The natural history of hydrocephalus: Detailed analysis of 182 unoperated cases

Abstract

Over a 20-year period 182 unoperated cases of hydrocephalus seen by one surgeon were followed up. Of these 81 cases (46%) were found to be alive with the hydrocephalus arrested spontaneously, nine remained progressive and 89 (49%) had died. Three remained untraced but were presumed alive. The series is representative of the disease in the community after the age of 3 months, and the life expectancy (actuarial) of the survivors into adult life is 26%. The age of onset is dependent upon the aetiology of the condition-the natural arrest occurs between 9 months and 24 years, but the patients remain at risk. Of the arrested survivors 73% were educable (I.Q. more than 50) and 38% had I.Q.s within the normal range (more than 85). Little or no physical handicap was seen in the 39 patients and only 11 had severely defective vision or were blind. No correlation was found between intelligence, head size, cortical thickness and duration of progressive disease. A high negative correlation existed between intelligence and physical disability, and neither improved with age. Many of the children show the classical features of brain damage which is thought to be due more to the original brain insult than to cortical thinning. Emotional disturbances were relatively few. It is concluded that surgery should be undertaken in rapidly progressive hydrocephalus in order to prevent further brain damage, but caution must be exercised in arresting or arrested cases.