Hydranencephaly

Case contributed by Assoc Prof Frank Gaillard

Presentation

Large head and neurologically abnormal.

Patient Data

Age: 4 days old
Gender: Male

Cranial ultrasound

Ultrasound

The majority of the skull is filled with fluid. Cerebral tissue is seen centrally (~thalami) and posteriorly (occipital lobes and cerebellum). No frontal or parietal lobes are identifiable. Falx is noted in the midline, and it appears normal. 

CT brain

CT

Non contrast CT demonstrates marked enlargement of the skull with no anterior circulation cerebral cortex identified. Parts of the occipital lobes, the thalami and the posterior fossa appear preserved. The falx is present. 

Case Discussion

Typical appearance of hydranencephaly, which is incompatible with life. 

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