[A case of adult moyamoya disease progressed after vascular reconstructive surgery]

We report an adult onset patient with moyamoya disease showing acute progress after contralateral vascular reconstructive surgery. A 47-year-old female developed cerebral infarction in the left corona radiata. A magnetic resonance (MR) angiography and a cerebral angiogram revealed severe stenosis extending from the terminal portion of left internal carotid artery (ICA) to the M1 portion. The right ICA showed slight stenosis. We performed direct bypass surgery (STA-MCA anastomosis) on the affected left side. MR angiography 1 month after surgery revealed the progressive stenosis of the C1 portion of the right ICA. While measurement of cerebral blood flow (CBF) showed a slight impairment of vascular reactivity to acetazolamide loading in the region of the right MCA, we continued without vascular reconstructive surgery for the right side because there was no ischemic attack. The patient had a transient sensory disturbance of the left upper extremity 16 months after surgery. MR angiography and a cerebral angiogram revealed more progressive stenosis extending from the right ICA to the M1 portion. CBF study showed a low CBF at rest and a negative response to acetazolamide loading in the region of the right MCA. Direct bypass surgery was performed on the right hemisphere. Follow-up study revealed an increment of rest CBF and improvement of vascular reactivity. We underlined the necessity for careful postoperation observation of progressive contralateral arterial stenosis using MR angiography and CBF study in adult onset patients with moyamoya disease.