Intrauterine hydrocephalus and ventriculomegaly: associated anomalies and fetal outcome.

Advances in fetal diagnostic techniques have opened many areas to prenatal anatomical scrutiny. Intrauterine hydrocephalus and ventriculomegaly are conditions which are readily diagnosed. Fetal intervention has been undertaken in humans in order to minimize the craniofacial disfigurement and to maximize the growth potential of the brain. To justify such an approach, the significance of all anomalies should be recognized prior to treatment. The authors have reviewed 41 cases of hydrocephalus diagnosed in utero in order to define associated anomalies and patient outcome. 75% of our personal series and 72% of the reviewed literature cases had other anomalies of the central nervous system. Other system malformations, some of which proved fatal, were seen commonly. Prenatal diagnostic techniques did not always reveal these additional problems. The outcome of these pregnancies is not good. Approximately one third of these fetuses have survived to be treated postnatally and to be followed up clinically. Only 7.5% of this series were felt to have attained normal developmental milestones. The remainder of the survivors have various focal and/or global cerebral deficits.