Dandy Walker malformation

Case contributed by Patan Academy of Health Sciences
Diagnosis almost certain

Patient Data

Age: 13 years
Gender: Female
ct

Large cystic area in the posterior fossa extending up to the fourth ventricle. Partial agenesis of the cerebellar vermis, which is superiorly placed. Cystic dilatation of the third and lateral ventricles. Hypodensity noted in periventricular white matter. Posterior fontanelle is still not fused.

Case Discussion

Dandy Walker malformation consists of a group of anomalies where there is a posterior fossa cyst that communicates with the fourth ventricle, as well as abnormal development of the cerebellar vermis.

In its classical presentation, Dandy Walker malformation consists of the triad of:

  1. Hypoplasia of the vermis and cephalad rotation of the vermian remnant.
  2. Cystic dilatation of the fourth ventricle extending posteriorly. Usually, the cerebellar hemispheres are displaced anterolaterally, but with a normal size and morphology.
  3. Enlarged posterior fossa with torcular-lambdoid inversion.

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