Pediatric moyamoya: current evidence and updates in evaluation and management.

PURPOSE OF REVIEW: Moyamoya is an important cause of stroke across the lifespan and accounts for up to 10% of pediatric ischemic strokes. Recent advances in genetic discovery, neuroimaging, surgical timing, and perioperative consensus have substantially changed the landscape of pediatric moyamoya care, necessitating a focused update for clinicians managing children with this disease. RECENT FINDINGS: Identification of RNF213 Arg4810Lys as the primary susceptibility variant and the role of a second-hit mechanism involving immune activation and hypoxia has advanced understanding of moyamoya pathogenesis and phenotypic heterogeneity. Multidelay ASL MRI has emerged as a valuable tool for perioperative perfusion assessment. A modified Delphi consensus established foundational perioperative care standards, while recent data highlight that surgical timing, postoperative hemodynamic management, and long-term neurocognitive and systemic outcomes remain critical gaps. SUMMARY: Surgical revascularization remains the mainstay of treatment and dramatically reduces stroke recurrence in children with moyamoya. Structured long-term surveillance encompassing neurocognitive assessment, mental health screening, and systemic comorbidity monitoring should be implemented as standard of care, as outcomes extend well beyond stroke prevention.