Moyamoya disease associated with renovascular hypertension

Findings at the autopsy of a 7-year-old Japanese girl with moyamoya disease associated with renovascular hypertension are reported. There was disruption of a saccular aneurysm located at the junction of the left posterior cerebral and the posterior communicating arteries. Intimal thickening of carotid, coronary, and renal arteries, similar to findings in cases of intimal hyperplasia of fibromuscular dysplasia, was observed. These findings indicate that moyamoya disease may actually be an intracranial manifestation in a systemic arterial disorder.