Guideline sources The following summarized guidelines for the evaluation and management of moyamoya disease are prepared by our editorial team based on guidelines from the European Stroke Organisation (ESO/VASCERN 2023), the Japan Neurosurgical Society (JNS/JSS/MHLW 2022), the American Heart Association (AHA/ASA 2021), the American Society of Hematology (ASH 2020), the Ministry of Health, Labour and Welfare of Japan (MHLW/PTSOW-RC 2012), the Moyamoya Disease Working Group (MD-WG 2012), and the American College of Chest Physicians (ACCP 2012). 1 2 3 4 5 6 7 Guidelines 1. Diagnostic investigations Diagnostic imaging: As per ESO 2023 guidelines, consider obtaining hemodynamic assessment (CT, MRI, SPECT, positron emission tomography, or ultrasound) in the diagnostic evaluation of all patients with Moyamoya disease to help in decision-making and for further analysis in guiding future decisions. E Show 3 more https://web.pathway.md/diseases/recGXkYZld2sicLvi 1/5 6/30/23, 1:05 AM Moyamoya disease Pathway As per AHA 2021 guidelines, consider obtaining MRA or CT angiography of the intracranial large arteries and extracranial vertebrobasilar arterial system to identify Moyamoya disease in patients with ischemic stroke or TIA. C As per PTSOW-RC 2012 guidelines: Obtain cerebral angiography for a definitive diagnosis of Moyamoya disease. Establish a definitive diagnosis with MRI when the following findings are fulfilled on time of flight imaging conducted using a scanner with a magnetostatic intensity of 1.5 T (especially 3.0 T): stenosis or occlusion of the terminal portion of the intracranial internal carotid artery or proximal portion of the anterior and/or middle cerebral arteries abnormal vascular networks in the basal ganglia ( 2 visible flow voids in the basal ganglia at least unilaterally can be deemed as representing an abnormal vascular network) bilaterality of above findings. B Consider obtaining SPECT or positron emission tomography for the diagnosis and assessment of the severity of cerebral ischemia in patients with ischemic-type Moyamoya disease. C Genetic testing: Insufficient evidence to recommend variant screening of RNF213 p.R4810K. I Avoid obtaining systematic variant screening of RNF213 p.R4810K in patients with Moyamoya disease, regardless of ethnicity. D 2. Medical management Intravenous thrombolysis: consider administering intravenous thrombolysis with recombinant tPA under careful evaluation of the risk of hemorrhagic complication in the hyperacute phase of cerebral ischemia in patients with Moyamoya disease. C Antiplatelet therapy: As per ESO 2023 guidelines, consider initiating long-term antiplatelet therapy to reduce the risk of embolic strokes in patients with non-hemorrhagic Moyamoya disease. E As per JNS 2022 guidelines, consider initiating oral antiplatelet therapy as a medical treatment in patients with ischemic Moyamoya disease. C As per AHA 2021 guidelines, consider initiating antiplatelet therapy, typically aspirin monotherapy, for the prevention of ischemic stroke or TIA in patients with Moyamoya disease and a history of ischemic stroke or TIA. C As per ACCP 2012 guidelines, consider initiating aspirin as initial therapy in pediatric patients with acute ischemic stroke secondary to Moyamoya disease. C As per PTSOW-RC 2012 guidelines, initiate antiplatelet therapy in patients with Moyamoya disease. B Blood pressure control: https://web.pathway.md/diseases/recGXkYZld2sicLvi 2/5 6/30/23, 1:05 AM Moyamoya disease Pathway Consider reducing systolic BP in the acute stage of hemorrhagic Moyamoya disease, as in patients with spontaneous intracerebral hemorrhage, considering the development of cerebral ischemia. C Consider maintaining appropriate BP control in patients with postoperative cerebral hyperperfusion syndrome while considering the concomitant cerebral ischemia. C Non-operative management: consider offering conservative management in adult asymptomatic patients with Moyamoya angiopathy, except in patients with both cerebral hemodynamic impairment and silent ischemic lesions in the same cerebral region. E 3. Surgical interventions Surgical revascularization, indications: As per VASCERN/ESO 2023 guidelines, perform surgical revascularization (evidence only for direct superficial temporal artery-middle cerebral artery bypass) in case of cerebral hemodynamic impairment and the presence of choroidal