Case contributed by Yaïr Glick
Diagnosis almost certain


Congenital hydrocephaly - assessment before decision on shunting.

Patient Data

Age: 5 months
Gender: Male

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.

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