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Moyamoya disease: Treatment and prognosis

Nijasri Charnnarong Suwanwela, MD
Section Editors
Jose Biller, MD, FACP, FAAN, FAHA
Douglas R Nordli, Jr, MD
Deputy Editor
John F Dashe, MD, PhD


Moyamoya disease is a unique chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation.

This topic will review the prognosis and treatment of moyamoya. Other aspects of the disease are discussed separately. (See "Moyamoya disease: Etiology, clinical features, and diagnosis".)


For children and adults with moyamoya and acute stroke, acute treatment is mainly symptomatic and directed towards reducing elevated intracranial pressure, improving cerebral blood flow, and controlling seizures [1]. Ventricular drainage and/or hematoma removal is often required for patients with intracerebral hemorrhage.

During hospitalization for acute stroke or surgery, the following management issues deserve special attention, especially in children with moyamoya [2]:

Precautions to minimize crying and hyperventilation: both can lower PaCO2 and thereby induce or worsen cerebral ischemia by causing vasoconstriction [2,3].

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Literature review current through: Oct 2017. | This topic last updated: Jan 05, 2016.
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  1. Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis ('moyamoya' disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 1997; 99 Suppl 2:S238.
  2. Roach ES, Golomb MR, Adams R, et al. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 2008; 39:2644.
  3. Parray T, Martin TW, Siddiqui S. Moyamoya disease: a review of the disease and anesthetic management. J Neurosurg Anesthesiol 2011; 23:100.
  4. Nomura S, Kashiwagi S, Uetsuka S, et al. Perioperative management protocols for children with moyamoya disease. Childs Nerv Syst 2001; 17:270.
  5. Scott RM, Smith JL, Robertson RL, et al. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg 2004; 100:142.
  6. Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009; 360:1226.
  7. Fujiwara J, Nakahara S, Enomoto T, et al. The effectiveness of O2 administration for transient ischemic attacks in moyamoya disease in children. Childs Nerv Syst 1996; 12:69.
  8. Pollak L. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009; 361:98; author reply 98.
  9. Monagle P, Chan AK, Goldenberg NA, 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; 141:e737S.
  10. Horn P, Bueltmann E, Buch CV, Schmiedek P. Arterio-embolic ischemic stroke in children with moyamoya disease. Childs Nerv Syst 2005; 21:104.
  11. McLean MJ, Gebarski SS, van der Spek AF, Goldstein GW. Response of moyamoya disease to verapamil. Lancet 1985; 1:163.
  12. Spittler JF, Smektala K. Pharmacotherapy in moyamoya disease. Hokkaido Igaku Zasshi 1990; 65:235.
  13. Hosain SA, Hughes JT, Forem SL, et al. Use of a calcium channel blocker (nicardipine HCl) in the treatment of childhood moyamoya disease. J Child Neurol 1994; 9:378.
  14. Yu JL, Wang HL, Xu K, et al. Endovascular treatment of intracranial aneurysms associated with moyamoya disease or moyamoya syndrome. Interv Neuroradiol 2010; 16:240.
  15. Kim SH, Kwon OK, Jung CK, et al. Endovascular treatment of ruptured aneurysms or pseudoaneurysms on the collateral vessels in patients with moyamoya disease. Neurosurgery 2009; 65:1000.
  16. Ueki K, Meyer FB, Mellinger JF. Moyamoya disease: the disorder and surgical treatment. Mayo Clin Proc 1994; 69:749.
  17. Fung LW, Thompson D, Ganesan V. Revascularisation surgery for paediatric moyamoya: a review of the literature. Childs Nerv Syst 2005; 21:358.
  18. Pandey P, Steinberg GK. Neurosurgical advances in the treatment of moyamoya disease. Stroke 2011; 42:3304.
  19. Matsushima T, Inoue T, Suzuki SO, et al. Surgical treatment of moyamoya disease in pediatric patients--comparison between the results of indirect and direct revascularization procedures. Neurosurgery 1992; 31:401.
  20. Ikezaki K, Matsushima T, Kuwabara Y, et al. Cerebral circulation and oxygen metabolism in childhood moyamoya disease: a perioperative positron emission tomography study. J Neurosurg 1994; 81:843.
  21. Golby AJ, Marks MP, Thompson RC, Steinberg GK. Direct and combined revascularization in pediatric moyamoya disease. Neurosurgery 1999; 45:50.
  22. Kawaguchi T, Fujita S, Hosoda K, et al. Multiple burr-hole operation for adult moyamoya disease. J Neurosurg 1996; 84:468.
  23. Kim SK, Cho BK, Phi JH, et al. Pediatric moyamoya disease: An analysis of 410 consecutive cases. Ann Neurol 2010; 68:92.
  24. Kronenburg A, Braun KP, van der Zwan A, Klijn CJ. Recent advances in moyamoya disease: pathophysiology and treatment. Curr Neurol Neurosci Rep 2014; 14:423.
