Large head size with convulsions. Prenatal U/S shows hydrocephalus.
Age: 1 month
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- 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.
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 DiscussionDandy-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.
- Wassmer E, Davies P, Whitehouse WP, Green SH: Clinical spectrum associated with cerebellar hypoplasia. Pediatr Neurol 28: 347–351, 2003