• Sarah Newman, J Tanner McMahon, Jason H Boulter, James G Malcolm, Juan M Revuelta Barbero, Joshua J Chern, Daniel L Barrow, and Gustavo Pradilla.
• Department of Neurosurgery, Emory University, Atlanta, Georgia, USA.
• Neurosurgery. 2022 Apr 1; 90 (4): 441446441-446.

BackgroundMoyamoya syndrome refers to a progressive stenosis of the internal carotid arteries and can be associated with sickle cell disease. These codiagnoses result in severe risk for stroke, even in patients on optimal medical management. Surgical revascularization has been shown to be safe in small case series.ObjectiveTo evaluate the efficacy of revascularization with direct comparison to a medically managed control group within a single institution.MethodsA retrospective cohort study of medically managed vs surgically revascularized patients with moyamoya syndrome and sickle cell disease was conducted. Demographic data and outcomes including the number of prediagnosis, postdiagnosis, and postrevascularization strokes were collected. Risk factors for stroke were identified using a binary logistic regression model, and stroke rates and mortality between groups were compared.ResultsOf the 29 identified patients, 66% were medically managed and 34% underwent surgical revascularization (50% direct and 50% indirect). Calculated stroke rates were 1 per 5.37 (medical management), 1 per 3.43 (presurgical revascularization), and 1 per 23.14 patient-years (postsurgical revascularization). There was 1 surgical complication with no associated permanent deficits. No risk factors for stroke after time of diagnosis were found to be significant.ConclusionThe results of this study demonstrate that revascularization is associated with a significant reduction in stroke risk, both relative to prerevascularization rates and compared with medical management. According to these findings, surgical revascularization offers a safe and durable preventative therapy for stroke and should be pursued aggressively in this patient population.Copyright © Congress of Neurological Surgeons 2022. All rights reserved.

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