Clinical Features

Most infants with congenital hydrocephalus are stillborn or die soon after birth, but the lives of some are prolonged. The head, if not enlarged at birth, grows at an abnormal rate. The continuing enlargement of the ventricles is initially compensated by separation of the sutures and distension of the fontanels. As the hydro-cephalus progresses, the infant becomes lethargic, devel-

figure 13.33

Congenital hydrocephalus due to occlusion of the aqueduct of Sylvius in a 3-year-old boy. A. Enormously enlarged head, small-appearing face, and setting-sun position of the eyeballs. B. Basal aspect of the brain shows huge, collapsed cerebral hemispheres; thin, distended floor of the third ventricle; and a small cerebellum. C. The cerebral wall is less than 1 cm thick. D. Membranous occlusion of the aqueduct (cresyl violet).

figure 13.33

Congenital hydrocephalus due to occlusion of the aqueduct of Sylvius in a 3-year-old boy. A. Enormously enlarged head, small-appearing face, and setting-sun position of the eyeballs. B. Basal aspect of the brain shows huge, collapsed cerebral hemispheres; thin, distended floor of the third ventricle; and a small cerebellum. C. The cerebral wall is less than 1 cm thick. D. Membranous occlusion of the aqueduct (cresyl violet).

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ops spasticity of the legs, and abducens palsy, and, if the pressure is not relieved by a shunting procedure, papill-edema and optic atrophy develop. The face appears small in relation to the enormously enlarged head, and the eyeballs deviate downward, producing the setting-sun sign (Fig. 13.33). Cranial ultrasound demonstrates the hydrocephalus at 15 to 18 weeks of gestation, and MRI identifies the underlying pathology postnatally.

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