Management of Patient with Moyamoya Disease

  • Elias Nammour
  • Eman NadaEmail author


A 45-year-old male patient with moyamoya disease is scheduled for a superficial temporal to middle cerebral artery bypass. He has a past medical history of hypertension and stroke with residual left arm weakness.


  1. 1.
    Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. N Engl J Med. 2009;360(12):1226–37.PubMedCrossRefGoogle Scholar
  2. 2.
    Parray T, Martin TW, Siddiqui S. Moyamoya disease: a review of the disease and anesthetic management. J Neurosurg Anesthesiol. 2011;23(2):100–9.PubMedCrossRefGoogle Scholar
  3. 3.
    Baaj AA, et al. Surgical management of moyamoya disease: a review. Neurosurg Focus. 2009;26(4):E7.PubMedCrossRefGoogle Scholar
  4. 4.
    Kuriyama S, et al. Prevalence and clinicoepidemiological features of moyamoya disease in Japan: findings from a nationwide epidemiological survey. Stroke. 2008;39(1):42–7.PubMedCrossRefGoogle Scholar
  5. 5.
    Chiu D, et al. Clinical features of moyamoya disease in the United States. Stroke. 1998;29(7):1347–51.PubMedCrossRefGoogle Scholar
  6. 6.
    Choi JU, et al. Natural history of moyamoya disease: comparison of activity of daily living in surgery and non surgery groups. Clin Neurol Neurosurg. 1997;99(Suppl 2):S11–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Yilmaz EY, et al. Moyamoya: Indiana University Medical Center experience. Arch Neurol. 2001;58(8):1274–8.PubMedCrossRefGoogle Scholar
  8. 8.
    Hosain SA, et al. Use of a calcium channel blocker (nicardipine HCl) in the treatment of childhood moyamoya disease. J Child Neurol. 1994;9(4):378–80.PubMedCrossRefGoogle Scholar
  9. 9.
    Hallemeier CL, et al. Clinical features and outcome in north American adults with moyamoya phenomenon. Stroke. 2006;37(6):1490–6.PubMedCrossRefGoogle Scholar
  10. 10.
    Smith ER, Scott RM. Surgical management of moyamoya syndrome. Skull Base. 2005;15(1):15–26.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Kuroda S, Houkin K. Moyamoya disease: current concepts and future perspectives. Lancet Neurol. 2008;7(11):1056–66.PubMedCrossRefGoogle Scholar
  12. 12.
    Guzman R, et al. Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article. J Neurosurg. 2009;111(5):927–35.PubMedCrossRefGoogle Scholar
  13. 13.
    Mesiwala AH, et al. Long-term outcome of superficial temporal artery-middle cerebral artery bypass for patients with moyamoya disease in the US. Neurosurg Focus. 2008;24(2):E15.PubMedCrossRefGoogle Scholar
  14. 14.
    Andrews BT, Chater NL, Weinstein PR. Extracranial-intracranial arterial bypass for middle cerebral artery stenosis and occlusion. Operative results in 65 cases. J Neurosurg. 1985;62(6):831–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Sakurai K, et al. A novel susceptibility locus for moyamoya disease on chromosome 8q23. J Hum Genet. 2004;49(5):278–81.PubMedCrossRefGoogle Scholar
  16. 16.
    Trigeminal neuralgia. Surg Neurol. 1996;45(5):406–8.Google Scholar
  17. 17.
    Fujimura M, 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(4):442–7.PubMedCrossRefGoogle Scholar
  18. 18.
    Manceau E, Giroud M, Dumas R. Moyamoya disease in children. A review of the clinical and radiological features and current treatment. Childs Nerv Syst. 1997;13(11–12):595–600.PubMedCrossRefGoogle Scholar
  19. 19.
    Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol. 1969;20(3):288–99.PubMedCrossRefGoogle Scholar
  20. 20.
    Fukuyama Y, Umezu R. Clinical and cerebral angiographic evolutions of idiopathic progressive occlusive disease of the circle of Willis (“moyamoya” disease) in children. Brain Dev. 1985;7(1):21–37.PubMedCrossRefGoogle Scholar
  21. 21.
    Baykan N, et al. Moyamoya disease and anesthesia. Paediatr Anaesth. 2005;15(12):1111–5.PubMedCrossRefGoogle Scholar
  22. 22.
