DEVELOPMENTAL DEFECTS AND MANAGEMENT IN PEDIATRIC DENTISTRY.pptx

Development

1. DEVELOPMENTAL DEFECTS AND MANAGEMENT IN PEDIATRIC DENTISTRY; CONGENITAL ABNORMALITIES IN CHILDREN RELATED TO PEDIATRIC DENTISTRY. MANOJ K

2. Contents Introduction Developmental Disturbances Of Jaw Agnathia Micrognathia Macrognathia Facial Hemihypertrophy Facial Hemiatrophy Developmental Disturbances Of Lips Orofacial Clefts Commissural Lip Pits Paramedian Lip Pits Double Lip Developmental Disturbances Of Palate Lateral Soft Palate Fistulas Developmental Disturbances Of Oral Mucosa Fordyce Granules Developmental Disturbances Of Gingiva Hereditary Fibromatosis Retrocuspid Papilla Developmental Disturbances Of Tongue Microglossia Macroglossia Ankyloglossia Fissured Tongue Hairy Tongue Varicosities

3. Developmental Disturbances Of Salivary Gland Aplasia Stafne Defect  Developmental Disturbances Of Teeth Size Shape Number Eruption Structure  Developmental Cyst Of Oral Region Palatal Cysts Of The New Born Nasolabial Cyst Globulomaxillary Cyst Nasopalatine Duct Cyst Median Palatal Cyst Median Mandibular Cyst Thyroglossal Duct Cyst Dermoid Cyst Common Orofacial Syndromes In Children Down Syndrome Patau Syndrome Turner Syndrome Klinefelter Syndrome Cleidocranial Dysplasia Marfan Syndrome Ellis-Van Creveld Syndrome Rubinstein- Taybi Syndrome Apert Syndrome Crouzon Syndrome Treacher - Collins Syndrome Pierre Robin Syndrome Papillon-Lefevre Syndrome Van Der Woude Syndrome Conclusion References

4. Introduction A large number of developmental anomalies, which involve the body in general and oral structure in particular can occur during the embryonic life.

5. Types of developmental anomalies Congenital anomalies: The defects, which are present at or before birth during the intrauterine life. Hereditary developmental anomalies: When certain defects are inherited by the offspring from either of the parent, called hereditary developmental anomalies. Acquired anomalies: Developed during intrauterine life due to some pathological environmental conditions. Hamartomatous anomalies: an excessive, focal overgrowth of mature, normal cells and tissues, which are native to that anatomic location. Idiopathic anomalies: Developmental anomalies of unknown cause.

6. Developmental disturbances of jaw

7. Agnathia A rare and lethal malformation characterized by  microstomia  (small mouth), aglossia , agnathia (absence of the lower jaw) and abnormally positioned ears. Autosomal recessive mode of inheritance. Principal anomaly derived from first  pharyngeal arch  as a consequence of failed mesenchymal migration of maxillary prominence and atrophy in the development of mandibular prominences OTOCEPHALY

8. Micrognathia Etiology of the congenital type - unknown Acquired type of micrognathia is of postnatal origin and results from disturbance in the area of the TMJ. TRUE MICROGNATHIA congenital or acquired

9. Macrognathia An increase in size of both jaws are frequently proportional to a generalized increase in size of entire skeleton, e.g. in pituitary gigantism. Surgical correction of such cases is feasible. Ostectomy, or resection of a portion of the mandible to decrease its length can be done. Paget's disease of bone Acromegaly Leontiasis ossea

10. Facial Hemihypertrophy Asymmetric overgrowth of one or more body parts. If the enlargement is confined to one side of the face - hemifacial hyperplasia. The enlargement becomes more accentuated with age, especially at puberty. Often the skin is thickened and may demonstrate increased pigmentation, hypertrichosis , telangiectasias , or nevus flammeus . 2:1 female-to-male predilection; right side of the body

11. Increased abdominal tumors, adrenal cortical carcinoma, and hepatoblastoma. Oral manifestations -

12. Facial hemiatrophy Slowly progressive atrophy of soft tissues of half of face, characterised by progressive wasting of subcutaneous fat, sometimes accompanied by atrophy of skin, cartilage, bone and muscle. Familial distribution has been found. Female to male ratio is 3 : 2. Parry-Romberg syndrome, progressive facial hemiatrophy .

