Hydranencephaly is the accepted term for a congenital anomaly that consists of
complete or nearly complete abscence of the cerebral hemisphere, together with intact meninges and skull.
A four months old male infant was admitted to CNUH on June, 1976 with the complaints of enlarged head, vomiting and irritability. General examination at hospital revealed a normal appearing, who had a high pitched cry and whose weight was 6.27kg. The movements of head, extremities and eye ball was good. Cranial-sutures were separated, and the cranial bones seemed to be floating. Transillumination of head disclosed diffusion of light in all direction except a little area of opacity on occipital area. Plane skull films showed marked suture separation with enlarged fontanel.
On pneumoencepalography, the air occupied entire frontal, temporal and partially parietal area. Right carotid angiography showed anterior cerebral artery and middle :cerebral artery but were thin and elongated.
Because of the results obtained from transillunination of head, pneumoencephalography ,and right carotid angiography, diagnosis of hydranencephaly was made. Ventriculo-peritoneal shunt operation was performed and several weeks after, it was replaced with ventriculo-atrial shunt.
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