collaterals in adult patients with Moyamoya disease with a hemorrhagic presentation. B Show 3 more As per JSS/JNS/MHLW 2022 guidelines: Consider performing surgical revascularization in patients with Moyamoya disease presenting with cerebral ischemic symptoms. C Consider performing revascularization surgery in patients with hemorrhagic Moyamoya disease, especially posterior hemorrhage, to prevent recurrent hemorrhage. C As per MHLW/PTSOW-RC 2012 guidelines: Consider performing revascularization in patients with hemorrhagic-type Moyamoya disease. C Perform surgical revascularization in patients with Moyamoya disease manifesting as cerebral ischemic symptoms. B Surgical revascularization (timing): consider waiting 6-12 weeks from an acute cerebrovascular event before performing surgery to reduce the rate of postoperative complications in patients with Moyamoya disease. E Show 3 more Surgical revascularization, settings: As per VASCERN/ESO 2023 guidelines, consider performing surgical revascularization in symptomatic and asymptomatic adult and pediatric patients with Moyamoya disease in a referral center and by a neurosurgeon with significant experience in surgical revascularization techniques. E As per ACCP 2012 guidelines, consider referring pediatric patients with Moyamoya to an appropriate center for consideration of revascularization. Surgical revascularization, technical considerations: As per VASCERN/ESO 2023 guidelines: https://web.pathway.md/diseases/recGXkYZld2sicLvi 3/5 6/30/23, 1:05 AM Moyamoya disease Pathway Consider performing direct/combined revascularization to reduce the risk of stroke in adult patients with Moyamoya disease. E Consider performing combined revascularization, whenever technically possible, to decrease the short-term risk of stroke in pediatric patients with Moyamoya disease. E As per ASA/AHA 2021 guidelines, consider performing surgical revascularization with direct or indirect extracranial-intracranial bypass for the prevention of ischemic stroke or TIA in patients with Moyamoya disease and a history of ischemic stroke or TIA. C 4. Specific circumstances Patients with Moyamoya syndrome: consider performing revascularization surgery, in addition to continuing regular blood transfusions, in pediatric and adult patients with sickle cell disease, Moyamoya syndrome, and a history of stroke or TIA. C 5. Follow-up and surveillance Follow-up imaging: as per ESO 2023 guidelines, obtain neuroimaging follow-up for postoperative evaluations of surgical efficacy and for long-term follow-up to evaluate the progression of angiopathy in patients with Moyamoya disease. E Show 5 more Clinical findings Patient demographics Symptoms Japanese Headache Personality or behavioral changes Past medical history Seizure Developmental delay Down's syndrome Neurological exam Neurofibromatosis type 1 Cognitive impairment Sickle cell disease Speech disturbance Stroke TIA Imaging findings Intracranial hemorrhage References https://web.pathway.md/diseases/recGXkYZld2sicLvi 4/5 6/30/23, 1:05 AM Moyamoya disease Pathway 1. Anna Bersano, Nadia Khan, Blanca Fuentes et al. European Stroke Organisation ESO Guidelines on Moyamoya angiopathy Endorsed by Vascular European Reference Network VASCERN . Eur Stroke J. 2023 Mar;8 1 55 84. Open 2. Miki Fujimura, Teiji Tominaga, Satoshi Kuroda et al. 2021 Japanese Guidelines for the Management of Moyamoya Disease: Guidelines from the Research Committee on Moyamoya Disease and Japan Stroke Society. Neurol Med Chir Tokyo). 2022 Apr 15;62 4 165 170. Open 3. M. R. DeBaun, L. C. Jordan, A. A. King et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28; 4 8 1554 1588. Open 4. Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis, Health Labour Sciences Research Grant for Research on Measures for Infractable Diseases. Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis). Neurol Med Chir Tokyo). 2012;52 5 245 66. Open 5. Edward R Smith, R Michael Scott. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr. 2012 Apr;9 4 353 60. Open 6. Paul Monagle, Anthony K C Chan, Neil A Goldenberg et al. Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 Feb;141 2 Suppl):e737S-e801S. Open 7. Dawn O Kleindorfer, Amytis Towfighi, Seemant Chaturvedi et al. 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association. Stroke. 2021 Jul;52 7):e364-e467. 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