Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?
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.
Department of Interventional Neuroradiology, Baptist Cardiac and Vascular Institute, Miami, FL, USA. italol@baptisthealth.net
Italo
I
Linfante
Francesca
F
Tari Capone
Guilherme
G
Dabus
Sergio
S
Gonzalez-Arias
Patricio E
PE
Lau
Edgar A
EA
Samaniego
D000293:Adolescent; D000798:Angiomatosis / Q000628:therapy*; D001165:Arteriovenous Malformations / Q000628:therapy*; D004621:Embolization, Therapeutic; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008577:Meningeal Neoplasms / Q000628:therapy*; D009460:Neurologic Examination; D010264:Paraplegia / Q000628:therapy*; D012878:Skin Neoplasms / Q000628:therapy*; D013120:Spinal Cord Neoplasms / Q000628:therapy*; D013131:Spine / Q000098:blood supply*; D013577:Syndrome; D013904:Thoracic Vertebrae / Q000098:blood supply