Dandy-Walker malformation with callosal dysgenesis and atretic occipital cephalocele

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Occipital swelling at the midline since birth.

Patient Data

Age: 1 month
Gender: Female

The MRI sequences demonstrate;

  • enlarged posterior fossa with cystic dilatation of the 4th ventricle, communicating with an enlarged posterior peri-cerebellar space through a hypoplastic cerebellar vermis.with lateral displacement of the cerebellar hemispheres
  • moderate dilatation of the 3rd, and lateral ventricles with dysgenesis of the corpus callosum
  • small soft tissue mass at the mid-occipital region with intracranial extension through a calvarial defect in keeping with an atretic occipital cephalocele

Case Discussion

MRI features of a Dandy-Walker malformation with associated dysgenesis of the corpus callosum, and atretic cephalocele.

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