Hydranencephaly

Case contributed by Yaïr Glick
Diagnosis almost certain

Presentation

Congenital hydrocephaly - assessment before decision on shunting.

Patient Data

Age: 5 months
Gender: Male
ct

Hydranencephaly: monoventricle surrounded by rind of cerebrum; importantly, lack of fused anterior cerebral mantle and presence of falx cerebri (anteroinferior part) rule out alobar holoprosencephaly.
Substantial macrocephaly due to massive hydrocephalus.

Case Discussion

Infant with congenital macrocephaly due to hydranencephaly with active hydrocephalus.

Conservative approach exhausted, hydrocephalus still active despite treatment with acetazolamide. A decision was reached with the parents that at first, an externalized ventricular shunt shall be placed, then, after excess CSF has been drained out and intracranial pressure normalizes, the distal shunt will be internalized into the peritoneum.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.