The Common Vein Copyright 2010
Dandy-Walker syndrome (DWS), or Dandy-Walker complex, is a congenital malformation of the cerebellum and associated subarachnoid spaces caused by a genetic abnormality and more often affecting females.
Structurally the disorder is centered in the posterior fossa characterized by partial or complete absence of the part vermis and enlargement of the 4th ventricle, and the cisterna magna that may also demonstrate an increase in pressure. There are three basic types of Dandy Walker deformities DWS malformation, DWS mega cisterna magna and DWS variant.
Functionally the motor system is affected and the patient may present clinically with motor impairment characterized by unsteadiness of gait and lack of muscle coordination. If there is associated increase in the intracranial pressure then clinical findings may include irritability, and vomiting. Associated congenital abnormalities include defects of the corpus callosum, cardiac and limb deformities.
The diagnosis may be made prenatally by fetal ultrasound, where enlargement of the CSF spaces around the cerebellum such as cisterna magna and 4th ventricle are characteristic findings. Fetal karyotyping following amniocentesis may also be helpful.
Treatment is based on anatomic deformities but the most easily correctible is the elevated intracranial pressure which is treated by decompression by placement of a surgically placed shunt.
Normal (a,b,d,e) and Dandy-Walker Syndrome (c,f) associated with
Absence of the Corpus Callosum and Lissencephaly
Courtesy James Donnelly MD Copyright 2010 All rights reserved 95466c01L03.8s