Overt hydrocephalus at birth--origin and outcome.

Based on a survey of the population, which included 202 infants with infantile hydrocephalus those who had had overt hydrocephalus at birth were selected and analyzed with respect to origin and outcome. The present series consisted of 47 infants (23% of the total series), 83% of whom were born at term and 17% before term. In the study period 1967-1982, the prevalence was 0.12/1,000 births. Among infants with maldevelopment of the CNS and/or other organs, the mortality before 2 years of age was 37%, and among the survivors the rate of neurological sequelae was 88%. The corresponding figures for infants with uncomplicated hydrocephalus, i.e., without other maldevelopment, was 20% and 44%, respectively. The prognosis was especially poor for infants born before term. It was concluded that the prognosis was largely determined by maldevelopment affecting the CNS macro- or microarchitecture.