    Nomura S, et al. Perioperative management protocols for children with moyamoya disease. Childs Nerv Syst. 2001;17(4–5):270–4.PubMedCrossRefGoogle Scholar
  23. 23.
    Soriano SG, Sethna NF, Scott RM. Anesthetic management of children with moyamoya syndrome. Anesth Analg. 1993;77(5):1066–70.PubMedCrossRefGoogle Scholar
  24. 24.
    Ogawa A, et al. Cerebral blood flow in moyamoya disease. Part 2: autoregulation and CO2 response. Acta Neurochir. 1990;105(3–4):107–11.PubMedCrossRefGoogle Scholar
  25. 25.
    Kuwabara Y, et al. Response to hypercapnia in moyamoya disease. Cerebrovascular response to hypercapnia in pediatric and adult patients with moyamoya disease. Stroke. 1997;28(4):701–7.PubMedCrossRefGoogle Scholar
  26. 26.
    Kikuta K, et al. Effects of intravenous anesthesia with propofol on regional cortical blood flow and intracranial pressure in surgery for moyamoya disease. Surg Neurol. 2007;68(4):421–4.PubMedCrossRefGoogle Scholar
  27. 27.
    Astrup J, et al. Minimum cerebral blood flow and metabolism during craniotomy. Effect of thiopental loading. Acta Anaesthesiol Scand. 1984;28(5):478–81.PubMedCrossRefGoogle Scholar
  28. 28.
    Cold GE, et al. CBF and CMRO2 during continuous etomidate infusion supplemented with N2O and fentanyl in patients with supratentorial cerebral tumour. A dose-response study. Acta Anaesthesiol Scand. 1985;29(5):490–4.PubMedCrossRefGoogle Scholar
  29. 29.
    Kurehara K, et al. Cortical blood flow response to hypercapnia during anaesthesia in Moyamoya disease. Can J Anaesth. 1993;40(8):709–13.PubMedCrossRefGoogle Scholar
  30. 30.
    Adachi K, et al. Early postoperative complications in patients with Moyamoya disease—a comparison of inhaled anesthesia with total intravenous anesthesia (TIVA). Masui. 2005;54(6):653–7.PubMedGoogle Scholar
  31. 31.
    Sakamoto T, et al. Risk factors for neurologic deterioration after revascularization surgery in patients with moyamoya disease. Anesth Analg. 1997;85(5):1060–5.PubMedCrossRefGoogle Scholar
  32. 32.
    Michenfelder JD. Anesthesia for cerebral surgery. In: Stanley TH, Petty WC, editors. New anesthetic agents, devices and monitoring. 1st ed; 1983. p. 45–9.CrossRefGoogle Scholar
  33. 33.
    Brown SC, Lam AM. Moyamoya disease—a review of clinical experience and anaesthetic management. Can J Anaesth. 1987;34(1):71–5.PubMedCrossRefGoogle Scholar
  34. 34.
    Kansha M, et al. Anesthetic management of children with moyamoya disease. Clin Neurol Neurosurg. 1997;99(Suppl 2):S110–3.PubMedCrossRefGoogle Scholar
  35. 35.
    Sato K, Shirane R, Yoshimoto T. Perioperative factors related to the development of ischemic complications in patients with moyamoya disease. Childs Nerv Syst. 1997;13(2):68–72.PubMedCrossRefGoogle Scholar
  36. 36.
    Iwama T, Hashimoto N, Yonekawa Y. The relevance of hemodynamic factors to perioperative ischemic complications in childhood moyamoya disease. Neurosurgery. 1996;38(6):1120–5. discussion 1125-6PubMedGoogle Scholar
  37. 37.
    Orihashi K, et al. Near-infrared spectroscopy for monitoring cerebral ischemia during selective cerebral perfusion. Eur J Cardiothorac Surg. 2004;26(5):907–11.PubMedCrossRefGoogle Scholar
  38. 38.
    Yusa T, Yamashiro K. Local cortical cerebral blood flow and response to carbon dioxide during anesthesia in patients with moyamoya disease. J Anesth. 1999;13(3):131–5.PubMedCrossRefGoogle Scholar
  39. 39.
    Tagawa T, et al. Regional cerebral blood flow, clinical manifestations, and age in children with moyamoya disease. Stroke. 1987;18(5):906–10.PubMedCrossRefGoogle Scholar
  40. 40.
    Sumikawa K, Nagai H. Moyamoya disease and anesthesia. Anesthesiology. 1983;58(2):204–5.PubMedCrossRefGoogle Scholar
  41. 41.
