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Moyamoya disease

Key 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 Moyamoya Disease Working Group (MD-WG 2012), the Ministry of Health, Labour and Welfare of Japan (MHLW/PTSOW-RC 2012), and the American College of Chest Physicians (ACCP 2012).
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Guidelines

1.Diagnostic investigations

Diagnostic imaging: as per ESO 2023 guidelines, consider obtaining hemodynamic assessment (CT, MRI, SPECT, PET, 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.
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2.Medical management

Intravenous thrombolysis: consider administering intravenous thrombolysis with rtPA under careful evaluation of the risk of hemorrhagic complication in the hyperacute phase of cerebral ischemia in patients with Moyamoya disease.
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  • BP control

  • Nonoperative management

3.Surgical interventions

Surgical revascularization, indications, VASCERN/ESO: 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.
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  • Surgical revascularization (timing)

  • Surgical revascularization (settings)

  • Surgical revascularization (technical considerations)

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.
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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.
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