Clinical course of fetal hydrocephalus: 40 cases

The clinical course and outcome of hydrocephalus diagnosed in utero is not well understood. To approach this problem 40 cases were reviewed of intrauterine fetal hydrocephalus diagnosed with sonography, and follow-up information was obtained regarding them. Sonograms were evaluated for cerebral dimensions, biparietal diameter, brain mantle size, ventricular ratio, amount of amniotic fluid, and associated abnormalities. Neonatal brain sonograms and computed tomographic (CT) scans were reviewed also. Clinical charts were reviewed for maternal age and parity, referral source, family history, fetal age at diagnosis and delivery, mode of delivery, physical examination and/or autopsy findings, karyotype, amniotic alpha 1 fetoprotein level, cause of death, shunt placement after birth, and status of live infants. The observations indicate that the prognosis for fetal hydrocephalus is poor. Only six infants (15%) were alive after an average follow-up of 13 months. Three children were normal and the other three had neurologic abnormalities ranging from severe (paralysis and incontinence) to minimal (2-3 months delayed motor development). Thirty-four fetuses or neonates died. Nine families elected to terminate pregnancy. Ten opted for decompression at delivery for progressive hydrocephalus. Neural tube defects were present in 12 of 23 infants at delivery. Fourteen other infants had additional significant congenital abnormalities. Other abnormal sonographic findings included polyhydramnios (13 of 38), oligohydramnios or decreased fluid (nine of 38), neural tube defect (nine of 40), and other congenital abnormalities (nine of 40). These findings indicate that hydrocephalus diagnosed in utero by sonography is caused by a heterogeneous group of disorders. In general, the prognosis for normal development is poor. Individual prognoses, however, depend on the specific malformations and the interventions used.