Moyamoya Disease: A Rare Sickle Cell Trait Neurological Complication

Introduction: Sickle cell disease is an inherited blood disorder with multisystem complications, including cerebrovascular accidents primarily due to micro infarcts. However, people with sickle cell trait, the heterozygous form of sickle cell disease, usually lead a normal, healthy life. Very rarely, severe tissue hypoxia, acidosis, dehydration, and hypothermia in sickle cell trait increase red blood cell polymerization and sickling, which could lead to micro infarcts in blood vessels. Moyamoya disease is a rare neurovascular complication caused by repeated strokes after arterial cerebral infarction. It commonly manifests as ischemic stroke in young patients, but mainly as intracranial hemorrhage in adults. Case presentation: A 14-year-old girl with sickle cell trait, who had been living a normal and healthy life, started to complain of severe persisting headache, migraine, drowsiness, and a tendency to sleep. Brain magnetic resonance imaging and angiography revealed moyamoya disease. Hematological treatment with hydroxyurea, aspirin, and an NSAID was commenced for this patient. Conclusion: Under rare circumstances, sickle cell trait could lead to sickle cell disease complications. Although moyamoya has been seen in sickle cell disease, it is a very rare neurological complication of sickle cell trait. To the best of our knowledge, only three cases of moyamoya have been reported in individuals with sickle cell trait.