Download MBBS Burns and Plastic Surgery PPT 3 Cleft Lip Cleft Palate And Craniofacial Syndromes Lecture Notes

Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Burns and Plastic Surgery PPT 3 Cleft Lip Cleft Palate And Craniofacial Syndromes Lecture Notes

Cleft lip, cleft palate and

craniofacial syndromes

Department of Burns and Plastic Surgery

Cleft lip and palate


Epidemiology

Embryology


? Face starts forming by 4 rth week and completed by 8 th week
? Palate formation is completed by 10 th week



What is unique about craniofacial development?

? Dual origin
? Tissue interactions
? Elaborately choreographed morphogenic movements




Cyclopia

? Part of holoprosencephaly
? Holoprosencephaly also associated with-


Establishment and fusion of the facial

prominences
? 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

The frontonasal prominence

? forehead, midline of the nose, the philtrum, the middle portion of the

upper lip, and the primary palate.

? b/l cleft lip


The lateral nasal prominences

? alae of the nose
? failure in the fusion between the lateral nasal prominences and either

the frontonasal or the maxillary processes

The maxil ary prominences

? upper jaw and the sides of the face
? the sides of the upper lip
? the secondary palate.


The mandibular prominences

? Lower jaw and lip
? Very rare
? Wide array of phenotypes

Pharyngeal arches


ETIOPATHOGENESIS


ETIOPATHOGENESIS

They can be classified in to 4 groups

1. Chromosomal

2. Single gene

3. Multifactorial

4. Mitochondrial


ETIOPATHOGENESIS

Etiopathogenesis


3. Environmental factors

Maternal Smoking or tobacco

exposure

Viral infections

Poor nutrition

Drugs

Teratogens- Rubel a,

cortisone/steroids/ mercaptopurine

Methotrexate, Valium, Dilantin



Diagnosis

USG


Problems with clefts


Feeding problems

Oronasal fistula

Nasal regurgitation

Bottle cup, spoon feeding

30-45 deg angle to aid swallowing


Syndromes

? Inherited syndrome


Teratogenic syndrome

? Fetal alcohol syndrome
? Fetal phenytoin syndrome
? Fetal valproate syndrome

Management by Multidisciplinary approach


Cheiloplasty

Cleft palate surgery

Von langenback
VY palatoplasty by Veau
Furlows palatoplasty
Wardil-kilner Pushback palatoplasty


Bone grafting

Recent advances

Fetal endoscopic approach
Fetal surgery in intrauterine life (less than 20 weeks)
Open fetal surgery
Conclusion

? Second most common congenital anomaly
? Embryogenesis and etiology to be kept in mind
? Team approach
? Research to be aimed at Epigenetic modification

Thank You