[Treatment of cutaneous vertebral medullary angiomatosis]

Cutaneous vertebral medullary angiomatosis, also known as Cobb's syndrome, is a part of spinal arteriovenous metameric syndromes (SAMs), is derived from the vascular malformation triad of skin, bone, and spinal cord involvement. It is poorly managed with current treatment modalities. We reviewed the treatment of Cobb's syndrome series and summarized the experiences.

A total of 61 cases of Cobb's syndrome with spinal cord dysfunction were treated at our department from February 2003 to December 2007. The treatment followed the same strategy: First step-investigating the pathogenic mechanisms by symptom onset, MRI, angiography and the response to initial treatment. Second step-treating the problematic parts of the lesions with embolization alone, surgery alone or combination of both. Forty-eight cases were embolized, 3 cases treated with surgery and 10 cases treated with a combination of both. Nidus involving pathogenic mechanism for spinal cord were eliminated, completely and nearly disappeared in 36 cases and partially in 25 cases. All patients were followed up regularly.

Among these 61 cases, the pathogenic mechanisms were identified as hemorrhage, mass effect, ischemia and venous hypertensive myelopathy. Two or more mechanisms could coexist in the same patient. More than 1-year's spinal cord function follow-up showed: excellent in 9 cases, good in 26, fair in 23 and worse in 3.

Not all lesions of Cobb's syndrome can be or should be cured anatomically. Pathogenic mechanism should be analyzed carefully and the treatment should focus on the special cord-affecting targets. Long-term improvement or stabilization may be achieved.