Time trends and demographics of deaths from congenital hydrocephalus in children in the United States: National Center for Health Statistics data, 1979 to 1998

J Neurosurg. 2005 Aug;103(2 Suppl):113-8. doi: 10.3171/ped.2005.103.2.0113.


Object: Congenital hydrocephalus has an estimated population incidence of 0.2 to 0.8/1000 live births. With improvements in techniques for cerebrospinal fluid shunting, treatment of hydrocephalus has become safe and routine, yet data describing mortality from congenital hydrocephalus or demonstrating improvements in mortality with the advent of modern treatment are scarce. The authors' analysis sought to rectify this situation.

Methods: The authors performed an electronic search of National Center for Health Statistics death certificate databases to identify deaths from 1979 to 1998 attributed to congenital hydrocephalus, spina bifida with hydrocephalus, and acquired hydrocephalus (both obstructive and communicating) in all children in the US aged 1 day to 20 years. Mortality rates were defined as deaths per 100,000 person-years and were analyzed for differences on the basis of age, race, sex, and year. The authors identified 10,406 deaths attributed to childhood hydrocephalus within the 20-year study period. The overall mortality rate was 0.71 per 100,00 person-years. Mortality rates were highest in infants, with 3979 deaths; they were similar between girls and boys. Compared with white infants, black infants had higher relative risk (RR) for death caused by congenital hydrocephalus (RR 1.46, p value < 0.0001) and acquired hydrocephalus (RR 2.58, p value < 0.0001) but not for that caused by hydrocephalus with spina bifida (RR 0.65, p value < 0.0001). From 1979 to 1998, the mortality rate due to congenital hydrocephalus declined 66.3%, from spina bifida with hydrocephalus it declined by 30.4%, and from acquired hydrocephalus it declined by 67.5%.

Conclusions: Mortality rates from childhood hydrocephalus have declined in US children over the previous 20 years. Black race is associated with higher mortality rates in infants for both congenital and acquired hydrocephalus, whereas sex has no effect.

MeSH terms

  • Adolescent
  • Age Distribution
  • Black People / statistics & numerical data
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Hydrocephalus / ethnology
  • Hydrocephalus / etiology
  • Hydrocephalus / mortality*
  • Infant
  • Male
  • National Center for Health Statistics, U.S.
  • Risk
  • Spinal Dysraphism / complications
  • United States / epidemiology
  • White People / statistics & numerical data