13. Painless cleft, the 'coup de sabre ', near the midline of the face or forehead. In addition to facial wasting, ipsilateral salivary glands and hemiatrophy of the tongue, unilateral involvement of the ear, larynx, esophagus, kidney and brain. Pigmentation disorders, vitiligo, contralateral trigeminal neuralgia and ocular complications

14. Developmental disturbances of lips and palate

15. Orofacial clefts Disturbances in growth of the tissue processes or their fusion may result in the formation of orofacial clefts. Defective fusion of the medial nasal process with the maxillary process leads to cleft lip (CL). Failure of the palatal shelves to fuse results in cleft palate (CP). Approximately 45% of cases are CL + CP, 30% - isolated CP 25% - isolated CL.

16. The cause of non syndromic clefts does not follow any simple mendelian pattern of inheritance but appears to be heterogeneous. Lateral facial cleft Caused by lack of fusion of the maxillary and mandibular processes 0.3% of all facial clefts. Occur as an isolated defect or associated with other disorders, such as mandibulofacial dysostosis. Unilateral or bilateral, extending from commissure towards the ear, resulting in macrostomia .

17. The oblique facial cleft extends from the upper lip to the eye. Nearly always associated with CP and severe forms are incompatible with life. May involve the nostril, or it may laterally bypass the nose as it extends to the eye. Represents only 1 in 1300 facial clefts. Failure of fusion of the lateral nasal process with the maxillary process

18. Median cleft of the upper lip is an extremely rare anomaly Results from failure of fusion of the medial nasal processes. May be associated with oral-facial- digital syndrome and Ellis-van Creveld syndrome. Agenesis of the primary palate associated with holoprosencephaly.

19. CL ± CP is more common in males > females. Male- to- female ratio for isolated CL is 1.5: 1 Isolated CP is more common in females Clefts of both the hard and soft palates are twice as common in females. About 80% of cases of CL will be unilateral, with 20% bilateral Approximately 70% of unilateral CLs occur on the left side.

20. The prevalence of cleft uvula is much higher than CP, 1 in every 80 white individuals. In Asian and Native American populations - as high as 1 in 10. Cleft uvula is less common in blacks. In some instances a submucous palatal cleft develops. Frequently a notch in the bone is present along the posterior margin of the hard palate.

21. Treatment and management Primary lip closure is usually accomplished during first few months of life, followed later by repair of palate. Prosthetic and orthopedic appliances are used to mold or expand the maxillary segments before closure of the palatal defect. Later in childhood, autogenous bone grafts can be placed in the area of alveolar bone defect. Secondary soft tissue & orthognathic procedures may be used to improve function and cosmetic appearance.

22. Paramedian lip pits Believed to arise from persistent lateral sulci on the embryonic mandibular arch. Typically present as bilateral and symmetric fistulas on either side of the midline of the vermilion of the lower lip. Range from subtle depressions to prominent humps. Congenital fistulas of the lower lip; congenital lip pits

23. Usually inherited as an autosomal dominant trait in combination with cleft lip and/or cleft palate Recent genetic studies have shown that microdeletions at chromosome bands 1q32-q41 are the cause of van der Woude syndrome in some families.

24. Commissural pits and fistulas Unilateral or bilateral depression or pit that occurs on the vermilion surface of either lip but more commonly on the lower lip The lip sometimes appears swollen, accentuating the appearance of the pits In several cases preauricular pits have been reported in association with commissural pits.

25. Double lip Redundant fold of tissue on the mucosal side of the lip. Congenital cases - arise during the second to third month of gestation. Acquired double lip may be a component of Ascher syndrome, or it may result from trauma or oral habits. Upper lip is affected much more often than the lower lip With the lips at rest, the condition is usually unnoticeable, but when the patient smiles, excess fold of tissue is visible.

