Abstract

Twenty-one premature infants with various underlying conditions developed hydrocephalus. The characteristic clinical pictures as well as the therapeutic problems are discussed. The causative underlying conditions included intraventricular hemorrhage in the neonate or fetus, broncho-pulmonary dysplasia, congenital malformations such as holoprosencephaly with chromosome anomalies, and others. The pathophysiological aspects were considered to be characterized by the underdevelopment of major cerebrospinal fluid pathway, extremely high intracranial compliance, and demyelinating process of the nervous system. The shunt problems in these patients with such low body weight were also unique, showing an extremely high incidence of postshunt hydrocele, shunt migration into the scrotum, shunt malfunction, slit-like ventricle, and isolated ventricles. Intensive management, combined with better control of the hydrocephalus with these concepts, will help to improve the long-term outcome of these premature infants.

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