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Moyamoya
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Moyamoya disease (MMD)
          • Synonyms

            • Idiopathic progressive arteriopathy of childhood, spontaneous occlusion of circle of Willis (COW)
          • Definitions

            • Progressive bilateral stenosis or occlusion of terminal internal carotid artery (ICA)/proximal COW with abnormal vascular network at base of brain
              • Compensatory development of collateral vascular network = "puff of smoke" (hazy network of basal collaterals)
            • Primary (idiopathic) moyamoya = MMD
              • More common in Japan, Korea
            • Secondary (acquired) moyamoya
              • Cranial radiation
              • Atherosclerosis
              • Neurofibromatosis (NF)

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Angiographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              • Reporting Tips

                                                Selected References

                                                1. Han Q et al: Quantitative analysis of revascularization in ischemic moyamoya disease via whole-brain computed tomography perfusion: a retrospective single-center study. Medicine (Baltimore). 99(7):e19168, 2020
                                                2. Ravindra VM et al: Preoperative computed tomography perfusion in pediatric moyamoya disease: a single-institution experience. J Neurosurg Pediatr. ePub, 2020
                                                3. Terrell D et al: Cerebral revascularization for moyamoya syndrome associated with sickle cell disease: a systematic review of the literature on the role of extracranial-intracranial bypass in treating neurologic manifestations of pediatric patients with sickle cell disease. World Neurosurg. 137:62-70, 2020
                                                4. Bang OY et al: Moyamoya disease and spectrums of RNF213 vasculopathy. Transl Stroke Res. 11(4):580-9, 2019
                                                5. Boulouis G et al: Nontraumatic pediatric intracerebral hemorrhage. Stroke. 50(12):3654-61, 2019
                                                6. Goyal P et al: Neuroimaging of pediatric arteriopathies. J Neuroimaging. 29(3):287-308, 2019
                                                7. Li J et al: Imaging of moyamoya disease and moyamoya syndrome: current status. J Comput Assist Tomogr. 43(2):257-63, 2019
                                                8. Liu ZW et al: Collateral circulation in moyamoya disease: a new grading system. Stroke. 50(10):2708-15, 2019
                                                9. Ravindran K et al: Surgical outcomes for pediatric moyamoya: a systematic review and meta-analysis. J Neurosurg Pediatr. 1-10, 2019
                                                10. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                                11. Acker G et al: Surgical management of moyamoya disease. Stroke. 49(2):476-82, 2018
                                                12. Cho KC et al: Rete middle cerebral artery anomalies: a unifying name, case series, and literature review. J Neurosurg. 131(2):453-61, 2018
                                                13. Huang S et al: Etiology and pathogenesis of moyamoya disease: an update on disease prevalence. Int J Stroke. 12(3):246-53, 2017
                                                14. Fujimura M et al: Moyamoya disease. Front Neurol Neurosci. 40:204-20, 2016
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Pathology

                                                      • Clinical Issues

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Moyamoya disease (MMD)
                                                        • Synonyms

                                                          • Idiopathic progressive arteriopathy of childhood, spontaneous occlusion of circle of Willis (COW)
                                                        • Definitions

                                                          • Progressive bilateral stenosis or occlusion of terminal internal carotid artery (ICA)/proximal COW with abnormal vascular network at base of brain
                                                            • Compensatory development of collateral vascular network = "puff of smoke" (hazy network of basal collaterals)
                                                          • Primary (idiopathic) moyamoya = MMD
                                                            • More common in Japan, Korea
                                                          • Secondary (acquired) moyamoya
                                                            • Cranial radiation
                                                            • Atherosclerosis
                                                            • Neurofibromatosis (NF)

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Angiographic Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          • Image Interpretation Pearls

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Han Q et al: Quantitative analysis of revascularization in ischemic moyamoya disease via whole-brain computed tomography perfusion: a retrospective single-center study. Medicine (Baltimore). 99(7):e19168, 2020
                                                                                              2. Ravindra VM et al: Preoperative computed tomography perfusion in pediatric moyamoya disease: a single-institution experience. J Neurosurg Pediatr. ePub, 2020
                                                                                              3. Terrell D et al: Cerebral revascularization for moyamoya syndrome associated with sickle cell disease: a systematic review of the literature on the role of extracranial-intracranial bypass in treating neurologic manifestations of pediatric patients with sickle cell disease. World Neurosurg. 137:62-70, 2020
                                                                                              4. Bang OY et al: Moyamoya disease and spectrums of RNF213 vasculopathy. Transl Stroke Res. 11(4):580-9, 2019
                                                                                              5. Boulouis G et al: Nontraumatic pediatric intracerebral hemorrhage. Stroke. 50(12):3654-61, 2019
                                                                                              6. Goyal P et al: Neuroimaging of pediatric arteriopathies. J Neuroimaging. 29(3):287-308, 2019
                                                                                              7. Li J et al: Imaging of moyamoya disease and moyamoya syndrome: current status. J Comput Assist Tomogr. 43(2):257-63, 2019
                                                                                              8. Liu ZW et al: Collateral circulation in moyamoya disease: a new grading system. Stroke. 50(10):2708-15, 2019
                                                                                              9. Ravindran K et al: Surgical outcomes for pediatric moyamoya: a systematic review and meta-analysis. J Neurosurg Pediatr. 1-10, 2019
                                                                                              10. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                                                                              11. Acker G et al: Surgical management of moyamoya disease. Stroke. 49(2):476-82, 2018
                                                                                              12. Cho KC et al: Rete middle cerebral artery anomalies: a unifying name, case series, and literature review. J Neurosurg. 131(2):453-61, 2018
                                                                                              13. Huang S et al: Etiology and pathogenesis of moyamoya disease: an update on disease prevalence. Int J Stroke. 12(3):246-53, 2017
                                                                                              14. Fujimura M et al: Moyamoya disease. Front Neurol Neurosci. 40:204-20, 2016