26. Lateral soft palate fistulas

27. Developmental disturbances of oral mucosa

28. Fordyce's granules ( fordyce's disease) Small yellow spots, either discretely separated or forming, often projecting slightly above the surface of the tissue. Bilaterally symmetrical pattern seen on the mucosa of the cheeks opposite the molar teeth. Very rarely a benign sebaceous gland adenoma, keratin-filled pseudocysts may develop from the ducts of these sebaceous glands.

29. Developmental disturbances of gingiva

30. Fibromatosis Gingivae Dense, diffuse, smooth, or nodular over growth of the gingival tissues of one or both arches, usually appearing at the time of eruption of the permanent incisors . It is often so firm and dense that it may prevent the normal eruption of teeth. It is not painful and shows no tendency for hemorrhage. When tooth eruption is impeded, surgical removal of the excessive tissue and exposure of the teeth are indicated . Elephantiasis Gingivae, Hereditary Gingival Fibromatosis

31. Retrocuspid papilla It’s a small, elevated nodule located on the lingual mucosa of the mandibular cuspids. This soft, well-circumscribed, mucosal nodule, commonly bilateral , is located between the free gingival margin and the mucogingival junction. It is exceedingly common in children , occurring in 99% of those between the ages of eight and 16 years.

32. Developmental disturbances of tongue

33. Aglossia And Microglossia Syndrome Commonly observed is a rudimentary, small tongue. Fetal cell traumatism in the first few weeks of gestation . As a consequence of lack of muscular stimulus between alveolar arches, these do not develop transversely and the mandible does not grow in an anterior direction, producing a severe dentoskeletal malocclusion. No predilection for gender and has no genetic implicat ions

34. Macroglossia Down syndrome and Beckwith- Wiedemann syndrome. True macroglossia and pseudomacroglossia . Surgery is done to reduce tongue size and thereby improve function. Tongue hypertrophy, enlarged tongue.

35. Ankyloglossia or tongue tie Exist when the interior frenulum attaches to the bottom of the tongue and subsequently restricts free movement of the tongue . Feeding problems are noticed early in an infant's life. Speech defects, especially articulation of the sounds: l, r, t, d, n, th , sh and z. Dental problems causing a persistent gap between the mandibular incisors. Frenulectomy is recommended.

36. Cleft tongue Occurs due to lack of merging of the lateral lingual swellings Partially cleft tongue is more common and is manifested simply as a deep groove in the midline of dorsal surface. O ften found as one feature of oral-facial-digital syndrome . Food debris and microorganisms may collect in the base of the cleft and cause irritation.

37. Fissured tongue Characterized by grooves that vary in depth and are noted along the dorsal and lateral aspects of the tongue. Usually asymptomatic, unless debris is entrapped within the fissure or when it occurs in association with geographic tongue. The prominence of the condition appears to increase with age. When prominent, the fissures or grooves may be interconnected, separating the tongue dorsum into what may appear to be several lobules. Melkersson -Rosenthal syndrome

38. Hairy tongue Hypertrophy of filiform papillae on the dorsal surface of the tongue is seen usually due to lack of mechanical stimulation and debridement. Often occurs in individuals with poor oral hygiene. Hairy tongue is rarely symptomatic, although overgrowth of Candida albicans may result in glossopyrosis . Patients complain a tickling sensation in the soft palate and the oropharynx during swallowing. In more severe cases, patients complain of a gagging sensation. Lingua Nigra, Lingua Villosa , Black Hairy Tongue

39. Lingual thyroid nodule The lingual thyroid is a condition in which follicles of thyroid tissue are found in the substance of the tongue, possibly arising from a thyroid anlage that failed to 'migrate' to its predestined position. The benign enlargement of lingual thyroid tissue is thought to be due to functional insufficiency of the chief thyroid gland in the neck, since some patients with such a lingual lesion are without a demonstrable main thyroid gland.

40. Lingual thyroid may be manifested clinically as a nodular mass in or near the base of the tongue Chief symptoms - dysphagia, dysphonia, dyspnea, hemorrhage with pain, or a feeling of tightness or fullness in the throat. Usually recommended that a patient with an ectopic thyroid gland should have a trial of replacement thyroid hormone therapy before excision is contemplated, since this will often decrease the size of lesion and make surgery unnecessary.

