Dandy Walker Malformation

Dandy–Walker malformation consists of a triad of congenital anomalies: hypoplasia or agenesis of the cerebellar vermis, marked dilatation of the fourth ventricle, often with an enlarged posterior fossa, and supratentorial hydrocephalus. Only 1 to 4% of infantile hydrocephalus cases are associated with the Dandy Walker malformation. Most patients are children and most present during the first year of life. Recently, deletion of two linked cerebellar genes, Z1C1 and Z1C4, has been found to be related to nonsyndromic Dandy– Walker malformation.