Management of Moyamoya disease: A systematic review and meta-analysis on surgical revascularization, outcomes and clinical manifestations

Abstract

Background: Moyamoya disease (MDD), defined by a chronic, progressive stenosis of the terminal portion of the internal carotid arteries (ICA) on both sides, carries the anomalous vascular information network, which functions as a collateral pathway to the brain. The aim is to understand the management of moyamoya disease (MMD) in terms of the approaches and different types of arterial revascularisations (direct, indirect, and combined), regardless of the pathological mechanism of origin to be investigated.

Materials and methods: A review was conducted following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The review focused on moyamoya disease, outcomes before and after revascularisation, and related treatment modalities (STA-MCA) in various databases, including ScienceDirect and PubMed/MEDLINE, using the PRISMA guidelines, R, and Excel. Only studies published in English up to August 2025 were included.

Results: Our systematic review included 4800 patients, comprising (N=2866, 60%), patients, (N=2024,42%), surgical interventions, and (N=2112,44%,), revascularisations. See Table 1. (N=1199, 25%), patients included (N=977, 20%), surgical approaches (N=615, 13%), and revascularisations.

Conclusion: This study has shown that moyamoya disease (MMD) is a vascular concern that, regardless of ethnicity, is very rare; cases have been reported in Europe and the United States, as well as in the Hispanic population, but none have been reported in African regions. Therefore, encountering this pathology cannot be ruled out, and one should be up-to-date on the types of revascularisations, whether STA-MCA, ACA-PCA.