Moyamoya Disease in Adult: Post-Bypass Symptomatic Hyperperfusion


Physicians have tried various nonsurgical treatments for moyamoya disease (MMD), but none has so far proven effective [1]. Surgical revascularization, by contrast, increases collateral irrigation and improves cerebral hemodynamics in MMD, thereby reducing the risk of subsequent ischemic insult by improving cerebral hemodynamics in MMD [2]. The various revas-cularization techniques used with MMD can be roughly classified into three categories according to the use of arterial anastomosis: (1) indirect nonanastomotic revascularization surgery; (2) direct anastomotic bypass surgery, usually superficial temporal artery (STA)-middle cerebral artery (MCA) bypass; and (3) combined surgery. Many kinds of indirect revascularization are effectively used for pediatric MMD [3], but surgical options for adult MMD are quite different. The two situations differ for the following reasons: (1) adults have less fragile cortical branches with larger diameter than children, so direct bypass is technically less challenging [4, 5] ; and (2) direct bypass theoretically provides more immediate resolution of ischemic conditions by improving cerebral hemodynamics shortly after surgery [6]. Postoperative changes in cerebral hemodynamics are both marked and abrupt, however, and often induce symptomatic hyperperfusion, particularly in MMD patients with preoperative chronic sustained profound ischemia [7, 8]. Consequently, patients should be carefully managed after direct bypass surgery. In this chapter, the authors review the pertinent literature and relate their personal experience, paying special attention to hyperperfusion after direct bypass in the treatment of adult MMD.


