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.