Pediatric Moya Moya Syndrome

  • Etiology:
    — Progressive steno-occlusive disease of the terminal internal carotid artery
    — Secondary to or associated with sickle cell disease, neurofibromatosis type 1, Trisomy 21, tuberous sclerosis, radiation therapy
  • Imaging:
    — Progressive stenosis and occlusion of distal internal carotid arteries and proximal branches of anterior cerebral arteries and middle cerebral arteries
    — Reconstitution of M1 and A1 segments via thalmoperforating arteries (puff of smoke on angiography) and cortical collaterals
  • Imaging MRI:
    — Dilated perforators in diencephalon
    — T1WI post contrast: Sulcal enhancement
    — T2WI: Lack of flow voids and subacute or chronic ischemic injury with hypointense white matter under ischemic cortex
    — FLAIR: Failure to suppress sulcal cerebrospinal fluid
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:

Radiology Cases of Moya Moya Syndrome

Radiology Cases of Moya Moya Syndrome in Neurofibromatosis Type I

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.