堀 聡、柏崎大奈、秋岡直樹、浜田秀雄、桑山直也、黒田 敏
A 39-year-old male was diagnosed as moyamoya disease and underwent bilateral encephalo-duro-arterio synangiosis (EDAS) when he was 9 years old. During 30 years after bilateral EDAS, he experienced no cerebrovascular events. However, he presented with mild consciousness disturbance and vomiting, and was transferred to local hospital. Brain CT showed the intracerebral hemorrhage associated with ventricular hematoma. He was referred to our hospital for further investigation and treatment. Cerebral angiography showed faint collaterals through bilateral EDAS and development of basal moyamoya vessels. SPECT showed decreased cerebral blood flow (CBF) and cerebrovascular reactivity (CVR) in the right frontal lobe. We diagnosed as the delayed cerebral hemorrhage due to rupture of fragile moyamoya vessels very long after indirect bypass. He underwent repeat bypass surgery (STA-MCA anastomosis and encephalo-duro-myo-arterio-pericranial synangiosis; EDMAPS) on the right side. Cerebral hemodynamics improved after surgery. He has been free from cerebrovascular events after surgery. Hemorrhagic events very long after indirect bypass surgery in pediatric-onset moyamoya disease are rare. However, the lifetime follow-up strategy may be necessary. Repeat bypass surgery may be a powerful tool to prevent hemorrhagic events.