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.
Oberoi
Sapna
S
Division of Hematology-Oncology, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Bansal
Deepak
D
Singh
Paramjeet
P
Marwaha
Ram K
RK
D002533:Cerebral Angiography; D002675:Child, Preschool; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D009072:Moyamoya Disease / Q000150:complications* / Q000473:pathology; D020521:Stroke / Q000209:etiology* / Q000473:pathology; D017086:beta-Thalassemia / Q000150:complications*