Sunday, July 7, 2013

Cleft lip and cleft palate

Development

Face develops from 3 processes – frontonasal, maxillary and mandibular

Intermaxillary segment is formed when medial growth of maxillary prominences cause fusion of medial nasal prominences in midline. In adult this segment forms philtrum, 4 incisors and primary palate.
Secondary palate forms from the outgrowth of maxillary prominences called palatine shelves
Primary and secondary palate fuse at the incisive foramen to form the definitive adult palate

Types 
  • Cleft lip alone 15%
  • Cleft lip and palate 45%
  • Cleft palate only 40%
  • Sometimes cleft can not be seen externally - submucous

Etiology
  • Genetic
  • Environmental – maternal epilepsy and drugs like steroids, diazepam and phenytoin
  • Syndromic- most common Pierre Robin sequence ( isolated cleft palate , glossoptosis or posteriorly placed tongue and retrognathia). Other syndromes Treacher Collins, down etc

Complications 
  • Swallowing 
  • Sucking
  • Defective speech
  • Recurrent URTI and its sequelae
  • Cosmetic problem

Primary management

Antenatal diagnosis of cleft lip (not isolated cleft palate) possible at 18 weeks of gestation by USG.
Referred to cleft surgeon if appropriate for counseling to allay fear

Photographs of before and after surgery are invaluable
Most babies born with cleft lip and palate feed and thrive well but some may require assistance
Major respiratory obstruction is uncommon and occurs exclusively in children with Pierre robin sequence

Definitive management

Cleft lip repair is commonly performed between 3-6 months of age whereas cleft palate is frequently performed between 6- 18 months of age.
Millard’s criteria (rule of 10) – cleft lip is repaired when child is 10 weeks old, child gains weight of 10 pounds and its Hb reaches 10g/dl. Millard’s cleft lip repair.
Cleft palate surgery when child is 10 months old weighs 10 kg and HB is 10g/dl. Wardill-kilner push back operation

Secondary management

Following surgery regular review regarding hearing, speech, dental development and facial growth should be done

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