Effects of Extracranial–Intracranial Bypass for Patients With Hemorrhagic Moyamoya Disease

Abstract

Background and Purpose-About one half of those who develop adult-onset moyamoya disease experience intracranial hemorrhage. Despite the extremely high frequency of rebleeding attacks and poor prognosis, measures to prevent rebleeding have not been established. The purpose of this study is to determine whether extracranial-intracranial bypass can reduce incidence of rebleeding and improve patient prognosis. Methods-This study was a multicentered, prospective, randomized, controlled trial conducted by 22 institutes in Japan.Adult patients with moyamoya disease who had experienced intracranial hemorrhage within the preceding year were given either conservative care or bilateral extracranial-intracranial direct bypass and were observed for 5 years. Primary and secondary end points were defined as all adverse events and rebleeding attacks, respectively. Results-Eighty patients were enrolled (surgical, 42; nonsurgical, 38). Adverse events causing significant morbidity were observed in 6 patients in the surgical group (14.3%) and 13 patients in the nonsurgical group (34.2%). Kaplan-Meier survival analysis revealed significant differences between the 2 groups (3.2%/y versus 8.2%/y; P=0.048). The hazard ratio of the surgical group calculated by Cox regression analysis was 0.391 (95% confidence interval, 0.148-1.029).Rebleeding attacks were observed in 5 patients in the surgical group (11.9%) and 12 in the nonsurgical group (31.6%), significantly different in the Kaplan-Meier survival analysis (2.7%/y versus 7.6%/y; P=0.042). The hazard ratio of the surgical group was 0.355 (95% confidence interval, 0.125-1.009). Conclusions-Although statistically marginal, Kaplan-Meier analysis revealed the significant difference between surgical and nonsurgical group, suggesting the preventive effect of direct bypass against rebleeding. Clinical Trial Registration

Current Status of Revascularization Surgery for Moyamoya Disease: Special Consideration for Its ‘Internal Carotid-External Carotid (IC-EC) Conversion’ as the Physiological Reorganization System

“…In fact, the Japanese Stroke Guideline recommends direct revascularization surgery for the patients with moyamoya disease manifesting as cerebral ischemic symptoms orrhagic-onset patients, revascularization could be considered but adequate scientific evidence had been lacking (Recommendation grade C1). Nevertheless, recent evidence by JAM trial strongly encourages direct revascularization surgery for reducing the risk for re-bleeding in adult moyamoya disease patients presenting with intracranial hemorrhage (Miyamoto et al 2014), although the statistical significance was marginal. Sub-group analysis of the JAM trial is currently undertaken to further clarify the patient population among hemorrhagic-onset moyamoya disease in which revascularization surgery exerts particular benefit by preventing re-bleeding.…”

“…Recently, the extracranial-intracranial bypass was also shown to reduce the risk of rebleeding in hemorrhagic-onset patients with moyamoya disease by the Japan Adult Moyamoya (JAM) trial: a multicenter randomized control trial to compare the incidence of re-bleeding rate between surgical and non-surgical groups of hemorrhagic-onset moyamoya disease (Miyamoto et al 2014). Long-term outcome of the extracranial-intracranial bypass for moyamoya disease is favorable, but cerebral ischemia and hyperperfusion syndrome are potential complications of this procedure, which could lead to neurological deterioration in the acute stage (Fujimura et al 2007;Kim et al 2008;Ohue et al 2008).…”

A Case of Neurofibromatosis Type 1 Complicated with Repeated Intracerebral Hemorrhage due to Quasi-Moyamoya Disease

“…According to the Japan adult moyamoya trial, extracranial-intracranial bypass surgery is effective to reduce incidence of rebleeding and improved patient prognosis [21].…”

Surgical treatment of adult moyamoya disease with combined sta-mca bypass and edas: demonstration of technique in video presentation

“…The literature suggests that in addition to antiplatelet medications, both direct and indirect surgical revascularization techniques are effective in reducing the risk of ischemia and hemorrhage (5,7,18,24). In this article, we review the natural history and surgical treatment of MD along with a case presentation in which surgical technique is demonstrated in video.…”