DANDY-WALKER MALFORMATION IN THE MECKEL SYNDROME

The Meckel syndrome (MS) is an autosomal recessive disorder classicall y defined by the triad of occipital encephalocele, multicystic kidneys , and polydactyly. Here we describe 3 sibs with varying manifestations of MS, The propositus had isolated cystic renal disease, The other si bs were both prenatally diagnosed with renal disease, polydactyly, and the Dandy-Walker malformation, an unusual central nervous system defe ct in MS, These findings are discussed in the context of the phenotypi c expression of MS and the nosology of this disorder and the cerebro-r eno-digital (Meckel-like) syndromes. (C) 1995 Wiley-Liss, Inc.