Increased intraventricular pressure without ventriculomegaly in children with shunts: 'Normal volume' hydrocephalus

Five patients with shunt-dependent hydrocephalus were observed to have apparently normal ventricular size despite marked increases in ventricular pressure after shunt malfunction. Elastance (dP/dV) was determined in four of these patients by removng increments of cerebrospinal fluid and measuring the resulting pressure. These patients without ventricular enlargement and with markedly increased ventricular pressure had high elastance. This group of patients with 'normal volume' hydrocephalus had distal shunt occlusions, in contrast to previously reported patients with cephalic shunt obstructions after ventricular decompression. Initial shunting in early infancy, prolonged shunt dependency, and lack of recent shunt revision were common factors in these patients. Markedly elevated pressure with normal volume is a threatening clinical entity, requiring prompt surgical intervention.