Abstract
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.