Dandy-Walker variant

Case contributed by Mohammad A. ElBeialy
Diagnosis almost certain

Presentation

Large head size with convulsions. Prenatal U/S shows hydrocephalus.

Patient Data

Age: 1 month
Gender: Female
ct
  • moderate supratentorial dilatation of the ventricular system. The bodies of the lateral ventricles are widely separated and parallel with high-riding 3rd ventricle

  • mega cisterna magna with retro-cerebellar cystic lesion of CSF density communicating with the 4th ventricle

  • hypoplasia of the cerebellar vermis

  • no mass lesion or parenchymal area of abnormal attenuation

  • normal cortical sulci

  • no extra-axial collection

Diagnosis:

Dandy-Walker variant with moderate supratentorial hydrocephalus and mega cisterna magna as well as retro-cerebellar cyst communicating with the 4th ventricle through hypoplastic vermis. Associated agenesis of the corpus callosum.

Case Discussion

Dandy-Walker variant is associated with variable degrees of vermian hypoplasia. A retrocerebellar cyst is seen communicating with the 4th ventricle with supratentorial hydrocephalus. Association with agenesis of the corpus callosum is common. DW variant is more common than DW malformation.

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