Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?

Case report

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Cutaneomeningospinal angiomatosis, or Cobb syndrome, is a rare metameric developmental disorder presenting as an extradural-intradural vascular malformation that involves bone, muscle, skin, spinal cord, and nerve roots.

A 14-year-old girl with a red nevus involving the T6–9 dermatomes on the left side of her back presented with a 5-year history of bowel and bladder incontinence, paraplegia, and lower-extremity sensory loss. Magnetic resonance imaging demonstrated a hemangioma in the T-8 and T-9 vertebral bodies and a spinal cord AVM nidus extending from T-6 to T-9. The AVM was successfully embolized and the patient regained lower-extremity strength, ambulation, and normal sphincter functions after 5 years of having been wheelchair bound.

The authors report the restoration of ambulation after endovascular embolization of a large spinal AVM in a patient with long-standing paraplegia due to Cobb syndrome.

Article Information

Address correspondence to: Italo Linfante, M.D., Interventional Neuroradiology, Endovascular Neurosurgery, Baptist Cardiac and Vascular Institute, 8900 North Kendall Drive, Miami, Florida 33176. email: italol@baptisthealth.net.

Please include this information when citing this paper: published online January 6, 2012; DOI: 10.3171/2011.12.SPINE11636.

© AANS, except where prohibited by US copyright law.

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Figures

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    A: Sagittal thoracic T2-weighted MR image demonstrating an intramedullary AVM between T-6 and T-9 (black arrow) and hyperintense signal in the T-8 and T-9 vertebrae (white arrows), suggestive of a vertebral angioma. B: Postembolization sagittal T2-weighted MR image revealing substantial reduction of the AVM-related flow voids.

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    A: Superselective left T-8 radiculomedullary angiogram demonstrating the AVM nidus. Vascular involvement of soft tissues surrounding the AVM in a metameric distribution (arrowhead, A). B: Microcatheter injection proximal to the AVM nidus. C: Postembolization angiogram demonstrating no residual contrast opacification of the AVM nidus.

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    A: Hyperplastic arteria radicularis magna (arrowhead) arising from the left T-9 intercostal artery and providing arterial inflow to the AVM nidus. B: Microcatheter injection proximal to the AVM nidus through the arteria radicularis magna. C: Postembolization angiogram demonstrating the Onyx cast (arrow) and no residual contrast opacification of the AVM nidus.

  • View in gallery

    A: Superselective spinal angiogram of the right T-7 radiculomedullary branch demonstrating the AVM nidus. B: Microcatheter injection proximal to the AVM nidus. C: Postembolization angiogram demonstrating the Onyx cast (arrow) and no residual nidus.

  • View in gallery

    Two months after the first embolization, the remaining portion of the AVM nidus arising from the left T-6 radiculomedullary artery was selected. A and B: Angiograms from the guide catheter and the microcatether demonstrating residual AVM nidus. C: Postembolization angiogram demonstrating an Onyx cast (arrow) and no residual AVM nidus.

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