Acute Fulminant Cerebral Edema: A Case Series at a Large Pediatric Tertiary Center

Acute fulminant cerebral edema is a poorly understood but serious neurologic condition resulting in profound neurologic disability or mortality. Here we present a case series of four children that presented to our institution with new neurologic dysfunction and neuroimaging evidence of cerebral edema. Ages ranged from 2 to 7 years with the most common presenting features being altered mental status, vomiting, and/or seizure. Two patients had normal head computed tomography, but follow-up imaging performed within 15 hours demonstrated fulminant edema. One patient was positive for influenza, and one had neuropathology consistent with acute hemorrhagic leukoencephalitis. Two had no identified cause. Treatments included broad-spectrum antibiotics and acyclovir, hyperosmolar agents, intravenous steroids, and decompressive craniectomy. Only one patient survived. Acute encephalopathy complicated by fulminant cerebral edema is a rapidly evolving and often fatal neurologic condition. Early identification with neuroimaging and intervention may improve outcomes. Repeat neuroimaging should be considered if initial imaging is normal but there is persistent or progressive unexplained encephalopathy. Further studies are required to determine optimal diagnostic and management strategies.