Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome: a literature search and report of two cases

Abstract

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.

Fig 1.

Case 1. Sagittal and axial T2-weighted images demonstrate the dilated venous recipient pouch for fistula 1 as well as dilated veins on the dorsal and ventral aspects of the cord (arrows, A and B). Contrast-enhanced MRA maximum intensity projections demonstrate the large venous recipient pouch of fistula 1 (arrowheads, C and D) and secondary draining veins (arrow, D). Left common iliac artery injection demonstrates a large radiculomedullary artery (curved arrow, E) arising from the left lateral sacral artery (arrow, E) and ascending toward the large venous recipient pouch supplying the fistula (not shown). Microcatheter injection at the level of fistula 1 opacifying the recipient venous pouch (arrow, F) and demonstrating secondary drainage into superiorly (arrowhead, F) and inferiorly (curved arrow, F) draining veins. Native and subtracted images from a right intercostal artery injection (T12) demonstrating the second (2) of the 3 pial fistulas (arrowhead, G) located at the level of inferior endplate of T11. Venous drainage is into a short venous channel (star), draining into a common channel (curved arrow) receiving venous drainage from both smaller fistulas (2 and 3). This common channel then drained into the venous pouch, which was the main recipient venous pouch for the largest fistula (1; arrow, G and H). Selective left T12 injection demonstrates pial fistula 3 (arrowhead, I), also at the level of the inferior endplate of T11.

Fig 2.

Case 2. Sagittal postcontrast fat-saturated T1-weighted images demonstrating partly enhanced extradural lesion in the dorsal epidural space extending from C5 to T2 with mass effect on the thecal sac (arrow, A). Axial T2-and T1-weighted MR imaging demonstrates a right dorsolateral epidural lesion of mixed signal intensity, consistent with a hematoma and probable vascular channels (arrows, B and C). Arch injection demonstrating an AVM (arrow) involving the cervical spine with venous drainage into the epidural venous (curved arrows, D) and subsequently the paraspinous veins (arrowheads, D). Right vertebral artery injection demonstrating a fistulous arteriovenous connection to a dilated multilobulated epidural vein (arrow, E) secondarily draining into extraspinal veins via a stenotic connection (curved arrow, E). Selective right costocervical (deep cervical branch) injection demonstrating supply to the AVM (F). Selective left costocervical (deep cervical branch) injection demonstrating supply to the AVM (G).