Abstract

Objective: To evaluate and identify the risk factors associated with the pathogenesis of congenital hydrocephalus in a large specific population.

Methods: An International Classification of Diseases (ICD)-9 database search of patients with congenital hydrocephalus treated at the University of Mississippi Medical Center between 1998 and 2007 was performed. All recruited patients were interviewed, assessing maternal age, onset of prenatal care, geographic location of pregnancy, maternal diabetes and chronic hypertension, pregnancy induced hypertension, pre-eclampsia, eclampsia, single or multiparous gestation, maternal alcohol, tobacco and drug use, infection and trauma during gestation, trauma or sexually transmitted disease at parturition, and other family members with hydrocephalus.

Results: In this 10 year retrospective study, several significant risk factors were identified among 596 well defined cases of congenital hydrocephalus. The identified risk factors included lack of prenatal care, multiparous gestation, maternal diabetes, maternal chronic hypertension, maternal hypertension during gestation and alcohol use during pregnancy. Of these patients with congenital hydrocephalus, 12.1% identified an additional family member also diagnosed with hydrocephalus. No differences in risk factors were identified between sporadic and familial congenital hydrocephalus cases except for an increased incidence of multiparous pregnancies and prenatal care in the first trimester in familial cases.

Conclusions: A number of key risk factors have been identified to be strongly associated with the development of congenital hydrocephalus in an infant. The prevalence of familial patterns of inheritance for congenital hydrocephalus suggests a broader role for genetic factors in the pathogenesis of congenital hydrocephalus.