Pediatric Moya Moya Syndrome

  • Etiology: secondary
  • Clinical: seen in neurofibromatosis type 1, Down syndrome, sickle cell disease, radiation therapy, Williams syndrome, Alagille syndrome, hemangioma, hyperthyroidism

Radiology Cases of Moya Moya Syndrome

MRA of Moya Moya syndrome in neurofibromatosis type I
AP view of a 3D maximum intensity projection of an MRA of the neck with contrast (above) and AP (below left) and inferior (below right) views of a 3D maximum intensity projection of an MRA of the brain without contrast shows diffuse hypoplasia of the left internal carotid artery. There is stenosis and occlusion of the cavernous and supraclinoid segments of the left internal carotid artery and then there is a thin left M1 segment without evidence of collaterals in the basal ganglia. Additionally, aneurysms are noted in the left A1 and A2 segments.