41. Developmental disturbances of salivary gland

42. Aplasia Any one or group of salivary glands may be absent, unilaterally or bilaterally. The CT scan or MRI will indicate the gland's absence and its replacement by fat and fibrous tissue. Aplasia occurs for unknown reasons as an isolated finding or in conjunction with other developmental defects such as, hemifacial microsomia, mandibulofacial dysostosis. Treatment is directed at relieving xerostomia and its effects. Salivary substitutes, frequent mouth washes, fluoride therapy, and good oral hygiene all play a role in successful management.

43. Stafne cyst It’s a unusual form of slightly aberrant salivary gland tissue where a developmental inclusion of glandular tissue is found within. Radiographically, the lesion appears as an ovoid radiolucency located between the inferior alveolar canal and the inferior border of the mandible in the region of the second or third molars. The classic Stafne cyst is described in the posterior mandible, an anterior variant presenting as a round or ovoid radiolucency in the area between the central incisors and first premolars exists; however, it is less common.

44. Developmental anomalies of teeth

45. Disturbance In The Size Of The Teeth

46. Microdontia Teeth which are smaller than normal True generalized microdontia - Seen in pituitary dwarfism, Down syndrome. Relative generalized microdontia Microdontia involving only a single tooth These size abnormalities are thought to originate during the morpho -differentiation stage of development. Clinical significance- Teeth are often spaced which may be disturbing cosmetically. No specific treatment is indicated for this condition.

47. Macrodontia Macrodontia refers to teeth that are larger than normal. True generalized macrodontia - associated with pituitary gigantism Relative generalized macrodontia Macrodontia of single teeth Otodental syndrome, consisting of high frequency hearing loss and globe shaped fused molar teeth, is another condition that involves macrodontia .

48. Developmental disturbances in shape of the teeth

49. Gemination Occurs from an attempt at division of a single tooth germ, with resultant incomplete formation of two teeth. The structure is usually one with two completely or incompletely separated crowns that has a single root and root canal. Seen in the deciduous as well as the permanent dentition. It is result of Schizodontism , the splitting of a tooth germ during development or from synodontism , the fusion of normal tooth bud with one from a developing supernumerary tooth.

50. Fusion It has been thought that some physical force or pressure produces union of developing teeth and their subsequent fusion. If this contact occurs early before calcification begins, the two teeth may be completely united to form a single large tooth. Grahnen and Granath have reported that fusion of teeth is more common in the deciduous than in permanent dentitions. Fusion results in one anomalous large crown in place of two normal teeth and is having a single enlarged root or two roots.

51. Concrescence It is a form of fusion which occurs after root formation has been completed. In this condition, the teeth are united by cementum only. It is thought to arise as a result of traumatic injury or crowding of teeth with resorption of the interdental bone so that the two roots are in contact and become fused by the deposition of cementum between them. Concrescence may occur before or after the teeth have erupted Rarely involves deciduous dentition

52. Dilaceration ‘Dilaceration’ refers to an angulation, or a sharp bend in the root or crown of a formed tooth The condition is thought to be due to trauma during tooth formation with the result that the position of the calcified portion of the tooth is changed and the remainder of the tooth is formed at an angle. Dilacerated teeth frequently present difficult problems at the time of extraction. Caution should be exercised when extracting this tooth. Dilaceration can be seen in both the permanent and deciduous dentitions, and is common in posterior teeth and in the maxilla.

53. Talon cusp It resembles an eagle’s talon , projecting lingually from cingulum of maxillary or mandibular permanent incisor. Composed of normal enamel and dentin and contains a horn of pulp tissue. It extends to the incisal edge as a prominent 'T' shaped projection . Occurs during the morphodifferentiation stage. Some are sharp and spike like while others are rounded. Prophylactically restoring the groove is done to prevent caries. In case of occlusal interference it should be removed but exposure of pulp horn necessitates endodontic therapy.

