Advancements in Pediatric Moyamoya Disease: A Critical Review of Surgical Decision-Making, Outcomes, and Future Directions.

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Abstract

Pediatric moyamoya disease (MMD) is a progressive steno-occlusive arteriopathy that carries a high risk of recurrent ischemic stroke and neurocognitive decline if left untreated, with a more aggressive course in children under 4 years of age than in older children or adults. This narrative review critically synthesizes original clinical studies published between 2018 and 2026 to provide an evidence-based surgical decision-making framework. A literature search of PubMed/MEDLINE was performed, including only original articles (case series >10 patients, cohort studies) reporting surgical outcomes in children ≤18 years with MMD or moyamoya syndrome. In large contemporary series, indirect revascularization achieves postoperative stroke-free survival rates exceeding 95% and 20-year event-free survival of 97% in young children. Direct or combined bypass provides superior angiographic revascularization but does not significantly lower the long-term stroke rate compared with indirect techniques. Preoperative assessment of cerebrovascular reactivity using BOLD MRI or SPECT with acetazolamide is the strongest predictor of surgical benefit. Preliminary machine learning models using intraoperative arterial blood pressure waveforms can predict postoperative neurological events with an AUROC of 0.738. A four-step surgical algorithm based on age, hemodynamic status and vascular anatomy is proposed. Priorities for future research include an international pediatric registry, validation of AI-based risk stratification tools, and randomized comparisons of direct versus indirect bypass in children aged 4-8 years.

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