FAMILIAL DANDY-WALKER MALFORMATION ASSOCIATED WITH MACROCEPHALY, FACIAL ANOMALIES, DEVELOPMENTAL DELAY, AND BRAIN-STEM DYSGENESIS - PRENATAL-DIAGNOSIS AND POSTNATAL OUTCOME IN BROTHERS - A NEW SYNDROME

Brothers are reported with an apparently new constellation of manifest ations including Dandy-Walker complex (DWC), migrational brain disorde r, macrocephaly, and facial anomalies. The first brother presented at birth, the second was detected prenatally with DWC and the pregnancy t erminated. Fetal brain histopathology showed DWC associated with brain stem dysgenesis. Inheritance is likely autosomal or X-linked recessive . An extensive review of the differential diagnosis of DWC is provided . (C) 1994 Wiley-Liss, Inc.