Severe hydrocephalus (antenatal)

Case contributed by Abhijit Chikhlikar
Diagnosis probable

Presentation

8 months of amenorrhoea

Patient Data

Age: 30 years
Gender: Female
ultrasound

Ultrasound of the abdomen demonstrates a foetus. Intracranially there is mostly fluid, with only a thin rim of cerebral tissue. The falx cerebri is present.

Case Discussion

The differential for such an appearance is: 

  • hydranencephaly
    • thalami are often visible and are not fused
    • not associated with midline facial abnormalities
    • no cortex present or sometimes small islands of tissue
  • severe hydrocephalus
    • falx usually present, but may be absent due to severe long-standing hydrocephalus 
    • bilateral choroid plexus 
    • thalami not fused
    • not associated with midline facial abnormalities
  • alobar holoprosencephaly + / - very large dorsal cyst of holoprosencephaly
    • usually coexisting midline facial abnormalities
    • no falx
    • residual cortical tissue often has a cup or pancake morphology, fused across the mid-line anteriorly

In this case there appears to be a thin rim of cerebral tissue compressed up against the inside of the skull, favouring hydrocephalus. 

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