Slitlike Ventricle Syndrome: A Life-Threatening Presentation

Severely increased intracranial pressure can be life-threatening in shunted children who do not experience ventricular enlargement. This condition is termed normal ventricular hydrocephalus and represents the most severe form of slit ventricle syndrome. CASE REPORT:A 7-year-old girl with a repaired lumbosacral myelomeningocele and shunted at birth who presented with headache, vomiting, seizure, and deterioration of level of consciousness was admitted to the pediatric intensive care unit. Because her ventricles were small to slitlike on cranial computed tomographic (CT) scan, the shunt was presumed to be working. Although the cerebrospinal fluid analysis was normal, she received initial empirical treatment of viral encephalitis. Twenty-four hours after admission, she evolved with apnea and bradycardia, requiring ventilatory support. Repeated CT scans were unchanged from one study to the next. After 48 hours, her condition worsened, and cerebrospinal pressure during lumbar puncture reached more than 30 mm Hg despite the serial CT scan disclosing no ventricular enlargement. She underwent a shunt revision that showed that the catheter was occluded and had adhered to the ventricular wall. The shunt was replaced, resulting in dramatic neurological improvement. This report highlights a life-threatening condition involving chronically shunted children who present severe intracranial hypertension without ventriculomegaly and may often be neglected or unrecognized by emergency physicians or general neurosurgeons.