Abstract

Background and purpose: Surgery is recommended as the treatment of choice for hemorrhagic Moyamoya disease (MMD). The rationale of surgery and the choice of procedure are poorly understood. The aim of this paper is to present latest evidence, from cellular, biomechanical and population data, surgical treatment options and their effect on the outcome of hemorrhagic MMD. Methods: We systematically reviewed the latest evidence from cellular, biomechanical and populational studies including our own meta-analysis for rationalization of management of MMD. We searched major databases from inception to latest articles available till October 2018. All major breakthroughs including basic research to randomized controlled trials (RCTs) and human case–control studies related to hemorrhagic MMD were included. Our meta-analysis was performed in accordance to the standard Cochrane. Result: Evidence at cellular, biomechanical and RCT levels was presented. For our meta-analysis, we included eight studies, totaling at 632 patients. Our results rationalized the use of surgical methods in support of surgical management of MMD. We showed that surgery in MMD resulted in a significant lower risk of future stroke ([Formula: see text], 95% [Formula: see text]–0.38). Among different surgical methods, the indirect bypass group had a lower risk for sedentary stroke risk reduction compared with the direct bypass group (RR[Formula: see text]=[Formula: see text]3.36, 95% CI[Formula: see text]=[Formula: see text]1.53–7.36). No significant differences were observed in perioperative complications between the two methods. Conclusion: Surgery remains a mainstay for the management of MMD. We concluded that current evidence in biomechanical and our own meta-analysis is in support of surgery being an effective management of hemorrhagic MMD. We deduced insights into research for early detection, characterization and follow up of patients with MMD.

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