Sudden Death Due to Vestibular Schwannoma: Caution in Emergent Management

Objective: To enhance understanding of the possible dangers of large vestibular schwannomas and their potential to cause sudden death through rare situations such as brain herniation precipitated by cerebrospinal fluid pressure shifts. Patient: An otherwise healthy 32-year-old woman with a magnetic resonance imaging demonstrating a cystic cerebellopontine angle mass, presumptive for vestibular schwannoma, experienced sudden onset of headaches, nausea, and vomiting. Intervention: The patient was admitted to the intensive care unit, started on anticonvulsants, investigated with serial computed tomography (CT) scans, and then elective intubation and ventriculostomy when her condition worsened. Main Outcome Measure(s): Clinical and radiological findings, as well as outcome of interventions. Results: In the first hours of admission, the patient underwent serial CT scans that demonstrated fourth ventricle compression and hydrocephalus. When the patient's condition worsened, elective intubation was undertaken and an external ventricular drain was inserted. Very high intracranial pressures were noted despite cerebrospinal fluid drainage. Postoperative CT and magnetic resonance imaging demonstrated ascending transtentorial and tonsillar herniation as well as scattered cortical infarcts. Brain death was determined and care was withdrawn 5 days later. Conclusion: This is a very rare patient report of sudden death caused by a large vestibular schwannoma despite assertive emergent management. There was no intratumoral or intracranial haemorrhage. Brain death was likely a result of ascending transtentorial herniation that can occur with large posterior fossa tumors and be exacerbated by cerebrospinal fluid shifts after ventriculostomy.