Cause:Genetic, but lip alone is separate genetically from isolated cleft palate; plus perhaps in utero environmental factors (vitamin deficiencies cause in rats); autosomal recessive; recurrence in subsequent siblings ~3%, much lower if different father, no change if mother changes town of residence, hence genetic not environmental (Nejm 1995;333:161)
Pathophys:Failure of maxilla and nasal fusion in midline
1/1000 Caucasian births, 1/5000 black births; 3rd most common congenital anomaly after club feet and radiologic spina bifida. Increased incidence in inbred populations; associated (25%) with other anomalies too
50% are lip and palate, 25% lip alone (male > female), 25% palate alone (female > male)
Social; malocclusion; chronic otitis and hearing loss from eustachian tube dysfunction; mechanical speech problems; FTT from poor caloric intake or associated anomalies
Rx:
Test hearing and refer to ENT, or state cleft lip program available in most states
Feed w squeezable not rigid bottles (Cochrane Rev 2004;[3]:CD003315); speech rx postop emphasizing hard consonants; blowing exercises through mouth (pinwheel, wind instrument)
Surgical timing is controversial—some say repair at age 6 wk if lip alone; at age 14 weeks if palate involved too; but must repair before start speech; typically multistage procedure
Dental prosthesis