54. Dens in dente Thought to arise as a result of an invagination in the surface of a tooth crown before calcification. Etiology - increased localized external pressure, focal growth retardation. The permanent maxillary lateral incisors are most frequently involved and appears as an accentuation in the development of lingual pit. Radiographically - pear shaped invagination of enamel and dentin with a narrow constriction at the opening on the surface of tooth and closely approximating pulp in depth. dens invaginatus , dilated composite odontome

55. Dens evaginatus Appears clinically as an accessory cusp or a globule of enamel on the occlusal surface between the buccal and lingual cusps of premolars, unilaterally or bilaterally, although it has been reported to occur rarely on molars, cuspids, and incisors. It is thought be due to proliferation and evagination of an area of the inner enamel epithelium and subjacent odontogenic mesenchyme into the dental organ during early tooth development. Occlusal tuberculated premolar; Leong’s premolar; Evaginated Odontome ;

56. Taurodontism The term " taurodontism " describes a peculiar dental anomaly in which the body of the tooth is enlarged at the expense of the roots. Third molars are most affected and lack constriction at apex. Involved teeth tend to be rectangular in shape rather than taper towards the roots. Pulp lacks the usual constrictions at the cervical of the tooth and the roots are exceedingly short. The bifurcation or trifurcation may be only a few millimeters above the apices of the roots.

57. Causes of taurodontism Hamner and his associates believe that taurodont is caused by failure of Hertwig's epithelial sheath to invaginate at the proper horizontal level. May affect either the deciduous or permanent dentition, although permanent tooth involvement is more common. The teeth involved are almost molars, sometimes only a single tooth, at other times several molars in the same quadrant.

58. Enamel pearl These are white, dome shaped calcified projections of enamel, usually located at the furcation areas of the molar teeth. Maxillary molars are more frequently affected than any other teeth. Histologically - these are composed of normal appearing enamel sometimes with a central core of dentin. It is believed that epithelial component of HERS may sometimes retain its ameloblastic potential and therefore may synthesize enamel in some focal areas in place of cementum.

59. Developmental disturbances in number of teeth

60. Anodontia Syndromes associated - Ectodermal dysplasia, Chondroectodermal dysplasia, Achondroplasia, Rieger syndrome. Mutations in MSX1, PAX9 and EDA have been identified in familial severe agenesis

61. Supernumerary teeth Develops from a third tooth bud arising from the dental lamina near the permanent tooth bud, or possibly from splitting of the permanent tooth bud itself Multiple Supernumerary teeth, many of them impacted, are characteristically formed in cleidocranial dysplasia . Syndromes associated- aperts , Gardner, Downs, Crouzon, OFD. Clinical features- crowding, caries incidence, may develop dentigerous cyst. Treatment - mostly non functional and can be extracted.

62. Predeciduous dentition Described as hornfied epithelial structures without roots, occurring on the gingiva over the crest of the ridge, which may be easily removed. Arise from an accessory bud of the lamina ahead of the deciduous bud. In consideration of complications as Riga- Fede -disease, feeding problems, possibility of infection and hyper mobility, most of the time extraction is the treatment of choice.

63. Developmental disturbances in eruption of teeth

64. Eruption sequestrum It’s a tiny spicule of bone overlying the crown of an erupting permanent molar, immediately following the emergence of cusp tip through oral mucosa. As the tooth continues to erupt and cusps emerge, fragment of bone completely sequestrates through the mucosa and is lost. Etiology - as tooth erupts they will separate a small osseous fragment from surrounding bone. If it is large or eruption is fast, complete resorption can't occur and sequestrum forms. The clinical significance is that child complains of soreness in the area during eating, no treatment required as it corrects itself.

65. Disturbances in structure of teeth

66. Amelogenesis imperfecta Represents a group of hereditary defects of enamel. It is entirely an ectodermal disturbance, since mesodermal component of teeth are normal. All teeth of both dentitions are affected to some degree. The crowns of the teeth may or may not show discolouration if present ranges from yellow to dark brown; in some cases enamel may be absent or have a chalky texture or cheesy consistency. Hypoplastic Hypocalcification Hypomaturation

67. Enamel hypoplasia Defined as an incomplete or defective formation of the organic enamel matrix of teeth. a. heredity type b. caused by environmental factors. Etiology nutritional deficiency (vitamin A, C, D) exanthematous disease (chicken pox, measles, scarlet fever) hypocalcemia birth injury local infection or trauma ingestion chemicals (chiefly fluoride) idiopathic causes

68. Dentinogenesis imperfecta

69. Roots may be short and blunted The cementum, PDL and supporting bone appear normal. In type 3 it is characterized as shell teeth representing a dentinal disturbance in which enamel is normal while dentin is thin and the pulp chambers are enormous. The large size of pulp chambers is not due to resorption but due to defective dentin formation. The dentin is composed of irregular tubules with large areas of uncalcified matrix.

