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Endovascular treatment of symptomatic moyamoya

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Abstract

Moyamoya is a rare though important source of neurological morbidity as a result of both ischemic and hemorrhagic sequelae. Although a litany of series detailing the endovascular management of cerebral ischemia is present in the literature, only a paucity of such reports exists for moyamoya. A systematic review of the literature was performed for patients with moyamoya managed with endovascular techniques in addition to the contribution of an additional case managed at our institution. We evaluated treatment approach (angioplasty and/or stent), complications, and both angiographic and clinical outcomes at last follow-up. Results from a total of 28 endovascular procedures were collected (11 stenting, 17 angioplasty alone). Procedural success, defined as a lack of both angiographic and clinical recurrence at follow-up, was achieved after seven procedures (25 %). This rate did not significantly differ between disease type (moyamoya disease vs moyamoya syndrome, p = 1.0) and treatment approach (angioplasty alone vs stenting, p = 1.0). The overall monthly angiographic and clinical recurrence rates were 9.3 and 8.0 %, respectively. Clinically devastating intracerebral hemorrhage was seen after two procedures (7 %), and in an additional three procedures, the treated vessel could not be effectively dilated (11 %). There is no evidence that angioplasty or stenting improves the natural history of moyamoya. Both are associated with significant rates of early angiographic and/or clinical recurrence of symptoms. Taken with the risk of procedural complications, the current limited data should advise against attempted endovascular treatment of moyamoya.

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References

  1. Chimowitz MI, Lynn MJ, Derdeyn CP et al (2011) Stenting versus aggressive medical therapy for intracranial arterial stenosis. N Engl J Med 365:993–1003

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  2. EC/IC Bypass Study Group (1985) Failure of extracranial-intracranial arterial bypass to reduce the risk of ischemic stroke. Results of an international randomized trial. N Engl J Med 313:1191–1200

    Article  Google Scholar 

  3. Eicker S, Etminan N, Turowski B, Steiger HJ, Hanggi D (2011) Intracranial carotid artery stent placement causes delayed severe intracranial hemorrhage in a patient with moyamoya disease. J Neurointervent Surg 3:160–162

    Article  Google Scholar 

  4. El-Hakam LM, Volpi J, Mawad M, Clark G (2010) Angioplasty for acute stroke with pediatric moyamoya syndrome. J Child Neurol 25:1278–1283

    Article  PubMed  Google Scholar 

  5. Fukui M, Kono S, Sueishi K, Ikezaki K (2000) Moyamoya disease. Neuropathology 20(Suppl):S61–64

    Article  PubMed  Google Scholar 

  6. Fung LW, Thompson D, Ganesan V (2005) Revascularization surgery for paediatric moyamoya: a review of the literature. Childs Nerv Syst 21:358–364

    Article  PubMed  Google Scholar 

  7. Gross BA, Du R (2013) The natural history of adult moyamoya in a North American cohort. J Clin Neurosci 20:44–48

    Article  PubMed  Google Scholar 

  8. Gross BA, Du R (2013) Adult moyamoya after revascularization. Acta Neurochir 155:247–254

    Article  PubMed  Google Scholar 

  9. Guzman R, Lee M, Achrol A et al (2009) Clinical outcome after 450 revascularization procedures for moyamoya disease. J Neurosurg 111:927–935

    Article  PubMed  Google Scholar 

  10. Hallemeier CL, Rich KM, Grubb RL, Chicoine MR, Moran CJ, Cross DT et al (2006) Clinical features and outcome in North American adults with moyamoya phenomenon. Stroke 37:1490–1496

    Article  PubMed  Google Scholar 

  11. Karasawa J, Kikuchi H, Furuse S, Kawamura J, Sakaki T (1978) Treatment of moyamoya disease with STA-MCA anastomosis. J Neurosurg 49:679–688

    Article  PubMed  CAS  Google Scholar 

  12. Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H (1992) Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg 77:84–89

    Article  PubMed  CAS  Google Scholar 

  13. Khan N, Dodd R, Marks MP, Bell-Stephens T, Vavao J, Steinberg GK (2011) Failure of primary percutaneous angioplasty and stenting in the prevention of ischemia in Moyamoya angiopathy. Cerebrovasc Dis 31:147–153

    Article  PubMed  Google Scholar 

  14. Kornblihtt LI, Cocorullo S, Miranda C, Lylyk P, Heller PG, Molinas FC (2005) Moyamoya syndrome in an adolescent with essential thrombocythemia: successful intracranial carotid stent placement. Stroke 36:E71–73

    Article  PubMed  Google Scholar 

  15. Lee SJ, Ahn JY (2007) Stenosis of the proximal external carotid artery in an adult with moyamoya disease: moyamoya or atherosclerotic change? Neurol Med Chir (Tokyo) 47:e9

    Google Scholar 

  16. Marks MP, Wojak JC, Al-Ali F, Jayaraman M, Marcellus ML, Connors JJ, Do HM (2006) Angioplasty for symptomatic intracranial stenosis: clinical outcome. Stroke 37:1016–1020

