Dandy-Walker Syndrome in Children Homepage

This page was last updated on May 9th, 2017

 

Authors

Ira Bowen, B.A.

Caleb Standafer, B.S.

Mark D. Krieger, M.D.

Section Editor

Shlomi Constantini, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum and the surrounding spaces. The term Dandy-Walker represents several abnormalities of brain development, which are classified as malformation, mega cistern magna, and variant. Dandy-Walker malformation involves a partial or complete agenesis of the cerebellar vermis. The variant type is less severe and involves an underdeveloped cerebellar vermis and a cyst space. Dandy-Walker cysts are enlarged fourth ventricles that result from a malformation of the cerebellar vermis and atresia of the foramina of Luschka and Magendie.

Key Points

  • Enlarged fourth ventricle consistent finding: This congenital malformation results in an enlarged fourth ventricle. There is often associated hydrocephalus.
  • Treatment option of shunting or fenestration: Shunting and/or fenestration represent treatment options.
  • Patients followed for hydrocephalus: Treatment is low-risk, but prolonged follow-up is necessary.