Multifocal and metameric spinal cord arteriovenous malformations. Review of 19 cases

Abstract

We describe 19 (16.0%) multiple vascular malformations (AVMs) in 119 spinal cord arteriovenous malformations (SCAVMs). The associated lesions were eight vertebral vascular malformations, two cutaneous, four limbs, four radicular AVMs, three bifocal SCAVMs; one patient had a bifocal cord lesion associated with vertebral and limb localisations. Various syndromic associations were seen: nine Cobb, two Klippel-Trenaunay-Weber, one Parkes Weber. An additional subgroup of unclassified associations is constituted by seven cases with bifocal intradural uni or multimetameric lesions. In our SCAVMs series, the incidence ofmultipie vascular lesions is high, in particular multifocal intradural malformations. Metameric distribution is the most frequent type of multiplicity. Identification of the myelomeric level involved in SCAVM allows segmental link between various lesions of mesodermal or neural crest origin to be discussed.

Figure 1

Case 1. Left vertebral angiogram (A) shows the extensive SCAVM nidus, the right one (B) demonstrates the associated bone AVM.

Figure 2

Case 13. (A) LI injection visualizes the spinal cord nidi as well as the spinal AVM. Iliac arteriography (B and C) opacifies the thigh AVM partially resected in the past.

Figure 3

Case 14. Costocervical injection (A and B) shows bifocal SCAVM with distinct venous drainage.

Figure 4

Case 18. L2 injection (A) demonstrates a large SCAVM. L3 (B) visualises a radicular AVM draining into the same venous outlets (C).

Figure 5

Schematic representation of the arterial determination of myelomeric levels.

Figure 6

Case 16. Vertebral injection opacifies a SCAVM at C5 (A) and the thyrocervical trunk injects a C5 nerve AVM (B) draining into the SCAVM outlets.