Craniofacial anomalies

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Craniofacial anomalies

  1. 1. Craniofacial anomalies INDIAN DENTAL ACADEMY Leader in continuing dental education www.indiandentalacademy.com www.indiandentalacademy.com
  2. 2. Craniofacial Anomalies Malformation Disruption Deformation www.indiandentalacademy.com
  3. 3. Cleft Lip ,Cleft Palate and Other Orofacial Clefts www.indiandentalacademy.com
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  16. 16. Mechanism of palatal shelf elevation *Intrinsic Force within the shelf *Accumulation of Glycosaminoglycans *EGF and TGF stimulate production of Hyluronan *Increase in vascularity *Contraction of elastic fibres or muscle fibres. *Unequal division in the palatal and the oral epithelium *Neurotransmitters like Serotonin *Increase in MMP-3 *Increase in Vimentin expression *Master controlling gene is FSP-1,ssh, www.indiandentalacademy.com
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  22. 22. Midpalatal suture www.indiandentalacademy.com
  23. 23. www.indiandentalacademy.com Zone Of Intense Proliferation
  24. 24. www.indiandentalacademy.com A-P Direction Of Collagen Fibres
  25. 25. www.indiandentalacademy.com Transversely oriented collagen fibres
  26. 26. www.indiandentalacademy.com Network Of Collagen Fibres In centre Of Suture
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  29. 29. Sharpey fib www.indiandentalacademy.com
  30. 30. B S T www.indiandentalacademy.com TRANSITIONAL SPACE
  31. 31. SINUOUS AND IMBRICATING NATURE OF SUTURE www.indiandentalacademy.com
  32. 32. NARROWED ZONE OF CELLULAR PROLIFERATION www.indiandentalacademy.com
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  35. 35. LIGHT MICROSCOPIC VIEW www.indiandentalacademy.com
  36. 36. www.indiandentalacademy.com ELECTRON MICROSCOPIC VIEW
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  38. 38. Genetics Candidate gene Gene locus Name of gene TGF alfa 2p13 TGF beta 19q13.1-q13.3 MSX1 RAR alfa 4p16.1 17q12 DLX2 2q32 BCL3 19q13.1 2q32 transforming growth factor alfa transforming growth factor beta homeobox gene retinoic acid receptor alfa distal-less homeobox 2 B cell leukemia unknown gene but different from DLX possible genes are FN1,IHH,IGFBP2 unknown gene unknown gene 4q25-q31.1 www.indiandentalacademy.com 6p23
  39. 39. Teratogens • Ethyl alcohol • Retinoids • Hype rthermia • Smoking • Diphenylhydantoin • Trimethadoine • Aminopte rin www.indiandentalacademy.com
  40. 40. Site of action of teratogens in electron transport system 3-acetly-pyridine 6-nicotinic acid analogues hypoxia boric acid CO FMN 1/2O2 +2H acetoactate oxaloacetate NAD+ NADH Dhase CoQ.cty.b cyt.c cytochrome oxidase ketoglutarate phenytoin ATP www.indiandentalacademy.com H2O
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  42. 42. *Role of Retinoic Acid Retinoc acid can inhibit the proliferation of and induce the apoptosis of EMS cells. *Role of Folic Acid Deficiency A high dose of 6 mg of folic acid should be recommended for the reduction of orofacial clefts during early pregnancy. www.indiandentalacademy.com
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  50. 50. Group I These are defined as clefts of the lip only. www.indiandentalacademy.com
  51. 51. Group II These are defined as clefts of the palate only i.e. secondary palatal clefts. www.indiandentalacademy.com
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  53. 53. Group III These are defined as clefts of the lip, alveolus and palate i.e. complete cleft lip and palate www.indiandentalacademy.com
  54. 54. Group IV These are defined as clefts of the lip and alveolus www.indiandentalacademy.com
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  56. 56. Kernahan’s Striped ‘Y’ Classification www.indiandentalacademy.com
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  59. 59. Unilateral cleft lip www.indiandentalacademy.com
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  61. 61. Deviation of nasal septum www.indiandentalacademy.com
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  68. 68. Superior constrictor Palatopharyngeus m. www.indiandentalacademy.com
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  74. 74. CRANIOFACIAL CLEFTS Primary cleft www.indiandentalacademy.com Secondary cleft
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  78. 78. Lateronasomaxillary cleft Primary Cleft Maxillomandibular Cleft Medionasomaxillary Cleft www.indiandentalacademy.com
  79. 79. Tessiers Classification www.indiandentalacademy.com
