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February 1970

Picture of the Month

Author Affiliations

Boston; Los Angeles
From the University of California Medical Center, Los Angeles.

Am J Dis Child. 1970;119(2):139-140. doi:10.1001/archpedi.1970.02100050141009

Denouement and Discussion 


Manifestations  Infants with hydranencephaly may have no symptoms during the first few months of life as they are then functioning at a midbrain level. The usual newborn reflexes, Moro, tonic neck, rooting, and sucking, are present and active. This is not so if there is increased intracranial pressure associated with the hydranencephaly. With increasing intracranial tension, the head size increases, the fontanels bulge, and a setting sun sign appears. The infant becomes irritable, develops spastic quadriparesis, fails to gain, and usually succumbs to an infection.The signs of increasing pressure indicate the presence of intracranial pathology which must be differentiated from hydrocephalus or subdural effusion. Transillumination is a valuable adjunct in this differential diagnosis. The child with hydranencephaly and head enlargement has very little cerebral tissue, and the transillumination is marked. In the child with hydrocephaly and early ventricular enlargement, the cortical tissue is sufficient to