Repeat bypass surgery for intracranial hemorrhage 30 years after indirect bypass for moyamoya disease

A 39-year-old man had been diagnosed with moyamoya disease and underwent a bilateral encephalo-duro-arterio-synangiosis(EDAS)intervention at the age of 9 years. During the 30 years after his bilateral EDAS, he experienced no cerebrovascular events. However, at age 39, he suddenly presented with mild consciousness disturbance and vomiting and was transferred to a local hospital. Brain CT showed an intracerebral hemorrhage associated with ventricular hematoma. He was referred to our hospital for further investigation and treatment. Cerebral angiography showed faint collaterals through the site of the 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 him with delayed cerebral hemorrhage due to delayed rupturing of fragile moyamoya vessels after indirect bypass. The patient underwent a repeat bypass surgery(STA-MCA anastomosis and encephalo-duro-myo-arterio-pericranial synangiosis;EDMAPS)on the right side. He showed improvement in cerebral hemodynamics after surgery, and has since remained free from cerebrovascular events. Hemorrhagic events occurring a very long time after indirect bypass surgery in pediatric-onset moyamoya disease are rare. In such cases, a lifelong follow-up strategy may be necessary. Repeat bypass surgery may be a powerful tool to prevent such hemorrhagic events.