Presentation
18 month old with large head circumference and failure to meet developmental milestones.
Patient Data
Age: 1 year
Gender: Male
From the case:
Dandy Walker malformation
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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 edema.