    Zipfel GJ, Fox DJ Jr, Rivet DJ. Moyamoya disease in adults: the role of cerebral revascularization. Skull Base. 2005;15(1):27–41.PubMedPubMedCentralCrossRefGoogle Scholar
  42. 42.
    Kim SH, et al. Risk factors for postoperative ischemic complications in patients with moyamoya disease. J Neurosurg. 2005;103(5 Suppl):433–8.PubMedGoogle Scholar
  43. 43.
    Lee SH, et al. Optimum degree of hemodilution for brain protection in a canine model of focal cerebral ischemia. J Neurosurg. 1994;80(3):469–75.PubMedCrossRefGoogle Scholar
  44. 44.
    Veeravagu A, et al. Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions. Neurosurg Focus. 2008;24(2):E16.PubMedCrossRefGoogle Scholar
  45. 45.
    Malley RA, Frost EA. Moyamoya disease: pathophysiology and anesthetic management. J Neurosurg Anesthesiol. 1989;1(2):110–4.PubMedCrossRefGoogle Scholar
  46. 46.
    Januszewski J, et al. Flow-based evaluation of cerebral revascularization using near-infrared indocyanine green videoangiography. Neurosurg Focus. 2014;36(2):E14.PubMedCrossRefGoogle Scholar
  47. 47.
    Pandey P, Steinberg GK. Neurosurgical advances in the treatment of moyamoya disease. Stroke. 2011;42(11):3304–10.PubMedCrossRefGoogle Scholar
  48. 48.
    Chu W, et al. Anaphylactic shock after intravenous administration of indocyanine green during robotic partial nephrectomy. Urol Case Rep. 2017;12:37–8.PubMedPubMedCentralCrossRefGoogle Scholar
  49. 49.
    Olsen TW, et al. Anaphylactic shock following indocyanine green angiography. Arch Ophthalmol. 1996;114(1):97.PubMedCrossRefGoogle Scholar
  50. 50.
    Kaku Y, et al. STA-MCA bypass under local anesthesia. Acta Neurochir Suppl. 2018;129:79–84.PubMedCrossRefGoogle Scholar
  51. 51.
    Ngan Kee WD, Gomersall CD. Extradural anaesthesia for caesarean section in a patient with moyamoya disease. Br J Anaesth. 1996;77(4):550–2.PubMedCrossRefGoogle Scholar
  52. 52.
    Sharma SK, et al. Epidural anesthesia for a patient with moyamoya disease presenting for cesarean section. Anesth Analg. 1994;79(1):183–5.PubMedCrossRefGoogle Scholar
  53. 53.
    Venkatesh B, Taggart PC. Anaesthetic management of a patient with Moyamoya disease for caesarean section. Can J Anaesth. 1994;41(1):79–80.PubMedCrossRefGoogle Scholar
  54. 54.
    Abouleish E, Wiggins M, Ali V. Combined spinal and epidural anesthesia for cesarean section in a parturient with moyamoya disease. Acta Anaesthesiol Scand. 1998;42(9):1120–3.PubMedCrossRefGoogle Scholar
  55. 55.
    Ahn HJ, et al. Effect of preoperative skull block on pediatric moyamoya disease. J Neurosurg Pediatr. 2008;2(1):37–41.PubMedCrossRefGoogle Scholar
  56. 56.
    Chui J, et al. Anesthetic management of patients undergoing intracranial bypass procedures. Anesth Analg. 2015;120(1):193–203.PubMedCrossRefGoogle Scholar
  57. 57.
    Yu J, et al. Progress on complications of direct bypass for moyamoya disease. Int J Med Sci. 2016;13(8):578–87.PubMedPubMedCentralCrossRefGoogle Scholar
  58. 58.
    Iwama T, et al. Peri-operative complications in adult moyamoya disease. Acta Neurochir. 1995;132(1–3):26–31.PubMedCrossRefGoogle Scholar
  59. 59.
    Fung LW, Thompson D, Ganesan V. Revascularisation surgery for paediatric moyamoya: a review of the literature. Childs Nerv Syst. 2005;21(5):358–64.PubMedCrossRefGoogle Scholar
  60. 60.
    Scott RM, et al. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg. 2004;100(2 Suppl Pediatrics):142–9.PubMedGoogle Scholar
  61. 61.