70. Dentin dysplasia Normal enamel but atypical dentin formation with abnormal pulp morphology.

71. Radiographic features

72. Regional odontodysplasia Etiology - Somatic mutation latent virus residing in odontogenic epithelium which becomes active during tooth development. Irregular in appearance with evidence of defective mineralization Radiographic features: shows a marked reduction in radio density so that the teeth assumes a ghost appearance. Histologic features: Marked reduction in the amount of dentin, widening of pre dentin layer, presence of large areas of inter globular dentin, irregular dentin tubules pattern. Maxillary > mandibular . M axillary central incisor, lateral incisor and cuspid

73. Developmental cyst of oral region

74. Nasopalatine duct cyst Affects the midline anterior maxilla Swelling (52-88%), discharge (25%), and pain (20-23%). A salty taste in the mouth. Devitalization of the pulps of associated teeth occurs. Nasopalatine Canal Cyst, Incisive Canal Cyst

75. Globulomaxillary cyst Found within the bone at the junction of the globular portion of the medial nasal process and the maxillary process, usually between the maxillary lateral incisor and cuspid teeth. Rarely, the cyst does become infected, and patient may complain of local discomfort or pain in the area. Appears as an inverted, pear-shaped radiolucent area between the roots of the lateral incisor and cuspid, usually causing divergence of the roots of these teeth.

76. Nasoalveolar cyst Arise at the junction of the globular process, the lateral nasal process, and the maxillary process as a result of proliferation of entrapped epithelium along the fusion line. May cause a swelling in the mucolabial fold as well as in the floor of the nose. Superficial erosion of the outer surface of the maxilla may be produced by pressure of the nasoalveolar cyst. The cyst should be surgically excised, although care must be exercised to prevent perforation and collapse of the lesion. Nasolabial Cyst, Klestadt's Cyst

77. Palatal and Alveolar cysts of Newborn Epstein's Pearls, Bohn's Nodules, Gingival Cysts Of The Newborn

78. Palatal cysts of the newborn present as multiple (usually less than six), 1-4 mm, yellow-white, mucosal papules of the posterior hard palate, and occasionally of the anterior soft palate. The cysts are usually somewhat larger and less numerous than the gingival cysts of the alveolar processes in newborns No treatment is required for gingival or palatal cysts of the newborn. The cysts are very superficial and within weeks will rupture to harmlessly spill their contents into the oral or pharyngeal environment.

79. Median Palatal Cyst Firm or fluctuant swelling of the midline of the hard palate posterior to the palatine papilla. The lesion appears most frequently in young adults. Often it is asymptomatic, but some patients complain of pain or expansion. Occlusal radiographs demonstrate a well – circumscribed radiolucency in the midline of the hard palate Microscopic examination shows a cyst that is usually lined by stratified squamous epithelium. Chronic inflammation may be present in the cyst wall.

80. Median Mandibular Cyst It represents a fissural cyst in the anterior midline of the mandible that develops from epithelium entrapped during fusion of the halves of the mandible during embryonic life Midline radiolucency found between or apical to the mandibular central incisor teeth . Cortical expansion may be noted. Surgical enucleation

81. Dermoid cyst Most commonly occur in the mid line of the floor of the mouth, although occasionally they are displaced laterally or develop in other locations. If the cyst develops above the geniohyoid muscle, a sublingual swelling may displace the tongue toward the roof of the mouth and create difficulty in eating, speaking, or even breathing The lesion is usually slow growing and painless, presenting as a doughy or rubbery mass that frequently retains pitting after application of pressure. Surgical removal

82. Common Oro Facial Syndromes In Children

83. Down’s syndrome

84. Periodontal diseases occur in 90% of cases. Prevalence of dental caries is low. Eruption of both deciduous and permanent teeth is delayed. Malalignment of teeth is common. Broad, short neck, hypogenitalia , short broad hands. Showing brachydactyly, single palmar crease, hyperflexibility of joints.

