Dandy-Walker Syndrome in Children Homepage

 

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.