Dandy-Walker malformation with callosal dysgenesis and atretic occipital cephalocele
Diagnosis almost certain
Presentation
Occipital swelling at the midline since birth.
Patient Data
Age: 1 month
Gender: Female
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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.