Chronic ("normal pressure") hydrocephalus in childhood and adolescence. A review of 16 cases and reappraisal of the syndrome

Abstract

"Normal pressure" hydrocephalus (NPH) is generally considered to be a disorder of the adult and geriatric population. Only a few reports have described the possible occurrence of this condition in children. A series of 16 patients aged less than 20 years forms the basis of the present report. Among these 16 patients, 11 had a clearly identified etiologic factor and 7 had had a shunt previously implanted. The majority of patients exhibited at least two elements of the adult's triad of psychomotor retardation (14 cases) and/or psychotic-like symptoms (4 cases), gait anomalies (8 cases), and sphincter disturbances (3 cases). Six patients had their intracranial pressure (ICP) monitored. ICP values were estimated to be within the normal limits for age. All the 16 patients underwent shunting or shunt revision. Surgical results were as follows (mean follow-up 20 +/- 17.2 months): a good response to shunting was obtained in 12 cases ("cured": 5, improved: 7), while the other 4 patients failed to improve. It seems likely that associated parenchymal disorders have played a major role in therapeutic failures. In children showing ventricular dilation on computed tomographic (CT) analysis and a clinical picture of subtle psychomotor deterioration, it may be difficult to distinguish an active disorder of the CSF dynamics from "arrested hydrocephalus." Since intracranial manometry cannot be undertaken as a routine procedure, less invasive methods such as cerebrospinal fluid (CSF) tap test, psychometric, or urodynamic tests deserve special attention as reliable predictors of outcome after shunting. Because most patients undergo shunting without prior assessment of their CSF pressure, the term "chronic hydrocephalus" is proposed as an alternative designation to "NPH," since there is little argument for maintaining an instrumentally based definition of the syndrome.