• Rachael Mariadas, Shinuo Liu, Mugdha Sachdeva, Sunil Unnikrishnan, Hui Yuan Foong, and Marcus Stoodley.
• Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, New South Wales, Australia.
• World Neurosurg. 2023 Oct 1; 178: e65e71e65-e71.

BackgroundMoyamoya vasculopathy is a rare steno-occlusive cerebrovascular disorder presenting with ischemia or hemorrhage. There are racial and geographic differences in presentation and outcome. There is little information regarding moyamoya in Australia.MethodsMoyamoya patients undergoing surgery from 2001 to 2022 were studied retrospectively. The outcomes of revascularization surgery in adult and pediatric patients, with ischemic and hemorrhagic disease were analyzed, including functional outcomes, postoperative complications, bypass patency, and long-term rates of ischemic and hemorrhagic events.ResultsA total of 68 patients with 122 revascularized hemispheres and 8 posterior circulation revascularizations were included in this study. Eighteen patients were of Asian descent and 46 were of Caucasian origin. Presentation was with ischemia in 124 hemispheres and hemorrhage in six hemispheres. There were 92 direct, 34 indirect, and 4 combined revascularization surgeries performed. Early postoperative complications occurred in 3.1% (n = 4) of operations and delayed complications (infection, subdural hematoma) occurred after 4.6% (n = 6) of operations. Mean follow-up was 6.5 years (3-252 months). There was 100% patency of direct grafts at last follow-up. There were no hemorrhagic events following surgery and 1 new ischemic event 2 years after surgery. There was significant improvement in physical health functional outcomes at most recent follow-up (P < 0.05); mental health outcomes were not different between preoperative and postoperative assessments.ConclusionsThe majority of Australian moyamoya patients are Caucasian and the most common clinical presentation is ischemia. Revascularization surgery had excellent outcomes with very low rates of ischemia and hemorrhage, comparing favorably to the natural history of moyamoya vasculopathy.Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.

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