Moyamoya syndrome with ruptured aneurysm in α-thalassemia: A case report.

ABSTRACT

Moyamoya syndrome (MMS) refers to the moyamoya vascular disease associated with various systemic diseases and conditions, including sickle cell anemia, Fanconi anemia and iron deficiency anemia. However, the association between MMS and other hemoglobinopathies is less frequently observed. MMS, like moyamoya disease, is a cerebrovascular condition that is characterized by chronic progressive stenosis or occlusion at the ends of the bilateral internal carotid arteries, anterior cerebral arteries and the beginning of the middle cerebral arteries, and is secondary to the formation of an abnormal vascular network at the base of the skull. Patients with MMS are prone to thrombosis, aneurysm and bleeding. The present study reports the case of a 43-year-old man with α-thalassemia who presented with moyamoya vessels with a ruptured aneurysm bleeding into the ventricle. α-thalassemia is considered as an extremely rare but potential cause of MMS. Since MMS is a progressive disease, early diagnosis and treatment is vital to prevent the disease from worsening.