Severe headache as the only symptom of long-standing shunt dysfunction in hydrocephalic children with normal or slit ventricles revealed by computed tomography.

Four patients aged 11, 11, 8 and 18 years, shunted in early childhood for hydrocephalus, were followed in the outpatient clinic for severe headache from 3 months to 2 years. The headache lasted for days and up to a week, was usually severe, and was often followed by screaming attacks or aggressive behavior. In all patients repeated computed tomography disclosed a normal ventricular system or slit ventricles. The intracranial pressure (ICP) was measured in all four patients and showed an increased intracranial steady-state pressure between 20-50 mmHg, plateau waves and, in two of the patients, a very high intracranial pulse-pressure amplitude. At operation all patients had dysfunction of the shunt (most often of the distal end). In three patients ICP monitoring post-operatively showed normal intracranial pressure without any abnormal increments from the normal steady-state pressure. Thus, severe headache can be the only symptom of long-standing shunt dysfunction, even without ventricular dilation. In shunted hydrocephalic children complaining of headaches, ICP measurement is highly recommended.