ADAMS-OLIVER SYNDROME - A FAMILY WITH EXTREME VARIABILITY IN CLINICALEXPRESSION

We describe a mother and her 3 children with variable scalp defects an d limb defects consistent with a diagnosis of Adams Oliver syndrome al so presenting with additional anomalies including congenital heart dis ease, microcephaly, epilepsy, mental retardation, arrhinencephaly, hyd rocephaly, anatomic bronchial anomalies, and renal anomalies. The clin ical variation between the individuals is more pronounced than in prev iously reported families. (C) 1994 Wiley-Liss, Inc.