Introduction: Moyamoya disease (MMD), a cerebrovascular disease prevalent among pediatric populations that results in stenosis of the internal carotid artery, commonly requires neurosurgical interventions to revascularize. Although previous studies have compared perioperative outcomes between direct (DB) and indirect (IB) revascularization procedures, the comparative long-term outcomes for pediatric patients, namely stroke risk and mRS, remain largely unknown. Ultimately, this review attempts to address the question of whether direct revascularization (DB) for pediatric MDD patients improves long-term (≥1 year) stroke risk and mRS scores compared to indirect procedures (IB).
Methods: Databases including the Cochrane Library, CENTRAL, and PubMed were searched from inception to 1 November 2024, in addition to citation chaining. All pediatric patients with MMD (Ages 0-18) who have undergone direct or indirect surgical revascularization procedures were included. The primary outcomes assessed were the long-term (≥1 year) percentage of patients with an mRS score ≤ 2, and long-term stroke rate, compared across the DB and IB populations through pooled-analysis Kaplan-Meier curves. It is important to note that stroke risk includes TIAs, Hemorrhagic Strokes, and Ischemic Strokes.
Results: After 698 non-duplicated records were screened, a total of 10 studies yielding 1473 patients fulfilled eligibility criteria and were therefore included, in which 237 and 1236 pediatric patients with MMD underwent DB and IB procedures respectively. A meta-analysis of post-operative stroke outcomes demonstrated a 27% relative risk reduction (RR = 0.73; 95% CI [0.02, 0.08]; p=0.025) for those in the DB population compared to the IB population. Furthermore, A meta-analysis of mRS scores ≤ 2 in 3 studies (Total = 321; DB =47; IB = 274) demonstrated a significant 62% relative risk reduction in favor of DB treatment (RR = 0.38; 95% CI [-0.01, 0.01]; p=0.025).
Conclusion : DB is associated with lesser long-term risks of stroke and neurological disability relative to IB, mirroring the short-term consensus. It is important to note that, due to a lack of institutional access, many studies that may be relevant could not be accessed, which may alter the specific results.
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