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Endovascular treatment of symptomatic moyamoya

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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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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).

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