    Aoki N. Cerebrovascular bypass surgery for the treatment of Moyamoya disease: unsatisfactory outcome in the patients presenting with intracranial hemorrhage. Surg Neurol. 1993;40(5):372–7.PubMedCrossRefGoogle Scholar
  62. 62.
    Huang Z, et al. Clinical features and outcomes in 154 patients with haemorrhagic moyamoya disease: comparison of conservative treatment and surgical revascularization. Neurol Res. 2015;37(10):886–92.PubMedCrossRefGoogle Scholar
  63. 63.
    Park W, et al. Intracranial hemorrhage after superficial temporal artery-middle cerebral artery direct anastomosis for adults with moyamoya disease. World Neurosurg. 2018;119:e774–82.PubMedCrossRefGoogle Scholar
  64. 64.
    Yoshida Y, et al. Clinical course, surgical management, and long-term outcome of moyamoya patients with rebleeding after an episode of intracerebral hemorrhage: an extensive follow-up study. Stroke. 1999;30(11):2272–6.PubMedCrossRefGoogle Scholar
  65. 65.
    Fujimura M, et al. Significance of focal cerebral hyperperfusion as a cause of transient neurologic deterioration after extracranial-intracranial bypass for moyamoya disease: comparative study with non-moyamoya patients using N-isopropyl-p-[(123)I]iodoamphetamine single-photon emission computed tomography. Neurosurgery. 2011;68(4):957–64; discussion 964-5.PubMedCrossRefGoogle Scholar
  66. 66.
    Fujimura M, Tominaga T. Lessons learned from moyamoya disease: outcome of direct/indirect revascularization surgery for 150 affected hemispheres. Neurol Med Chir (Tokyo). 2012;52(5):327–32.CrossRefGoogle Scholar
  67. 67.
    Zhao WG, et al. Cerebral hyperperfusion syndrome after revascularization surgery in patients with moyamoya disease. Br J Neurosurg. 2013;27(3):321–5.PubMedCrossRefGoogle Scholar
  68. 68.
    Abd-El-Barr MM, et al. Recombinant human bone morphogenetic protein-2 as an adjunct for spine fusion in a pediatric population. Pediatr Neurosurg. 2011;47(4):266–71.PubMedCrossRefGoogle Scholar
  69. 69.
    Zhao M, et al. Risk factors for and outcomes of postoperative complications in adult patients with moyamoya disease. J Neurosurg. 2018;1:1–12.Google Scholar
  70. 70.
    Bacigaluppi S, et al. The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease: a review. Neurosurg Focus. 2009;26(4):E3.PubMedCrossRefGoogle Scholar
  71. 71.
    Fujimura M, 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(5):436–45.PubMedCrossRefGoogle Scholar
  72. 72.
    Inoue T, et al. Annual rupture risk of growing unruptured cerebral aneurysms detected by magnetic resonance angiography. J Neurosurg. 2012;117(1):20–5.PubMedCrossRefGoogle Scholar
  73. 73.
    Sekhar LN, et al. Cerebral revascularization for ischemia, aneurysms, and cranial base tumors. Neurosurgery. 2008;62(6 Suppl 3):1373–408; discussion 1408-10.PubMedGoogle Scholar
  74. 74.
    Jung YJ, et al. Ischemic complications occurring in the contralateral hemisphere after surgical treatment of adults with moyamoya disease. J Korean Neurosurg Soc. 2011;50(6):492–6.PubMedPubMedCentralCrossRefGoogle Scholar
  75. 75.
    Uchino H, et al. Edaravone reduces hyperperfusion-related neurological deficits in adult moyamoya disease: historical control study. Stroke. 2016;47(7):1930–2.PubMedCrossRefGoogle Scholar
  76. 76.
    Fujimura M, et al. Minocycline prevents focal neurological deterioration due to cerebral hyperperfusion after extracranial-intracranial bypass for moyamoya disease. Neurosurgery. 2014;74(2):163–70; discussion 170.PubMedCrossRefGoogle Scholar
  77. 77.
    Matsushima Y, et al. Perioperative complications of encephalo-duro-arterio-synangiosis: prevention and treatment. Surg Neurol. 1991;36(5):343–53.PubMedCrossRefGoogle Scholar

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© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.University of Massachusetts Medical SchoolWorcesterUSA
  2. 2.Department of Anesthesiology and Perioperative MedicineUniversity of Massachusetts Medical SchoolWorcesterUSA

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