Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by the narrowing of arteries at the brain's base. While cerebral angiography is the gold standard for diagnosis, high-resolution vessel wall magnetic resonance imaging (VW-MRI) has recently emerged as a non-invasive diagnostic tool. This systematic review aims to provide insights into the role of VW-MRI in enhancing the diagnosis and management of MMD. A systematic search was conducted in PubMed, Embase, Scopus, and Web of Science for relevant articles on December 5, 2023. Demographic data, the outer diameter of the vessel, vessel wall contrast enhancement (VW-CE) and its pattern, vessel wall thickening and its pattern, remodeling index, and vessel area were extracted. In the analysis proportions were pooled using a random-effects model with logit transformation where applicable. Quality assessment was conducted using the Newcastle-Ottawa scale. Of the 6035 identified studies in the primary search, 19 met the inclusion criteria. Among the reviewed studies, the most commonly reported variations in patients with MMD using VW-MRI was VW-CE (84.21%) and its pattern (52.63%). The pooled prevalence of enhanced lesions in MMD was 0.55 (95% CI: 0.29-0.79), with ratio of concentric lesions being 0.98 (95% CI: 0.72-1.00, I²: 37%). The remodeling index across three studies showed a pooled estimate of 0.41 (95% CI: 0.11-0.72, I²: 99%). VW-MRI emerges as a promising non-invasive tool for enhancing diagnosis of MMD, potentially aiding in disease differentiation and predicting complications. However, standardization and further research are essential to solidify VW-MRI's role.
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