Pediatric Moya Moya Disease

  • Etiology: Idiopathic and presumed non-inflammatory autoimmune process resulting in progressive steno-occlusive disease of the terminal internal carotid artery
  • 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:
    — Presentation in childhood – seizure and TIA and ischemic stroke
    — Presentation in adults – hemorrhagic stroke

Radiology Cases of Moya Moya Disease

MRA of Moya Moya disease
AP view of a 3D MR angiogram with contrast of the brain shows marked narrowing of the bilateral supraclinoid internal carotid arteries and nonvisualization of the A1 and M1 segments of the anterior and middle cerebral arteries with multiple lenticulostriate collateral vessels arising from the terminal portions of the supraclinoid internal carotid arteries which are reconstituting the A2 and M2 segments.