Dandy-Walker Syndrome

Dandy-Walker syndrome is a combination of several anomalies of the brain that exist together. These anomalies include a malformation of the cerebellum, hypoplasia of the cerebellar vermis, enlarged posterior fossa with upward displacement of the torcula and sinuses, and cystic dilation of the fourth ventricle. Dandy-Walker syndrome is also known by the names Dandy-Walker malformation, Dandy-Walker cyst, and Dandy-Walker deformity. Dandy-Walker variant consists of a cystic mass in the posterior fossa with variable hypoplasia of the cerebellar vermis and without enlargement of the posterior fossa.

The incidence of Dandy-Walker syndrome is reported as being in 1 in 10,000 to 30,000 infants, with reported survival rates of between 75% and 100%. This combination of brain malformations that occurs in Dandy-Walker syndrome can lead to a variety of ...