CASE HISTORY

Spontaneous normal delivery on 18.12.58. There was considerable difficulty with the delivery of the shoulder. The infant was noticed to have a cystic protrusion of Meninges through sagittal and lambdoid sutures with a defect in the cranium bones in these regions. He thrived but developed an infection of the cystic protrusion which proved to be a Pseudomonas pyocyanea infection. The child also developed a salmonella infection of the gastro-intestinal tract. Investigation of the urine for amino acids showed the presence of glutamine only. He was considered unsuitable for surgery. 20.2.59 The child had a convulsion but there did not appear to be any other CNS signs present. He developed a pyrexia, deteriorated gradually and died on 17.9.59. Left carotid arteriogram showed the anterior and middle cerebral arteries outlines but a bizarre pattern was demonstrated. The firstpart of the anterior cerebral artery instead of being convexforwards was convex backwards. The middle cerebral group were depressed. There was a general tendency of all the vessels to be posteriorly displaced and inclined towards the defect in the skull. Some of the vessels entered the meningoencephalocele.

PATHOLOGY

The specimen consists of one half of the brain with its encephalocele. Viewed from the front, the external appearance of the encephalocele can be clearly seen. From the back of the specimen, it can be seen that the skin had fused with the underlying nervous tissue and the whole had become locally infected, the infection being shown by the opaque, yellow, irregular spots. The condition is well localized ad there is no spreading meningitis.