To systematically characterize the clinical features, correlations, and therapeutic implications of participants with moyamoya disease (MMD)-related retinal artery occlusion (RAO) through a case series and pooled descriptive analysis.

A total of 9 participants with MMD-RAO (2020–2025) and 9 cases retrieved from published literature (1981–2025) were retrospectively reviewed to descriptively explore the association between MMD and RAO. Mean difference (MD) was adopted as the effect size for continuous variables, while odds ratio (OR) was used for categorical variables. Heterogeneity was evaluated using the Q statistic and I² index. Publication bias was assessed via funnel plots, and sensitivity analysis was performed using the leave-one-out method.

Eighteen participants were enrolled in the final analysis, with a male predominance (male:female=1.25:1). Limited improvement in best-corrected visual acuity (BCVA) was observed after treatment (logMAR range: 0.22–3.0). Central retinal artery occlusion (CRAO) was the predominant subtype (83.3%), followed by branch retinal artery occlusion (BRAO, 11.1%) and cilioretinal artery occlusion (CLRAO, 5.6%). Participants in our institutional cohort were significantly older than those reported in literature (52.89±9.53 vs 25.56±12.58y, P<0.001). Our cohort presented slightly worse initial BCVA (2.04±0.9 vs 1.90±0.68; MD=0.14, 95%CI: -0.60–0.88, P=0.711, I²=78.3%) and inferior visual recovery (1.99±0.9 vs 1.22±1.14; MD=0.77, 95%CI: -0.19–1.73, P=0.132, I²=82.5%), whereas complete visual recovery (logMAR=0.1) was documented in one BRAO case from published reports. The efficacy of intra-arterial thrombolysis (IAT) reached 60% (3/5) in our cohort, and the overall rate of visual improvement was 41.2%. Mild publication bias was detected; however, sensitivity analysis verified the robustness of primary results.

Participants with MMD-related RAO exhibit unique clinical characteristics differing from those with isolated RAO or uncomplicated MMD. This study identifies an “older MMD phenotype” complicated by RAO, and highlights the necessity of comprehensive vascular screening in elderly participants presenting with RAO to facilitate timely combined ophthalmological and neurological intervention.