Dandy-Walker Malformations

Abstract

Dandy-Walker malformation (DWM) is a rare congenital disorder that involves the cerebellum and the fourth ventricle. It is defined as agenesis of cerebellar vermis, cystic dilation of the fourth ventricle, and large posterior fossa with upward displacement of the tentorium, lateral sinuses, and torcular. The exact etiology of DWM remains unknown. Most patients are diagnosed within the first year of life. In nearly 90% of cases there is an accompanying hydrocephalus. DWM may be associated with several central nervous system and extracranial anomalies. Nearly half of these patients will have mental disability and approximately 20% will develop seizures. Treatment often includes surgical management of hydrocephalus.