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  81. 81. Two leading theories proposed for formation of rare clefts Classic Theory By Dursy and His: States that failure of fusion of the facial processes true clefts The Mesodermal penetration theory By Johnston in 1965: Recognises the importance of neural creast cells www.indiandentalacademy.com
  82. 82. Tessier Classification Cleft 0:*Represents delay in the closure of the anterior fontanel through the frontal bone,crista galli, midline of the nose,columella,lip,and maxilla and may actually involve the tongue,lower lip and the mandible. *It may give rise to frontonasal,frontoethmoidal, or frontal encephaloceles,duplication of the nasal septum and midline cleft of the lip. *Its cranial extension is cleft 14 www.indiandentalacademy.com
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  87. 87. Cleft 1 *The cleft courses through the frontal bone and the olfactory groove of the cribiform plate, between the nasal and the frontal process of the maxilla and through the maxilla between the central and lateral incisor. *It can cause telorbitism with a deep invagination of the dura in the area of the cribiform plate defect,nasoorbital encephalocele, notching of the nostrils and occasionally a cleft lip. www.indiandentalacademy.com *Cranial extension is cleft 13
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  91. 91. Cleft 2 *Cleft 2 is similar to cleft 1,but is slightly more lateral. *Its cranial extension is cleft 12 www.indiandentalacademy.com
  92. 92. Cleft 2 www.indiandentalacademy.com
  93. 93. Cleft 3 *It runs through the lacrimal bone,the frontal process of the maxilla and between the lateral incisor and the canine. *There is absence of the inferomedial wall of the orbit and lack of pneumatisation of the maxillary antrum. *Medial canthus is displaced inferiorly.There is coloboma of the lower eyelid,the conjuctiva and the nasal mucosa being seperated by a thin band of fibrous tissue. www.indiandentalacademy.com *Northbound continuation is cleft 11.
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  95. 95. Cleft 3 www.indiandentalacademy.com
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  98. 98. Cleft 4 *Similar to cleft 3 except that the clefting between the nose and the cheek is more laterally,sparing the nostrils. *The lip cresting take place lateral to the philtral crest www.indiandentalacademy.com
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  100. 100. Cleft 4 www.indiandentalacademy.com
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  106. 106. Cleft 5 *It runs through the orbital floor lateral to the infraorbital nerve and the maxillary sinus ending behind the canine in the premolar region. *The soft tissue deformity involves the coloboma of lateral third of lower lip,ending as the cleft of the lip. www.indiandentalacademy.com
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  110. 110. Cleft 6 *Seperates the maxilla from the malar bone. *The corresponding soft tissue deformity consists of coloboma of the lower lid and a furrow of skin from the coloboma to the angle of the mandible www.indiandentalacademy.com
  111. 111. Cleft 6 www.indiandentalacademy.com
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  113. 113. Cleft 7 *Courses between the malar and the temporal bone. * The zygomatic arch is usually absent. *The condyle,coronoid and the ramus suffer varying degrees of deformity. *The temporal muscle is either absent or atrophic forming a contineous temporomassetric muscle. *There may be cleft of the scalp or abnormal hair growth. www.indiandentalacademy.com *There may be a degrees of ear malformations
  114. 114. Cleft 8 *It is a frontozygomatic cleft extending to the greater wing of the sphenoid. *In the soft tissue there may be either a true cleft or notching of the lower eyelid. www.indiandentalacademy.com
  115. 115. Cleft 9 *It is an upper orbital cleft of the superolateral orbital ridge angle with a corresponding coloboma of the upper lid. www.indiandentalacademy.com
  116. 116. Cleft 6,7,8 bilateral www.indiandentalacademy.com
  117. 117. Cleft 10 *It is an upper central orbital cleft of the frontal bone,supraorbital ridge and the orbital rod,which is lateral to the supraorbital neurovascular bundle,causing an encephalocele. *It should be associated with a coloboma of the medial of the upper lid. www.indiandentalacademy.com
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  119. 119. Cleft 11 *It is an upper medial orbital cleft through the frontal bone,frontal sinus and lateral mass of the ethmoid. *The soft tissue deformity is coloboma of the medial third of the upper eyelid. www.indiandentalacademy.com
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