Congenital hydrocephalus--prevalence, prenatal diagnosis and outcome of pregnancy in four European regions

Eur J Paediatr Neurol. 2010 Mar;14(2):150-5. doi: 10.1016/j.ejpn.2009.03.005. Epub 2009 May 2.


Objective: To describe prevalence, prenatal diagnosis and outcome for fetuses and infants with congenital hydrocephalus.

Methods: Data were taken from four European registries of congenital malformations (EUROCAT). The registries included are based on multiple sources of information and include information about livebirths, fetal deaths with GA > or = 20 weeks and terminations of pregnancy for fetal anomaly (TOPFA). All cases from the four registries diagnosed with congenital hydrocephalus and born in the period 1996-2003 were included in the study. Cases with hydrocephalus associated with neural tube defects were not included in the study.

Results: Eighty-seven cases with congenital hydrocephalus were identified during the study period giving an overall prevalence of 4.65 per 10,000 births. There were 41 livebirths (47%), four fetal deaths (5%) and 42 TOPFA (48%). Nine percent of all cases were from a multiple pregnancy. Additional non-cerebral major malformations were diagnosed in 38 cases (44%) and karyotype anomalies in eight cases (9%). Median GA at TOPFA was 21 weeks. Among livebirths 61% were diagnosed prenatally at a median GA of 31 weeks (range 17-40 weeks) and median GA at birth was 37 weeks. Fourteen liveborn infants (34%) died within the first year of life with the majority of deaths during the first week after birth.

Conclusion: Congenital hydrocephalus is a severe congenital malformation often associated with other congenital anomalies. CH is often diagnosed prenatally, although sometimes late in pregnancy. A high proportion of affected pregnancies result in termination for severe fetal anomaly and there is a high mortality in livebirths.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / epidemiology
  • Denmark / epidemiology
  • Female
  • Fetal Diseases / epidemiology
  • Head / abnormalities
  • Humans
  • Hydrocephalus / diagnosis*
  • Hydrocephalus / epidemiology*
  • Infant
  • Infant, Newborn
  • Karyotyping
  • Male
  • Pregnancy
  • Pregnancy Complications / epidemiology
  • Pregnancy Outcome
  • Prenatal Diagnosis*
  • Prevalence
  • Registries
  • Switzerland / epidemiology
  • United Kingdom / epidemiology