Acute obstructive hydrocephalus and sudden death in children

Sudden death from obstructive hydrocephalus related to intracranial neoplasms has rarely been reported in the pediatric literature. We sought to review the presenting signs and symptoms of acute hydrocephalus resulting from intracranial mass lesions to guide clinicians in the early identification of these potentially reversible lesions.

All cases of sudden unexpected death attributable to obstructive hydrocephalus that occurred from 1990 through 1994 at the Hospital for Sick Children, Toronto, were retrospectively reviewed.

During the study period, seven children, ages 10 months to 15 years, died unexpectedly with acute obstructive hydrocephalus. Six children were apparently normal, and none had any known neurologic disease. All patients had a previously undiagnosed intracranial tumor located at a critical site for CSF flow: colloid cyst(n = 2), astrocytoma (n = 2), ependymoma (n = 2), suspected lymphoma (n = 1). Presenting features included vomiting in all cases, vomiting for longer than 2 weeks in three, headache in four, and lethargy in three. Five patients were misdiagnosed with viral illnesses, including three with presumed gastroenteritis who received intravenous rehydration therapy Focal gastrointestinal signs were absent.

This case series highlights a life-threatening but misleading presentation of intracranial tumors. The diagnosis of gastroenteritis should be made cautiously when headache and vomiting occur in the absence of focal intestinal complaints. A history of vomiting exceeding a few days' duration warrants further investigation. Persistent lethargy should be considered a neurologic rather than a nonspecific clinical sign. Heightened awareness of this neurosurgical emergency may lead to swift intervention and potential reversibility with diversion of CSF.