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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 28:584-589, March 2007
© 2007 American Society of Neuroradiology

Case Report
SPINE

Spinal Arteriovenous Malformations Associated with Klippel-Trenaunay-Weber Syndrome: A Literature Search and Report of Two Cases

M. Rohany^a, A. Shaibani^a,b, O. Arafat^a, M.T. Walker^a, E.J. Russell^a,b, H.H. Batjer^b and C.C. Getch^b

^a Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, Ill
^b Department of Neurological Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Ill

Address correspondence to Mehdi Rohany, MD, Northwestern University Medical School, Department of Radiology, 676 North St. Clair St, Ste 1400, Chicago, IL 60611; e-mail: mrohany{at}radiology.northwestern.edu

SUMMARY: Patients with Klippel-Trenaunay-Weber syndrome present with venous varices, cutaneous capillary malformations, and tissue hypertrophy, usually involving an extremity. A small but important subset also harbors arteriovenous malformations (AVMs) of the spine. We report 2 such cases, 1 with 3 concurrent spinal arteriovenous fistulas. These cases and our review of the literature emphasize the importance of screening the spine for AVMs. In addition, it is also important to investigate for the presence of multiple spinal AVMs.