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Dandy Walker Malformation

Abstract

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NORD is very grateful to William B. Dobyns, MD, Professor of Human Genetics, Neurology and Pediatrics at the Department of Human Genetics of the University of Chicago, for assistance in the preparation of this report.

Synonyms of Dandy Walker Malformation

  • Dandy-Walker Cyst
  • Dandy-Walker Deformity
  • Dandy-Walker Syndrome
  • DWM
  • Hydrocephalus, Internal, Dandy-Walker Type
  • Hydrocephalus, Noncommunicating, Dandy-Walker Type
  • Luschka-Magendie Foramina Atresia

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Dandy-Walker malformation (DWM) is a brain malformation that occurs during embryonic development of the cerebellum and 4th ventricle. The cerebellum is the area of the brain that helps coordinate movement, and is also involved with cognition and behavior. The 4th ventricle is a space around the cerebellum that channels fluid from inside to around the outside of the brain. DWM is characterized by underdevelopment (small size and abnormal position) of the middle part of the cerebellum known as the cerebellar vermis, cystic enlargement of the 4th ventricle and enlargement of the base of the skull (posterior fossa). DWM is sometimes (20-80%) associated with hydrocephalus, in which blockage of the normal flow of spinal fluid leads to excessive amounts of fluid accumulating in and around the brain. This leads to abnormally high pressure within the skull and swelling of the head, and can lead to neurological impairment.

Organizations related to Dandy Walker Malformation

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