The Management of Symptomatic Moyamoya Disease in Pediatric Patients: A Systematic Review and Meta-Analysis

BACKGROUND AND OBJECTIVES: The optimal management strategy for pediatric patients with symptomatic moyamoya disease (MMD) is not well established. This systematic review and meta-analysis compares surgical vs conservative management and direct/combined bypass (DB/CB) vs indirect bypass (IB) for pediatric patients with symptomatic MMD. METHODS: MEDLINE and PubMed were searched from inception to March 17, 2024. For analysis of surgical vs conservative treatment, the primary and secondary outcomes were follow-up ischemic stroke and intracranial hemorrhagic events, respectively. For analysis of DB/CB vs IB, the primary outcome was follow-up ischemic stroke, and secondary outcomes included follow-up transient ischemic attack, new or worsened seizures, symptomatic improvement, modified Rankin Scale score ≤2, and Matsushima grade A at the last follow-up. RESULTS: Twenty-two included studies yielded 1091 patients, with a median follow-up duration of 35.7 months. Regarding surgical vs conservative management, 428 patients were analyzed. Surgical treatment was associated with lower odds of ischemic stroke (odds ratios [OR] = 0.33 [95% CI, 0.11-0.97], P = .04), and intracranial hemorrhagic events tended to be lower with surgery (OR = 0.25 [0.06-1.03], P = .05). Regarding DB/CB techniques vs IB, 875 patients were analyzed. The groups had similar rates of ischemic stroke (OR = 0.79 [0.31-1.97], P = .61), transient ischemic attack (OR = 1.27[0.46-3.55], P = .64), new or worsened seizures (OR = 1.05[0.3-3.65], P = .93), symptomatic improvement (OR = 2.45[0.71-8.45], P = .16), and follow-up modified Rankin Scale ≤2 (OR = 1.21 [0.16-8.85], P = .85). CB was associated with higher Matsushima grade A relative to IB (OR = 3.44 [1.32-9.97], P = .01). CONCLUSION: Surgical revascularization yielded more favorable clinical outcomes than conservative management in this meta-analysis. Clinical outcomes were similar between DB/CB vs IB techniques. Surgical flow augmentation, either by DB/CB or IB, seems to benefit pediatric patients with symptomatic MMD.