Risk factors associated with the pathogenesis of congenital hydrocephalus were evaluated in a 10 year retrospective study of 596 cases identified at the University of Mississippi Medical Center between 1998 and 2007. Significant risk factors included lack of prenatal care, multiparous gestation, maternal diabetes, maternal chronic hypertension, pregnancy-induced hypertension, and alcohol use during pregnancy. Hydrocephalus was familial in 12% cases. Except for an increased incidence of multiparous pregnancies and prenatal care in the first trimester in familial cases, no differences in risk factors were identified between sporadic and familial congenital hydrocephalus. The prevalence of familial cases within this cohort is much higher than that reported in X linked congenital hydrocephalus (2-7%), and suggests that the strong genetic factor in etiology is attributed to non-X linked patterns of inheritance. 
COMMENT. Both genetic and environmental factors are involved in the pathogenesis of congenital hydrocephalus. Some risk factors identified in this study should be susceptible to preventive measures, including improved prenatal care and nutrition, avoidance of alcohol, and prompt treatment of hypertension.