Current prognosis in fetal ventriculomegaly

A review of 51 cases referred for evaluation of fetal ventriculomegaly revealed adequate follow-up data in 40 patients. Three of the fetuses were electively aborted and 37 were delivered. There were no cases of death in utero. Of the 37 infants who were delivered, 26 (70%) were treated with shunt placement for neonatal hydrocephalus. The reasons for nontreatment included: inaccurate diagnosis, resolution of hydrocephalus by the time of delivery, neonatal death, and parental wishes. Survival time in the 26 shunt-treated patients ranged from 5 days to 14 years (average 4.25 years); five of these patients died within the first 2 weeks of life. Among the 26 treated patients, satisfactory cognitive ability was found in 10 (38%). Of the seven surviving nontreated infants, satisfactory cognitive ability was demonstrated in six (86%). Children with myelomeningocele displayed cognitive development similar to that in patients without myelomeningocele. The findings suggest that, of patients with in utero diagnosis of ventriculomegaly, approximately one-half survive (4-year average follow-up interval) and 38% of the survivors treated with shunt insertion have normal cognitive development. No subgroup likely to benefit from in utero treatment of ventriculomegaly was identified. Associated central nervous system or systemic malformations were identified in 26 (70%) of the 37 who came to delivery. This community-acquired series, not collected from a high-risk obstetrical-perinatal service, may reflect the general experience of the neurosurgeon consulting in such cases.