Health - What is dandy walker variant, It is a brain malformation

What is dandy walker variant

Dandy Walker variant (medical condition): A less severe form of a brain malformation called Dandy-Walker. The brain malformation involves the development of a cyst which may interfere with the drainage of cerebrospinal fluid and lead to hydrocephalus. Many patients don't have symptoms until adulthood or remain asymptomatic but some may be severely affected.

Dandy-Walker malformation is a rare congenital malformation that involves the cerebellum and fourth ventricle. Dandy-Walker malformation is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. A large number of concomitant problems may be present, but Dandy-Walker malformation is recognized whenever these 3 features are found. Approximately 70-90% of patients have hydrocephalus, which often develops postnatally. Dandy-Walker malformation may be associated with atresia of the foramen of Magendie and, possibly, the foramen of Luschka.

Dandy-Walker malformation was first described by Dandy and Blackfan in 1914. Since the original description, additional studies have reported on the various morphologic features of the syndrome. Not until 1954 did Benda first emphasize that atresia of the cerebellar outlet foramina is not an essential feature of the condition and suggested the now widely accepted term Dandy-Walker malformation.

Studies by D'Agostino in 1963 and Hart et al in 1972 further defined the characteristic triad of Dandy-Walker malformation as consisting of

(1) complete or partial agenesis of the vermis.
(2) cystic dilatation of the fourth ventricle, and
(3) an enlarged posterior fossa with upward displacement of lateral sinuses, tentorium, and torcular herophili. This triad is typically found in association with supratentorial hydrocephalus, which should be considered a complication rather than part of the malformation complex.

Symptoms of Dandy-Walker variant

The list of signs and symptoms mentioned in various sources for Dandy-Walker variant includes the 4 symptoms listed below:

  Dilated fourth ventricle
  Malformed brain
  Underdeveloped cerebellar vermis

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