Introduction: The aim was to describe the prevalence and to estimate the prognosis of congenital hydrocephalus (HC) in fetuses and children.
Material and methods: Data for the study were taken from the Eurocat Register of Congenital Malformations for the County of Funen and from medical records. The study includes liveborn, intrauterine deaths, and induced abortions. All cases with HC born in the County of Funen in the years 1986-1998 were included. The followup period is three years after birth.
Results: The prevalence of HC was 0.4/1,000 births. There were 29 cases of HC, out of which 21 were liveborn. 41% had associated malformations, syndromes and/or chromosome abnormalities, and mortality of these compared to cases with isolated HC were significantly increased (p < 0.05). 18 children had shunt surgery and 12 children had one or more reoperations. At the age of three, four children had died, 12 had neurological problems related to their HC, and five children were described as normal.
Discussion: We found high mortality and morbidity in fetuses and children with HC. Mortality was significantly increased if associated malformations, syndromes and/or chromosome abnormalities were present. After prenatal diagnosis of HC it is important to look carefully for other malformations and to perform a karyotype before information about the prognosis of the fetus is given to the parents.