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Dandy-Walker syndrome (DWS) or Dandy-Walker complex is a congenital brain malformation that involves the cerebellum (an area at the back of the brain that controls movement) and the fluid-filled spaces around it.

Dandy-Walker malformation was first described by Dandy and Blackfan in 1914.

The part of the brain located between the two cerebellar hemispheres called cerebral vermis becomes partially or completely absent. Other key features include an enlargement of the fourth ventricle (one of four connected cavities in the brain) and cyst formation near the internal base of the skull. The three features form a triad.

It is a genetically sporadic disorder. Dandy-Walker syndrome occurs one in every 25,000 live births. Females are mostly affected.

An increase in size and pressure occurs in the fluid spaces surrounding the brain.

References:

National Institute of Neurological Disorders and Stroke, "NINDS Dandy-Walker Syndrome Information Page
Harrison's Principles of Internal Medicine, 17^th Ed.
Volpe, Joseph, J.Neurology of the Newborn, 4^th edition.

Published on Dec 30, 2010
Last Updated on Mar 12, 2016