85. Patau syndrome Microcephaly Scalp defects Microphthalmia Orofacial clefting Congenital heart defect Polydactyly Severe developmental retardation Survival beyond 3 years is exceptional

86. Edward’s syndrome Median life expectancy is 4 days with a range of 1 hour to 18 months Microcephaly , Dolichocephaly, Prominent occiput, Micrognathia, Overlapped flexed fingers, Congenital heart defects, Low-set malformed ears.

87. Turners syndrome IQ may be reduced or even normal, intelligence is normal. Several psychiatric disturbances like depression, low self-esteem are reported. Visual abnormalities Short stature Webbed neck High arched palate Premature eruption of teeth Micrognathia Short cranial base Mid-facial hypoplasia Coarctation of the aorta, VSD and multiple pigmented nevi.

88. Klinefelter syndrome Delayed speech, Average IQ (approx. 90), Delay in emotional development, Aggressiveness, Depression, Smaller calvarial size, Larger gonial angle, Both maxillary and mandibular prognathisms tend to occur, Taurodontism has been reported in some cases.

89. Cleidocranial dysostosis Autosomal dominant inheritance Brachycephalic skull with pronounced frontal and parietal bossing Hypoplastic maxilla and zygomas Face appear small with Hypertelorism Nose is broad at the base, with the bridge depressed Neck appears long, shoulders are narrow, droop markedly Clavicles are absent unilaterally or bilaterally. Ability of the patient to approximate the shoulders in front of the chest.

90. Oral manifestations High arched palate, Complete cleft of the hard and soft palates. Delayed union at the mandibular symphysis is characterstic . Underdeveloped premaxilla with relative mandibular prognathism . Multiple supernumerary teeth, multiple crown and root abnormalities, ectopic localization of teeth and lack of tooth eruption. Deciduous root resorption is extremely delayed or arrested.

91. Marfan syndrome Mutations in fibrillin type I gene. Dolichocephaly Retrognathia Cleft palate Mandibular prognathism Arachnodactyly Hyperextensibility of joints with recurrent dislocation, scoliosis, aorta aneurysm, mitral valve prolapse, thoracic cage deformities, pulmonary infections.

92. Ellis Van Creveld syndrome Autosomal recessive Chondrodysplasia of long bones with short extremities & stature Ectodermal dysplasia with nail hypoplasia and oral anomalies Congenital heart defects in 60% (single atrium) Neonatal teeth Partial anodontia Small teeth Delayed eruption

93. Rubinstein Taybi Syndrome Broad thumbs Facial dysmorphism Growth retardation and mental deficiency Microcephaly Prominent forehead Broad nasal bridge High arched palate Talon's cusp observed in 90% cases.

94. Apert syndrome Asymmetric cranial base Steep forehead Clover leaf skull Midface hypoplasia Mandibular prognathism . Hypertelorism High arched palate, High frequency of cleft palate, Anterior openbite , anterior and posterior cross bite Syndactyly.

95. Crouzon syndrome Craniosynostosis (pre-mature fusion of the cranial sutures) Protuberant frontal region Midface hypoplasia Mandibular prognathism Possible cleft palate Hypertelorism and Exophthalmos Nose: Parrot-beaked nose Cervical fusion ( Spina Bifida) Maybe mentally retarded

96. Pierre Robin syndrome Micrognathia cleft palate glossoptosis difficulty in respiration feeding problems syndactyly polydactyly hyperextensibility of joints congenital hip dislocations.

97. Papillon Lefevre Syndrome Papillon Lefevre , 1924 Autosomal recessive Clinical Features Palmar & Plantar keratosis Severe Periodontitis of both dentitions Premature loss of deciduous dentition (4 Years) Loss of permanent dentition by 14 years of age

98. Conclusion The diagnosis and management of dental anomalies constitute importance in Pediatric dentistry. A thorough knowledge of embryology, histology should be known for proper diagnosis of etiologic factors and treatment.

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