Cleft lip, cleft palate and
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craniofacial syndromesDepartment of Burns and Plastic Surgery
Cleft lip and palate
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Epidemiology
Embryology
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? Face starts forming by 4 rth week and completed by 8 th week
? Palate formation is completed by 10 th week
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What is unique about craniofacial development?
? Dual origin
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? Tissue interactions? Elaborately choreographed morphogenic movements
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Cyclopia
? Part of holoprosencephaly
? Holoprosencephaly also associated with-
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Establishment and fusion of the facial
prominences
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? basic morphology of the face is established between the 4th and 10th weeks? midline frontonasal prominence,
? 3 paired prominences, the maxillary, lateral nasal, and mandibular
prominences
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The frontonasal prominence
? forehead, midline of the nose, the philtrum, the middle portion of the
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upper lip, and the primary palate.? b/l cleft lip
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The lateral nasal prominences? alae of the nose
? failure in the fusion between the lateral nasal prominences and either
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the frontonasal or the maxillary processesThe maxil ary prominences
? upper jaw and the sides of the face
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? the sides of the upper lip? the secondary palate.
The mandibular prominences
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? Lower jaw and lip
? Very rare
? Wide array of phenotypes
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Pharyngeal archesETIOPATHOGENESIS
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ETIOPATHOGENESIS
They can be classified in to 4 groups
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1. Chromosomal2. Single gene
3. Multifactorial
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4. Mitochondrial
ETIOPATHOGENESIS
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Etiopathogenesis
3. Environmental factors
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Maternal Smoking or tobacco
exposure
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Viral infectionsPoor nutrition
Drugs
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Teratogens- Rubel a,
cortisone/steroids/ mercaptopurine
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Methotrexate, Valium, DilantinDiagnosis
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USG
Problems with clefts
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Feeding problems
Oronasal fistula
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Nasal regurgitation
Bottle cup, spoon feeding
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30-45 deg angle to aid swallowingSyndromes
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? Inherited syndromeTeratogenic syndrome
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? Fetal alcohol syndrome? Fetal phenytoin syndrome
? Fetal valproate syndrome
Management by Multidisciplinary approach
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Cheiloplasty
Cleft palate surgery
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Von langenback
VY palatoplasty by Veau
Furlows palatoplasty
Wardil-kilner Pushback palatoplasty
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Bone grafting
Recent advances
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Fetal endoscopic approach
Fetal surgery in intrauterine life (less than 20 weeks)
Open fetal surgery
Conclusion
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? Second most common congenital anomaly
? Embryogenesis and etiology to be kept in mind
? Team approach
? Research to be aimed at Epigenetic modification
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Thank You