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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March 16, 2015
Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome)
- Cobb syndrome is a complex of vascular malformations affecting the spinal cord, epidural space, vertebral body, paraspinal soft tissue, muscle, subcutaneous tissue, and skin that are derived from the same spinal metameric segment. For this reason, Cobb syndrome is also referred to as spinal arteriovenous metameric syndrome.
- Clinical features could be related to spinal cord hemorrhage, subarachnoid hemorrhage, mass effect of the spinal cord and roots from dilated arteries and veins, venous hypertension, and vascular steal phenomenon.
- Key Diagnostic Features: The finding of vascular malformations affecting more than two tissues in a given spinal metamere confirms the diagnosis. However, involvement of all of the tissue elements in the metamere is rare. Skin vascular malformation can manifest as a distinct nevus or a subtle discoloration, and may be the first recognizable sign of this complex disease. The vascular malformations often affect more than one spinal metameric segment.
- DDx: Spinal cord AVM, spinal cord AVF
- Rx: Palliative endovascular embolization. Due to the diffuse and extensive nature of the malformation, surgical excision or endovascular embolization aiming complete cure is difficult. Targeted embolization of the symptomatic lesion may be the choice of therapy.