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Free Content Imaging of the Diagnosis, Work-Up, and Treatment of Moyamoya Vasculopathy

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Moyamoya disease is a complex phenomenon with many potential etiologies and can present with a variety of clinical manifestations. Imaging plays a crucial role in the diagnosis of Moyamoya disease, and the radiologist may be the first to suggest the disease. Either invasive or noninvasive angiography can be used to establish the diagnosis, based on defined radiologic criteria. Once the diagnosis is established, cerebral perfusion imaging before and after a vasodilatory challenge can be used to assess cerebrovascular reserve. The definitive treatment for Moyamoya disease is surgical revascularization with a goal to increase cerebral blood flow to tissues at risk. There are a variety of surgical options for revascularization in broad categories of either direct or indirect bypasses. Noninvasive imaging plays a key role in the evaluation of patients following surgical revascularization to assess the effectiveness of surgery as well as for complications. Noncontrast CT or MR imaging can be used to evaluate for early postoperative complications. CTA can be used to assess the patency of bypass grafts. Perfusion imaging can be performed to assess the degree of improvement in cerebral perfusion.

Learning Objectives: Describe the imaging appearance of Moyamoya disease, identify typical postoperative findings after surgical revascularization, and recognize the complications of Moyamoya disease and its treatments.
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Keywords: ACA = anterior cerebral artery; ASL = arterial spin-labeling; CHS = cerebral hyperperfusion syndrome; ECA = external carotid artery; MMD = Moyamoya disease; MMS = Moyamoya syndrome; NF1 = neurofibromatosis type 1; PCA = posterior cerebral artery; STA = superficial temporal artery

Document Type: Research Article

Publication date: June 1, 2020

More about this publication?
  • Neurographics is the peer-reviewed, bimonthly educational journal of the American Society of Neuroradiology. The journal comprises articles selected from material presented at the ASNR Annual Meeting. Neurographics also publishes other high-quality submissions that are primarily educational and have a high emphasis on a pictorial approach. Neurographics offers CME credit for reading review articles and completing quiz-based self-assessment activities.

    Visit the ASNR Education Connection to view all available CME courses.

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