The wide spectrum of clinical expression in Adams-Oliver syndrome: a report of two cases

Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas i n the first child the typical features of ACC, syndactyly and transverse na il dystrophy were only mildly expressed and associated defects of the centr al nervous system and cardiac malformations were absent, the second child s uffered from a very severe expression of the syndrome, with a combination o f ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple c ardiac and central nervous system malformations which resulted in fatal cen tral respiratory insufficiency.