Cerebral Blood Flow Moyamoya Disease Superficial Temporal Artery Cerebral Hemodynamic Hyperperfusion Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Kuroda S, Houkin K (2008) Moyamoya disease: current concepts and future perspectives. Lancet Neurol 7:1056–1066PubMedCrossRefGoogle Scholar
  2. 2.
    Mesiwala AH, Sviri G, Fatemi N et al (2008) Long-term outcome of superficial temporal artery-middle cerebral artery bypass for patients with moyamoya disease in the US. Neurosurg Focus 24:E15PubMedCrossRefGoogle Scholar
  3. 3.
    Robertson RL, Burrows PE, Barnes PD et al (1997) Angiographic changes after pial synangiosis in childhood moyamoya disease. Am J Neuroradiol 18:837–845PubMedGoogle Scholar
  4. 4.
    Veeravagu A, Guzman R, Patil CG et al (2008) Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions. Neurosurg Focus 24:E16PubMedCrossRefGoogle Scholar
  5. 5.
    Zipfel GJ, Fox DJ Jr, Rivet DJ (2005) Moyamoya disease in adults: the role of cerebral revascularization. Skull Base 15:27–41PubMedCrossRefGoogle Scholar
  6. 6.
    Ishikawa T, Kamiyama H, Kuroda S et al (2006) Simultaneous superficial temporal artery to middle cerebral or anterior cerebral artery bypass with pan-synangiosis for moyamoya disease covering both anterior and middle cerebral artery territories. Neurol Med Chir (Tokyo) 46:462–468CrossRefGoogle Scholar
  7. 7.
    Kim JE, Oh CW, Kwon OK et al (2008) Transient hyperperfusion after superficial temporal artery/ middle cerebral artery bypass surgery as a possible cause of postoperative transient neurological deterioration. Cerebrovasc Dis 25:580–586PubMedCrossRefGoogle Scholar
  8. 8.
    Fujimura M, Kaneta T, Mugikura S et al (2007) Temporary neurologic deterioration due to cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with adult-onset moyamoya disease. Surg Neurol 67:273–282PubMedCrossRefGoogle Scholar
  9. 9.
    Kawaguchi S, Okuno S, Sakaki T (2000) Effect of direct arterial bypass on the prevention of future stroke in patients with the hemorrhagic variety of moyamoya disease. J Neurosurg 93:397–401PubMedCrossRefGoogle Scholar
  10. 10.
    Houkin K, Kuroda S, Ishikawa T et al (2000) Neovascularization (angiogenesis) after revascularization in moyamoya disease. Which technique is most useful for moyamoya disease? Acta Neurochir (Wien) 142:269–276CrossRefGoogle Scholar
  11. 11.
    Mizoi K, Kayama T, Yoshimoto T et al (1996) Indirect revascularization for moyamoya disease: is there a beneficial effect for adult patients? Surg Neurol 45:541–548; discussion 548–549PubMedCrossRefGoogle Scholar
  12. 12.
    Houkin K, Ishikawa T, Yoshimoto T et al (1997) Direct and indirect revascularization for moyamoya disease surgical techniques and peri-operative complications. Clin Neurol Neurosurg 99 (Suppl 2):S142–145PubMedCrossRefGoogle Scholar
  13. 13.
    Matsushima T, Inoue T, Suzuki SO et al (1992) Surgical treatment of moyamoya disease in pediatric patients – comparison between the results of indirect and direct revascularization procedures. Neurosurgery 31:401–405PubMedCrossRefGoogle Scholar
  14. 14.
    Hosoda K, Kawaguchi T, Shibata Y et al (2001) Cerebral vasoreactivity and internal carotid artery flow help to identify patients at risk for hyperperfusion after carotid endarterectomy. Stroke 32:1567–1573PubMedCrossRefGoogle Scholar
  15. 15.
    Piepgras DG, Morgan MK, Sundt TM Jr et al (1988) Intracerebral hemorrhage after carotid endarterectomy. J Neurosurg 68:532–536PubMedCrossRefGoogle Scholar
  16. 16.
    Ogasawara K, Yukawa H, Kobayashi M et al (2003) Prediction and monitoring of cerebral hyperper-fusion after carotid endarterectomy by using single-photon emission computerized tomography scanning. J Neurosurg 99:504–510PubMedCrossRefGoogle Scholar
  17. 17.
    Ohue S, Kumon Y, Kohno K et al (2008) Postoperative temporary neurological deficits in adults with moyamoya disease. Surg Neurol 69:281–286; discussion 286–287PubMedCrossRefGoogle Scholar
  18. 18.
    Fujimura M, Mugikura S, Kaneta T et al (2009) Incidence and risk factors for symptomatic cerebral hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in patients with moyamoya disease. Surg Neurol 71:442–447PubMedCrossRefGoogle Scholar
  19. 19.
    Heros RC, Scott RM, Kistler JP et al (1984) Temporary neurological deterioration after extracranial-intracranial bypass. Neurosurgery 15:178–185PubMedCrossRefGoogle Scholar
  20. 20.
    Furuya K, Kawahara N, Morita A et al (2004) Focal hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease. Case report. J Neurosurg 100:128–132CrossRefGoogle Scholar
  21. 21.
    van Mook WN, Rennenberg RJ, Schurink GW et al (2005) Cerebral hyperperfusion syndrome. Lancet Neurol 4:877–888PubMedCrossRefGoogle Scholar
  22. 22.
    Fujimura M, Kaneta T, Tominaga T (2008) Efficacy of superficial temporal artery-middle cerebral artery anastomosis with routine postoperative cerebral blood flow measurement during the acute stage in childhood moyamoya disease. Childs Nerv Syst 24:827–832PubMedCrossRefGoogle Scholar
  23. 23.
    Higashi S, Matsuda H, Fujii H et al (1989) Luxury perfusion syndrome confirmed by sequential studies of regional cerebral blood flow and volume after extracranial to intracranial bypass surgery: case report. Neurosurgery 25:85–89PubMedCrossRefGoogle Scholar
  24. 24.
    Fujimura M, Kaneta T, Shimizu H et al (2007) Symptomatic hyperperfusion after superficial temporal artery-middle cerebral artery anastomosis in a child with moyamoya disease. Childs Nerv Syst 23:1195–1198PubMedCrossRefGoogle Scholar
  25. 25.
    Ogasawara K, Komoribayashi N, Kobayashi M et al (2005) Neural damage caused by cerebral hyperperfusion after arterial bypass surgery in a patient with moyamoya disease: case report. Neurosurgery 56:E1380; discussion E1380PubMedCrossRefGoogle Scholar
  26. 26.
    Houkin K, Yoshimoto T, Abe H et al (1998) Role of basic fibroblast growth factor in the pathogenesis of moyamoya disease. Neurosurg Focus 5:e2PubMedCrossRefGoogle Scholar
  27. 27.
    Nanba R, Kuroda S, Ishikawa T et al (2004) Increased expression of hepatocyte growth factor in cerebrospinal fluid and intracranial artery in moyamoya disease. Stroke 35:2837–2842PubMedCrossRefGoogle Scholar
  28. 28.
    Soriano SG, Cowan DB, Proctor MR et al (2002) Levels of soluble adhesion molecules are elevated in the cerebrospinal fluid of children with moyamoya syndrome. Neurosurgery 50:544–549PubMedGoogle Scholar
  29. 29.
    Fujimura M, Watanabe M, Narisawa A et al (2009) Increased expression of serum matrix metallo-proteinase-9 in patients with moyamoya disease. Surg Neurol [in press, available online]Google Scholar
  30. 30.
    Chan PH (1996) Role of oxidants in ischemic brain damage. Stroke 27:1124–1129PubMedCrossRefGoogle Scholar
  31. 31.
    Fujimura M, Tominaga T, Chan PH (2005) Neuroprotective effect of an antioxidant in ischemic brain injury: involvement of neuronal apoptosis. Neurocrit Care 2:59–66PubMedCrossRefGoogle Scholar
  32. 32.
    Fujimura M, Kaneta T, Shimizu H et al (2009) Cerebral ischemia owing to compression of the brain by swollen temporal muscle used for encephalo-myo-synangiosis in moyamoya disease. Neurosurg Rev 32:245–249PubMedCrossRefGoogle Scholar
  33. 33.
    Ogasawara K, Inoue T, Kobayashi M et al (2004) Pretreatment with the free radical scavenger edaravone prevents cerebral hyperperfusion after carotid endarterectomy. Neurosurgery 55:1060–1067PubMedCrossRefGoogle Scholar

Copyright information

© Springer 2010

Authors and Affiliations

  1. 1.Department of NeurosurgerySeoul National University Hospital, Seoul National University College of MedicineJongno-guRepublic of Korea
  2. 2.Division of Cerebrovascular Surgery, Department of NeurosurgerySeoul National University Bundang Hospital, Seoul National University College of MedicineGyeonggi-doRepublic of Korea

Personalised recommendations