  25. Cho WS, Kim JE, Kim CH, et al. Long-term outcomes after combined revascularization surgery in adult moyamoya disease. Stroke 2014; 45:3025.
  26. Kazumata K, Ito M, Tokairin K, et al. The frequency of postoperative stroke in moyamoya disease following combined revascularization: a single-university series and systematic review. J Neurosurg 2014; 121:432.
  27. Fukui M. Current state of study on moyamoya disease in Japan. Surg Neurol 1997; 47:138.
  28. Ikezaki K. Rational approach to treatment of moyamoya disease in childhood. J Child Neurol 2000; 15:350.
  29. Aoki N. Cerebrovascular bypass surgery for the treatment of Moyamoya disease: unsatisfactory outcome in the patients presenting with intracranial hemorrhage. Surg Neurol 1993; 40:372.
  30. Han DH, Kwon OK, Byun BJ, et al. A co-operative study: clinical characteristics of 334 Korean patients with moyamoya disease treated at neurosurgical institutes (1976-1994). The Korean Society for Cerebrovascular Disease. Acta Neurochir (Wien) 2000; 142:1263.
  31. Derdeyn CP. Direct bypass reduces the risk of recurrent hemorrhage in moyamoya syndrome, but effect on functional outcome is less certain. Stroke 2014; 45:1245.
  32. Liu XJ, Zhang D, Wang S, et al. Clinical features and long-term outcomes of moyamoya disease: a single-center experience with 528 cases in China. J Neurosurg 2015; 122:392.
  33. Miyamoto S, Yoshimoto T, Hashimoto N, et al. Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: results of the Japan Adult Moyamoya Trial. Stroke 2014; 45:1415.
  34. Kim SK, Seol HJ, Cho BK, et al. Moyamoya disease among young patients: its aggressive clinical course and the role of active surgical treatment. Neurosurgery 2004; 54:840.
  35. Fujimura M, Mugikura S, Kaneta T, et al. Incidence and risk factors for symptomatic cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with moyamoya disease. Surg Neurol 2009; 71:442.
  36. Hayashi K, Horie N, Suyama K, Nagata I. Incidence and clinical features of symptomatic cerebral hyperperfusion syndrome after vascular reconstruction. World Neurosurg 2012; 78:447.
  37. Uchino H, Kuroda S, Hirata K, et al. Predictors and clinical features of postoperative hyperperfusion after surgical revascularization for moyamoya disease: a serial single photon emission CT/positron emission tomography study. Stroke 2012; 43:2610.
  38. Hwang JW, Yang HM, Lee H, et al. Predictive factors of symptomatic cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in adult patients with moyamoya disease. Br J Anaesth 2013; 110:773.
  39. Fujimura M, Inoue T, Shimizu H, et al. Efficacy of prophylactic blood pressure lowering according to a standardized postoperative management protocol to prevent symptomatic cerebral hyperperfusion after direct revascularization surgery for moyamoya disease. Cerebrovasc Dis 2012; 33:436.
  40. Kikuta K. Experiences using 3-tesla magnetic resonance imaging in the treatment of Moyamoya disease. Acta Neurochir Suppl 2008; 103:123.
  41. Kikuta K, Takagi Y, Fushimi Y, et al. "Target bypass": a method for preoperative targeting of a recipient artery in superficial temporal artery-to-middle cerebral artery anastomoses. Neurosurgery 2008; 62:1434.
  42. 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; 52:245.
  43. Smith ER, Scott RM. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr 2012; 9:353.
  44. Kuroda S, Ishikawa T, Houkin K, et al. Incidence and clinical features of disease progression in adult moyamoya disease. Stroke 2005; 36:2148.
  45. Hallemeier CL, Rich KM, Grubb RL Jr, et al. Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 2006; 37:1490.
  46. Morioka M, Hamada J, Todaka T, et al. High-risk age for rebleeding in patients with hemorrhagic moyamoya disease: long-term follow-up study. Neurosurgery 2003; 52:1049.
  47. Choi JU, Kim DS, Kim EY, Lee KC. Natural history of moyamoya disease: comparison of activity of daily living in surgery and non surgery groups. Clin Neurol Neurosurg 1997; 99 Suppl 2:S11.
  48. Kurokawa T, Tomita S, Ueda K, et al. Prognosis of occlusive disease of the circle of Willis (moyamoya disease) in children. Pediatr Neurol 1985; 1:274.
  49. Ezura M, Yoshimoto T, Fujiwara S, et al. Clinical and angiographic follow-up of childhood-onset moyamoya disease. Childs Nerv Syst 1995; 11:591.
  50. Kuroda S, Hashimoto N, Yoshimoto T, et al. Radiological findings, clinical course, and outcome in asymptomatic moyamoya disease: results of multicenter survey in Japan. Stroke 2007; 38:1430.
  51. Yilmaz EY, Pritz MB, Bruno A, et al. Moyamoya: Indiana University Medical Center experience. Arch Neurol 2001; 58:1274.
  52. Chiu D, Shedden P, Bratina P, Grotta JC. Clinical features of moyamoya disease in the United States. Stroke 1998; 29:1347.
  53. Numaguchi Y, Gonzalez CF, Davis PC, et al. Moyamoya disease in the United States. Clin Neurol Neurosurg 1997; 99 Suppl 2:S26.