MOYAMOYA DISEASE: Lessons from a Fatal Intracerebral Haemorrhage in a Young Patient Who Did Not Undergo Revascularisation

This report describes a fatal case of moyamoya disease to illustrate the consequences of not undertaking surgical revascularisation. 

A 13-year-old boy with no previous specialist follow-up was admitted with status epilepticus (Glasgow Coma Scale score 7). His history revealed progressive moyamoya disease diagnosed six years earlier, complicated by two ischaemic strokes, and managed solely with aspirin. Emergency non-contrast brain computed tomography demonstrated a massive intracerebral haemorrhage with intraventricular extension. Despite intensive care management including endotracheal intubation and external ventricular drainage, the clinical course was rapidly fatal. This tragic outcome highlights the catastrophic natural history of moyamoya disease in the absence of surgical revascularisation. It underscores the critical need for early detection, systematic referral to expert centres, and structured multidisciplinary management to prevent lethal complications. Surgical revascularisation remains the cornerstone disease-modifying therapy. 

Keywords: Moyamoya disease; Intracerebral haemorrhage; Child; Surgical revascularisation; Death; Case report.