Stroke in a young boy with β-thalassemia intermedia secondary to moyamoya syndrome.

Moyamoya disease is a rare cerebrovascular disorder, characterized by stenosis or occlusion of cerebral arteries. Well described with sickle cell anemia, its association with other hemoglobinopathies is a rarity. We report a 5-year-old boy with β-thalassemia intermedia, on hydroxyurea therapy, who presented with a stroke. Magnetic resonance angiography findings were consistent with bilateral moyamoya disease. The literature with regard to the pathogenesis and options of management is reviewed.