Fetal surgery for hydrocephalus: successful in utero ventriculoamniotic shunt for Dandy-Walker syndrome

The diagnosis of fetal hydrocephalus based on dilation of the ventricular system presents a broad range of management decisions. The options are presented and a case of Dandy-Walker syndrome managed by fetal ventriculoamniotic shunt placement is presented as an example. Under ultrasonic guidance, a shunt was placed at 30 weeks' gestation by later newborn Dubowitz examination. Delivery was delayed for five weeks, one to two weeks following probable shunt malfunction, after achieving fetal lung maturation. Follow-up six months after definitive neonatal ventricular shunting and three weeks after shunt revision revealed a socially active male infant with a motor development index of 87 and a psychomotor development index of 95. Potential advantages of fetal surgery including achievement of term gestation are presented. Proposed guidelines for determining the benefit of such procedures are also presented.