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 the cerebellar vermis, enlargement of the posterior fossa, and cystic dilatation of the fourth ventricle. The exact etiology of DWM remains unknown. DWM can have a dramatic appearance or develop unnoticed, whereas the majority of patients are diagnosed within the first year of life. In nearly 90% of cases there is an accompanying hydrocephalus. DWM is associated with several central nervous system and extracranial anomalies. Regarding prognosis, nearly half of these patients will be mentally retarded and approximately 20% will develop seizures. Concerning treatment, it usually involves the treatment of hydrocephalus.