ReviewChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2026
Moyamoya syndrome associated with hereditary spherocytosis: pathogenesis, management, and an illustrative case with rapid radiological progression.
Review in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundThe association between moyamoya syndrome (MMS) and hereditary spherocytosis (HS) is exceedingly rare, with only eight pediatric cases reported to date. This review explores the clinical intersection, pathophysiological theories, and management challenges of this dual pathology. CASE DESCRIPTION: We present a 13-year-old boy with known HS who presented with acute left limb weakness. Imaging confirmed MMS. Over a 3-year follow-up, serial magnetic resonance imaging (MRI) and digital subtraction angiography (DSA) demonstrated significant progression of cerebrovascular steno-occlusive disease. Management included chronic anemia correction, aspirin therapy, subsequent laparoscopic splenectomy, and ultimately, unilateral encephaloduroarteriosynangiosis (EDAS) revascularization surgery for symptomatic progression.
conclusionThis case, the ninth reported, suggests that chronic hemolysis and anemia in HS may potentiate rapid progression of MMS. It indicates the necessity of vigilant neurological monitoring in HS patients, advocates for maintaining hemoglobin levels > 110 g/L, and discusses the integrated roles of splenectomy and revascularization surgery. Early recognition and a multidisciplinary treatment strategy are crucial to mitigating stroke risk in this unique patient population.
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