Dandy Walker malformation

Case contributed by Dr Ian Bickle

Presentation

18 month old with large head circumference and failure to meet developmental milestones.

Patient Data

Age: 1
Gender: Male
MRI

Posterior fossa cystic abnormality communicating with the 4th ventricle with gross hydrocephalus.

Case Discussion

This is a classic case of a Dandy Walker malformation.  The most severe end of the Dandy Walker spectrum.

  • hypoplasia of the vermis and cephalad rotation of the vermian remnant
  • cystic dilatation of the fourth ventricle extending posteriorly 
  • enlarged posterior fossa with torcular-lambdoid inversion (torcular lying above the level of the lambdoid due to abnormally high tentorium

In addition in this case there is gross hydrocephalus as a consequence of the posterior fossa abnormalities, with periventricular high signal in keeping with trans-ependymal oedema.

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Case information

rID: 23241
Case created: 31st May 2013
Last edited: 23rd Jun 2017
Inclusion in quiz mode: Included

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