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CASE 6

Updated: Sep 6, 2019

Courtesy of Almoosa Specialist Hospital


10-years-old male with ventriculoperitoneal shunt



 

CASE 6 ANSWER

Dandy-Walker Malformation



A large simple cyst (yellow arrows) communicates with a dilated 4th ventricle (yellow star), expands the posterior fossa, leads to superior rotation of a hypoplastic vermis (green arrow), anteriorly displaces a flattened brain stem (red arrow), and causes obstructive hydrocephalus (white arrows). Note the presence of a ventriculoperitoneal shunt (black arrow).




Key Points


  • The classic form manifests as a posterior fossa cyst communicating with a dilated fourth ventricle and leading to the superior rotation of a hypoplastic vermis, elevation of the torcular herophili, scalloping of the occipital bone.

  • Hydrocephalus is a typical early manifestation.

  • Commonly associated with other syndromic and non-syndromic features, such as cortical dysplasia, corpus callosum malformations, and other findings.

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