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

J Neurosurg Spine. 2012 Apr;16(4):408-13. doi: 10.3171/2011.12.SPINE11636. Epub 2012 Jan 6.


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.

MeSH terms

  • Adolescent
  • Angiomatosis / therapy*
  • Arteriovenous Malformations / therapy*
  • Embolization, Therapeutic*
  • Female
  • Follow-Up Studies
  • Humans
  • Meningeal Neoplasms / therapy*
  • Neurologic Examination
  • Paraplegia / therapy*
  • Skin Neoplasms / therapy*
  • Spinal Cord Neoplasms / therapy*
  • Spine / blood supply*
  • Syndrome
  • Thoracic Vertebrae / blood supply