Moyamoya Syndrome in a Child with HbEβ-Thalassemia

Moyamoya is a progressive cerebrovascular disease associated with stenosis or occlusion of the arteries of the Circle of Willis, especially the supraclinoid internal carotid arteries (ICA), with consequent multiple collaterals. While it is common in sickle cell disease, it is rare in thalassemia. We present a 9-year-old, with HbEβ-thalassemia, who presented with headache, vomiting and episodes of transient hemiparesis. Initial imaging studies showed bilateral frontal old lacunar infarcts and narrowing of the ICA, which progressed to complete occlusion with compensatory dilatation of the basilar and vertebral arteries. She is maintained on anti-platelet therapy and is being evaluated for bypass surgery.