Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 4, 2011
Moyamoya Disease
- Moyamoya disease is a rare vascular disorder characterized by progressive, idiopathic narrowing of the supraclinoid segment of the internal carotid artery (ICA), with secondary collateralization.
- Seen more often in children and young females, it presents with developmental delay, poor feeding, TIAs, and alternating hemiplegia.
- Key imaging features: FLAIR: Bright sulci, asymmetric presence of prominent VR spaces, less often asymmetric ischemic foci. MRA: Narrowing of distal ICA and proximal circle of Willis vessels, and collateralization. "Puff or spiral of smoke" on angiography. Perfusion imaging: Hypoperfused areas which can guide further management.
- DDx: Subarachnoid hemorrhage, meningeal carcinomatosis, meningitis, high inspired oxygen, carotid dissection.
- Treatment: Medical and surgical: encephalo-duro-arterio-synangiosis (EDAS). Surgery helps but does not prevent the progression of disease.
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