Surgical Revascularization for Pediatric Patients with Sickle Cell Disease and Moyamoya Disease in the Prevention of Ischemic Strokes: A Single-Center Case Series and a Systematic Review

Background: This systematic review aims to identify and analyze the available evidence on the safety and efficacy of surgical revascularization for pediatric patients with sickle cell disease (SCD) and moyamoya disease (MMD).

Methods: A systematic review was conducted following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The following databases were searched: PubMed, Ovid MEDLINE, and Scopus. Studies included in the review were original research articles in peer-reviewed journals in which individual participant data were available. The articles were thoroughly examined and compared on study design, outcomes, and results. The authors reviewed their institution's database to identify pediatric patients with SCD and MMD who underwent surgical revascularization and were included in the analysis.

Results: A total of 53 patients were included and 82 hemispheres were intervened with direct or indirect surgical revascularization. Encephaloduroarteriosynangiosis (EDAS) was the most common procedure performed (42/82; 51.2%) followed by pial synangiosis (31/82; 37.8%). There was 1 intraprocedural complication. The median clinical follow-up was 37 months (interquartile range, 24.1-73.5 months) and during this period, 3 of 52 patients (5.8%) had ischemic strokes. All ischemic strokes occurred within the first 30 days after the surgery and the rate of ischemic stroke-free survival was 94.3% (95% confidence interval, 83.3-98.1). The estimated incidence rate of ischemic stroke was 1.42 events/100 patient-years (95% confidence interval, 0.46-4.4).

Conclusions: Our study suggests that surgical revascularization in pediatric patients with SCD and MMD is safe to perform and results in a low rate of future ischemic insults.