    Article  PubMed  Google Scholar 

  17. Miyamoto S (2004) Japan Adult Moyamoya Trial Group: Study design for a prospective randomized trial of extracranial–intracranial bypass surgery for adults with moyamoya disease and hemorrhagic onset—the Japan Adult Moyamoya Trial Group. Neurol Med Chir (Tokyo) 44:218–219

    Article  Google Scholar 

  18. Natarajan SK, Karmon Y, Tawk RG, Hauck EF, Hopkins LN, Siddiqui AH, Levy EI (2011) Endovascular treatment of patients with intracranial stenosis with moyamoya-type collaterals. J Neurointervent Surg 3:369–374

    Article  Google Scholar 

  19. Powers WJ, Clarke WR, Grubb RL et al (2011) Extracranial-intracranial bypass surgery for stroke prevention in hemodynamic cerebral ischemia. The Carotid Occlusion Surgery Study Randomized Trial. JAMA 306:1983–1992

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  20. Rodriguez GJ, Kirmani JF, Ezzeddine MA, Qureshi AI (2007) Primary percutaneous transluminal angioplasty for early moyamoya disease. J Neuroimaging 17:48–53

    Article  PubMed  Google Scholar 

  21. Santirso D, Oliva P, Gonzalez M, Murias E, Vega P, Gil A, Calleja S (2012) Intracranial stent placement in a patient with moyamoya disease. J Neurol 259:170–171

    Article  PubMed  Google Scholar 

  22. Scott RM, Smith ER (2009) Moyamoya disease and moyamoya syndrome. N Engl J Med 360:1226–1237

    Article  PubMed  CAS  Google Scholar 

  23. Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA (2004) Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg. 100 (2Suppl Pediatrics): 142–149

  24. Suzuki J, Takaku A (1969) Cerebrovascular “moyamoya” disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 20:288–299

    Article  PubMed  CAS  Google Scholar 

  25. Turk AS, Levy EI, Albuquerque FC et al (2008) Influence of patient age and stenosis location on wingspan in-stent restenosis. AJNR Am J Neuroradiol 29:23–27

    Article  PubMed  CAS  Google Scholar 

  26. Wojak JC, Dunlap DC, Hargrave KR, DeAlvareLA CHS, Connors JJ 3rd (2006) Intracranial angioplasty and stenting: long term results from a single center. AJNR AmJ Neuroradiol 27:1882–1892

    CAS  Google Scholar 

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The authors both declare no conflicts of interest/disclosures.

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Correspondence to Bradley A. Gross.

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Gerasimos Baltsavias, Zurich, Switzerland

This report by Gross et al. is a well-presented review of the literature on the methods of treatment, results, and complications of endovascular management of symptomatic moyamoya disease and syndrome. The few reports existing in the literature and reviewed by the authors included mostly adult cases. They demonstrated relatively high recurrence and complication rates, suggesting against endovascular treatment and in favor of surgical revascularization. No significant difference in their response to endovascular treatment was found between disease and syndromic cases. Although the collected reports reflect a relatively early and limited experience, this is in accordance with our own experience and current view.

Lotfi Hacein-Bey, Sacramento, USA

In this issue of Neurosurgical Review, Gross et al. present a comprehensive review of endovascular therapy for moyamoya disease or syndrome. Although the lack of effectiveness of angioplasty or stenting in moyamoya has long been an established concept for specialized teams, eloquently reported in a relatively large case series by Khan et al. (reference 13 in the article), this review is helpful in educating the readership in this time of rapid development of minimally invasive endovascular options for cerebrovascular disease. Since the initial description by Suzuki and Takaku in 1969, our understanding of the molecular foundation and the natural history of moyamoya disease has progressed in a relatively modest way. Certain features appear to be common to all varieties of moyamoya, including the Japanese, North American, and European forms of moyamoya disease and moyamoya syndrome. First, overexpression of basic fibroblast growth factor (bFGF) and transforming growth factor-beta (TGF-β) and a local inflammatory response result in marked intimal thickening from elastin accumulation and significant thinning of the internal elastic lamina which affect large arterial trunks, i.e., the carotid artery and the proximal MCA as well as pial arteries; in addition, there is overexpression of an angiogenic response within the meninges. Second, adult patients present with both ischemic and hemorrhagic events, including a risk of hyperperfusion after treatment. Third, there is often lack of correlation between the severity of angiographic findings and the clinical expression of the disease, in large part because of complex and dynamic rearrangements of global and regional cerebral blood flow. The histopathological changes which affect arteries in moyamoya are for a large part responsible for both a high risk of arterial rupture with angioplasty and a very high incidence of restenosis. Therefore, until the advent of a successful therapy for moyamoya, possibly relying on endothelial progenitor cells or vascular growth factors, the only effective treatment remains surgical, with either direct or indirect revascularization techniques.

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Gross, B.A., Thomas, A.J. & Frerichs, K.U. Endovascular treatment of symptomatic moyamoya. Neurosurg Rev 37, 579–583 (2014). https://doi.org/10.1007/s10143-014-0542-x

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  • DOI: https://doi.org/10.1007/s10143-014-0542-x

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