The Family History of "Simple" Congenital Hydrocephalus

A longitudinal study was carried out on the family history of 270 babies with uncomplicated congenital hydrocephalus. They had 453 siblings of whom 5 (11.1 per 1,000) had congenital hydrocephalus and 9 (19.9 per 1,000) had neural tube defects. These data, together with those obtained from the literature, indicate that the aetiology of the two conditions is probably the same in many cases, although there are unusual exceptions in special families, even apart from families with the sex-linked recessively inherited hydrocephalus. These findings suggest that the parents of children with uncomplicated hydrocephalus should have adequate antenatal tests for the detection of neural tube defects as well as for hydrocephalus and may need similar prophylactic treatment as do the parents of children with spina bifida and anencephaly.