Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026
Moyamoya syndrome associated with hereditary spherocytosis: pathogenesis, management, and an illustrative case with rapid radiological progression.
СтатьяКлинический случайОбзорПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Yan Lin, Lu Xia, Yang Bin, Ma Yan
Аннотация
BACKGROUND: The 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.
CONCLUSION: This 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.
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.
CONCLUSION: This 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.