Clinical outcome after near-fatal late shunt complication in hydrocephalus

Abstract

Shunt revision is inevitable in most shunted hydrocephalic children but the revisions rarely affect the prognosis markedly. This report presents the case of an otherwise healthy boy who, after an uneventful gestation and birth, manifested hydrocephalus in the neonatal period and was shunted at the age of 3 months. His subsequent development and school performance were normal until the age of 10 years, when he developed his first episode of shunt dysfunction. During the emergency shunt revision, intraventricular haemorrhage occurred, causing acute neurological symptoms and signs. In the promptly undertaken reoperation external drainage was inserted but accidentally without a functioning air needle. The intracranial pressure (ICP) rose to 40 cm H2O before correction. These incidents were followed by a 4-month period of unstable shunt function (12 reoperations) and deteriorating neurology. When stable shunt function was eventually achieved, he had severe contractures, minimal active motor function and no active speech. During a 2-year period of rehabilitation, he recovered almost normal motor function and normal intellectual function. Memory functions, vigilance and concentration span remained clearly subnormal. He returned to normal school and his performance is average. It is concluded that the relatively favourable outcome is the probable result of reversible partial herniation and increased ICP.