The prognosis of children with hydrocephalus and congenital heart disease.

The association of congenital hydrocephalus and heart disease in children is infrequent, but may present considerable dilemmas in management. This report describes the treatment and prognosis of 11 children with both clinical problems. There were 5 males and 6 females. Hydrocephalus occurred following aqueductal stenosis in 5 children and the Dandy-Walker malformation in 3. Three children were diagnosed with idiopathic hydrocephalus. Ten children underwent cerebrospinal fluid diversion procedures for control of hydrocephalus. Five children received pharmacological therapy for cardiac disease; 4 children required surgical correction. Two children died from medical conditions; 2 families declined treatment. Follow-up from 2 to 7 years in the remaining 7 children demonstrated moderate or severe neurodevelopmental disability in 5. One child at 2 years of age showed borderline developmental disability while 1 child is developing normally at 10 years of age. Overall the occurrence of symptomatic hydrocephalus and heart disease in the perinatal period resulted in mortality or neurodevelopmental disability in 9/11 children.