Trends in the Management of Adult Moyamoya Disease in the United States: Results of a Nationwide Survey

BackgroundMoyamoya disease is a rare, progressive, steno-occlusive disease of the distal branches of the carotid arteries associated with the development of a profuse basal collateral vascular network. In the United States, the disease occurs sporadically and literature on the topic in limited. With data lacking relative to moyamoya epidemiology and management, we report our survey results among a sample of neurovascular surgeons.MethodsA 16-question survey was sent as a hyperlink by electronic mail to 46 vascular neurosurgery centers and physicians who have published on this topic. Data included patient demographics, referral patterns, treatment, and follow-up.ResultsResponse rate was 70%. Of 32 respondents, 72% evaluated 10 or fewer adults per year, usually referred by a neurologist within their referral pattern. Fifteen (47%) respondents reported that the disease had a bilateral occurrence between 50% and 75% of the time. Most respondents (88%) reported that fewer than 25% of their patients had a previous surgery for the disease. Symptomatology (91%) was the criterion to recommend surgery. Nineteen (59%) respondents regularly assessed cerebrovascular reserve by the addition of acetazolamide challenge to their imaging method of choice, typically single photon emission computed tomography. Treatment was antiplatelets (55%) in asymptomatic adults and superficial temporal artery-to-middle cerebral artery bypass for >75% of symptomatic patients, as assessed by 48% of respondents. Yearly follow-up included cerebral angiography for both superficial temporal artery-to-middle cerebral artery bypass and indirect revascularization.ConclusionsOur survey results quantify epidemiologic trends of moyamoya disease in the United States. Finding a lack of consensus in management strategies, we conclude that collaborative efforts may help to create guidelines for the management of this puzzling entity.