teeth assume

a ‘ghost’appearance.•         Both enamel & dentin appear very thin & pulp

chamber is exceedingly large,•         Enamel layer is often not evident.

H/Fs•         Reduction in amount of dentin,•         Widening of predentin layer,•         Large areas of Inter Globular Dentin,•         Reduced Enamel Epithelium around non-erupted

tooth shows many irregular calcified bodies.

Treatment:.    Poor aesthetic appearance..    Extraction with restoration by prosthetic appliances.

DENTIN HYPOCALCIFICATION

 Failure of union of globules of dentin, leaving interglobular areas of uncalcified matrix.

 Hypocalcified dentin is softer than well formed dentin

 The causes are similar to environmental enamel hypoplasia e.g Parathyroid deficiency.

DEVELOPMENTAL DISTURBANCES IN THE GROWTH (ERUPTION) OF TEETH

•         Premature Eruption•         Eruption Sequestrum•         Delayed Eruption•         Multiple Unerupted Teeth•         Embedded & Impacted Teeth•         Ankylosed Deciduous Teeth

PREMATURE     ERUPTION •         Deciduous teeth seen into the oral cavity at the

time of birth – Natal Teeth.•         Teeth erupting within 1st 30 days of life –

Neonatal Teeth.•         Usually 1 or 2 teeth erupt early,•         Mostly mandible CI,•         Unknown etiology, though in some cases follow

familial pattern, or Adrenogenital syndrome developing in early life may cause premature eruption as hormones may alter the rate of eruption,

•         Such teeth are normal in all respects, but may be mobile and should be retained,

•         May cause feeding difficulties,•         Premature eruption of permanent teeth is

generally followed by premature shedding of deciduous teeth.

ERUPTION SEQUESTRUM

•         First described by Starkey & Shafer.•         It’s a tiny irregular spicule of bone overlying

the crown of an erupting permanent molar, found just prior to or immediately following the emergence of the tips of the cusps through oral mucosa.

•         It lies directly over the central occlusal fossa but is contained within the soft tissues

•         As the tooth continues to erupt & the cusps emerge, the fragment of bone completely sequestrates through the mucosa & is lost,

•         for few days it may lie on the crest of the ridge in a tiny depression,

•         On Radiograph, it appears as a tiny irregular opacity overlying the central occlusal fossa but separated from the tooth itself.

•         While molars erupt they occasionally separate a small osseous fragment from the surrounding contiguous bone in a fashion of a corkscrew.

•         Generally it resorbs before eruption, but if the fragment is large & eruption is fast, complete resorption does not occur & eruption sequestrum is observed.

•         Slight soreness in the area,•         No treatment required.

DELAYED ERUPTION•         Etiology unknown but may be associated with

certain systemic conditions like Rickets, Cretinism & Cleidocranial Dysplasia.

•         Local factors like Fibromatosis Gingivae.•         In deciduous, difficult to state unless eruption is

overly due,

MULTIPLE UNERUPTED TEETH•         Uncommon condition,•         Retained deciduous teeth, or•         Deciduous teeth shed but permanent teeth failed

to erupt, (Pseudoanodontia)•         Normal jaws & teeth on R/Fs,•         Lack of eruptive force,•         If due to endocrine dysfunction proper treatment

may lead to eruption.

EMBEDDED & IMPACTED TEETH

•         Embedded teeth are unerupted teeth because of of lack of a eruptive force.

•         Impacted teeth are those prevented from erupting by some physical barrier in the eruption path.

•         Lack of space due to crowding of the dental arches, or  the premature loss of deciduous teeth with subsequent partial closure of the area they occupied, causes partial or total impaction.

•         Another cause is the rotation of tooth buds resulting in teeth which are aimed in the wrong direction because their long axis is not parallel to a normal eruption path.

•         More commonly involved are maxillary & mandibular 3rd molars & Maxillary cuspids followed by premolars & supernumerary teeth.

•         Treatment depends upon type of teeth & the individual circumstances,

•         Impacted teeth cause resorption of roots of adjacent teeth,

•         May cause periodic pain & even trismus,•         A dentigerous cyst may develop around the

coronal portion of impacted tooth & may cause displacement of the tooth & destruction of bone.

Impacted 3rd Molars:They exhibit a variety of positions:

Mesioangular     Impactions :  Third Molar lies obliquely in the bone Crown pointing in the mesial direction, in contact

with the distal portion of root or crown of the 2nd molar.DistoAngular Impactions.

         3rd Molar lies obliquely in the bone.         Tooth faces distally towards the ramus for

mandibular teeth

Vertical Impactions:         3rd molar is in the normal vertical position .

         Lack of space for eruption due to impingement of anterior border of the ramus or distal surface of 2nd Molar.

Horizontal Impactions:         3rd molar in horizontal position with respect to the

body of the mandible or buccally/lingually/palatally for maxillary as well as mandibular 3rdmolars.

Impacted maxillary Canines:         These can be placed from horizontal to vertical.         Horizontally, they can be placed buccally and may

impinge on the adjacent teeth, wheras vertical impactions are mainly due to lack of space for eruption.

Note:Completely impacted teeth are within the bone and cannot become carious or infected.Partially impacted or embedded teeth may be prone for periodontal infection., or caries through the operculum.

Clinical Significance:         Trismus, Referred Pain, Infection,  Resorption of

adjacent teeth, Malocclusion, Dentigerous cysts

Treatment:         Exatraction of 3rd Molars, Repositioning of

Canines by orthodontia.

ANKYLOSED DECIDUOUS TEETH (Submerged Teeth)

•         These undergo variable degree of resorption & then become ankylosed,

•         Mostly affect deciduous mandible 2nd molars,•         This prevents their exfoliation & subsequent

replacement by permanent teeth.•         After the adjacent permanent teeth erupts it looks

submerged as it is below the level of occlusion•         Teeth impart a solid sound on percussion unlike

the dull cushion sound as of normal teeth.•         Partial absence of periodontal ligament, with

areas of apparent blending b/n the tooth root & bone,

•         Suspected etiology may be trauma, infection, disturbed local metabolism or genetic influence.

•         Surgical removal to prevent malocclusion, local periodontal disturbance or caries.