Developmental Disturbances of The Oral Cavity

IMPORTANT TERMINOLOGIES Developmental Disorder It is the abnormality, defect or disturbance which occurs during the developmental stages of the tissues or organs. Congenital Disorder It 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; Or In the later stages during growth of the affected tissues. Inherited Disorder The abnormality, defect or disturbance which is transmitted through genes to off springs in the subsequent generation. Genetic Disorder The abnormality, defect or disturbance which is caused by gene abnormality or mutation.

Craniofacial Anomalies (CFA)

Anomaly CFA : Irregularity/different from normal. : 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 Malformation
A 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

Hemangioma
A 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 head Asymmetrical 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 bcoz 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 Turners 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: Pagets 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

Classification By Hoyme et al Complex hemihyperplasia : body (atleast 1 arm & 1 leg) ipsilateral or contralateral

Involving half of the They can be

 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 Bells 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 patients life.

ABNORMALITIES OF DENTAL ARCH RELATIONS Disparity of the relation of one jaw to the other. Most accepted classification Angle (1899) Class I relations. Arches in normal mesiodistal

Class II Mandibular arch is distally placed in relation to the maxillary arch. Div 1Bilaterally distal, protruding maxillary incisors Div 2Bilaterally 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 tonguetie ) 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 Aschers 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 CUPIDs 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 / Median Group 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 Clefts Group II : Clefts of the Lip & Palate. Single: Unilateral Double: Bilateral Clefts Group 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. KERNAHENs 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. Classification 3 types: Simple : Multiple painless papules with central depressions & dilated canals Superficial (suppurative) : Baelzs 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 Crohns 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 & Langhans 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 syndrome Intestinal 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 Fordyces Granules Focal Epithelial Hyperplasia

FORDYCES GRANULES (FORDYCES DISEASE) Developmental anomaly characterized by heterotrophic collections of sebaceous glands at various sites in the oral cavity. Etiology May 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 (Hecks 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 bcoz 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 fibromatosis, Congenital macrogingivae) gingival

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-MANDIBULARLIMB 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 Acquired Congenital Vascular Malformations Lymphangiomas / Hemangiomas Muscular hypertrophy Down syndrome Acquired 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 or 2) 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 as Tongue Tie.

- More commonly seen; termed

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 mucogingival 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 bcoz 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 Tongue)

Plicata,

Plicated

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 SYNDROME Benign 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. Transverse 2. Cerebriform 3. 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 Tongue)

Rash

of

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 reappears 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, Monros 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, Asymptomatic Solitary 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 its 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. 2. 3. 4. 5. 6. Aplasia Xerostomia Hyperplasia of Palatal Glands Atresia Aberrancy Developmental Lingual Mandibular Salivary Gland Depression 7. Anterior Lingual Depression APLASIA (Agenesis) Absence of Salivary Glands, Any 1 or group of Salivary Glands, Uni / Bilateral, Its 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 Syndrome L> 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 & Xerostomia D> 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 patients quality of life Affecting dietary habits, nutritional status, Speech, Taste, Tolerance to dental prosthesis, & Increased susceptibility to dental caries Etiology Temporary Psychological Duct Calculi Sialoadenitis

Drug Therapy (Zyban Anti smoking) Permanent 1. SG Aplasia 2. Sjogrens Syndrome 3. Radiotherapy 4. Surgical Desalivation 5. Other systemic conditions like DM, Parkinsons 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 Sjogrens 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
TEETH DISTURBANCES IN THE SIZE OF THE

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 roots GEMINATION

 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 Eagles 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 formdeep 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 (Leongs 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: Klinefelters 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 Ectodermal Dysplasia,

with Hereditary

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 Interproximally, buccally or lingually. Associated Syndromes: - CleidoCranial dysplasia - Sturge-Weber Syndrome molars

- Gardners 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 matrix 2. Calcification matrix is mineralized 3. 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 Dominant IB Hypoplastic, Local Autosomal Dominant IC Hypoplastic, Local Autosomal Recessive ID Hypoplastic, Smooth, Autosomal Dominant IE Hypoplastic, Smooth X linked Dominant IF Hypoplastic, rough Autosomal Dominant IG Enamel Agenesis, Autosomal recessive. Type II Hypomaturative IIA Hypomaturation, pigmented Autosomal recessive IIB Hypomaturation, X-linked recessive IIC Snow Capped teeth, Autosomal dominant Type III IIIA IIIB Hypocalcified Autosomal dominant Autosomal recessive with with with

Type IV Hypomaturation-Hypoplastic Taurodontism IVA Hypomaturation-Hypoplastic taurodontism, Autosomal dominant IVB Hypoplastic-Hypomaturation taurodontism, Autosomal dominant Clinical Features:

HP - Enamel not formed to full normal thickness on newly erupted developing teeth, HC - Enamel is soft, HMEnamel 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, Hutchinsons 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 bcoz of absence of central tubercle or calcified centre.

 Mulberry / Moons / Fourniers 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, Its 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 Turners Teeth, & condition as Turners 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 Sheilds TYPE I May be associated with Osteogenesis Imperfecta TYPE II Autosomal dominant trait, Not associated with Osteogenesis Imperfecta TYPE 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/Fs TYPE 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/Fs Type 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 portionTHISTLE TUBE & within this area radiopaque foci resembling pulp stones may be found. H/P Type 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. Odontodysplasia 2. Odontogenic Dysplasia 3. 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 ghostappearance. 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 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.

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prosthetic

DEVELOPMENTAL DISTURBANCES (ERUPTION) OF TEETH Premature Eruption Eruption Sequestrum Delayed Eruption Multiple Unerupted Teeth

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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. Its 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, Canines by orthodontia. Repositioning of

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.