PATHOPHYSIOLOGY AND POSTNATAL OUTCOME OF FETAL HYDROCEPHALUS

At the National Kagawa Children's or Kobe University Hospital, 24 cases of fetal hydrocephalus were managed between 1982 and 1988. There were 8 simple, 11 dysgenetic, and 5 secondary cases of hydrocephalus, and the fetal age at diagnosis ranged between 24 and 40 weeks of gestation (average 33.4 weeks). All were diagnosed using ultrasonography, with either magnetic resonance imaging or whole-body computed tomography, additionally performed in 10 patients to determine their usefulness in evaluating the morphology. Four patients underwent transabdominal or transvaginal cephalocentesis in the prenatal period and intracranial pressure was measured during the drainage of cerebrospinal fluid in two of these. Postnatal outcome was analyzed for each type of hydrocephalus. The results suggested that in such cases the fetal brain is subjected to extremely high intracranial pressures resulting from a mixture of hydrocephalic pressure and intermittent uterine constriction. Immediately after birth, the biparietal diameter was found to be increased by an average of 7.7 mm and the hydrocephalic state was transformed into the neonatal type characterized by macrocephaly and a relatively low intracranial pressure. Overall mortality was 25% and 16 of the 24 infants underwent the postnatal shunt procedure, largely at the neonatal stage. The follow-up period varied from 4 months to 6 years (average, 25.8 months for nonfatal cases) and the mean intelligence or developmental quotient was 45.2. There were no significant differences in postnatal outcome between the three major types of fetal hydrocephalus. Findings revealed that the length of the gestation period after the diagnosis of hydrocephalus has a significant effect on outcome (P<0.01). Based on these results, it is suggested that fetal hydrocephalus may be extremely hypertensive and that impairment of neuronal functional development accompanying its prenatal progression can be irreversible. © 1990 Springer-Verlag.