Trifocal monomyelomeric spinal cord arteriovenous fistulae in a seven-year-old boy

Abstract

We describe a rare case of multiple arteriovenous fistulae of the spinal cord (SCAVF) in the same myelomer in a five-year-old boy. This case report consists of a trifocal SCAVF at the Th12 myelomeric level without communication between the three different fistulae. Two AVF were located posteriorly, bilateraly, in the spinal cord, fed by left and right posterior radiculopial arteries and one anteriorly in the anterior spinal axis. The venous drainage was independent for each lesion. The patient presents associated lesions characterized by cutaneous stain and inferior limb asymmetry. A metameric distribution is the explanation for the multiplicity of these lesions in a syndromic association related to Cobb syndrome. The patient was treated by transarterial embolization using glue with occlusion of the three different fistulae. The patient achieved a good improvement in neurological status.

Figures 1 and 2

Axial and sagittal MR views show abnormal vascular structures in Th 12 level corresponding to the SCAVM. The hyperintensity signal in the sagittal view corresponding to thrombus in metehemoglobin phase inside the venous aneurysm (arrow). There is also a hypersignal in the spinal cord itself, corresponding to cord suffering.

Figures 1 and 2

Axial and sagittal MR views show abnormal vascular structures in Th 12 level corresponding to the SCAVM. The hyperintensity signal in the sagittal view corresponding to thrombus in metehemoglobin phase inside the venous aneurysm (arrow). There is also a hypersignal in the spinal cord itself, corresponding to cord suffering.

Figure 3

Drawing with the three SCAVF in Tʼnl2 myelomer. One fistula is located anteriorly in the anterior spinal axis, fed by the left Th6 artery (A), draining through right and left L3 veins (V) and the other two fistula are located posteriorly. Note the independent venous outlet for each fistulae. The posterior fistula on the right side is fed by Th9 and Thl2 radiculopįal arteries (A) drains through the right L5 veins (V) and the fistula on the left side fed by Thll, Thl2 and L2 arteries (A) and drains through the right LI vein and the L5 vein (V).

Figures 4 and 5

Selective angiography in the left Th6 and left LI intercostal arteries shows the SCAVF in the anterior spinal axis (arrow). Note the venous drainage through right (arrowhead), left (double arrowhead) L3 vein and the filum terminale vein (triple arrowhead).

Figures 4 and 5

Selective angiography in the left Th6 and left LI intercostal arteries shows the SCAVF in the anterior spinal axis (arrow). Note the venous drainage through right (arrowhead), left (double arrowhead) L3 vein and the filum terminale vein (triple arrowhead).

Figures 6 and 7

Selective angiography in right Th9 and Thl2 arteries with the second fistulae located posteriorly in Th12 myelomer (arrow) on the right side. Note the venous drainage through the right L5 vein (arrowhead).

Figures 6 and 7

Selective angiography in right Th9 and Thl2 arteries with the second fistulae located posteriorly in Th12 myelomer (arrow) on the right side. Note the venous drainage through the right L5 vein (arrowhead).

Figures 8 and 9

Selective angiography in the left Thll and Thl2 arteries showing the third SCAVF located posteriorly in Thl2 myelomer, on the left side. Note the venous aneurysm (double arrowhead) and the anastomosis point between the left Thll and Thl2 (arrow). See the fistula point in Thl2 level (double arrow) and the venous drainage by the right LI and left L5 vein (arrowhead).

Figures 8 and 9

Selective angiography in the left Thll and Thl2 arteries showing the third SCAVF located posteriorly in Thl2 myelomer, on the left side. Note the venous aneurysm (double arrowhead) and the anastomosis point between the left Thll and Thl2 (arrow). See the fistula point in Thl2 level (double arrow) and the venous drainage by the right LI and left L5 vein (arrowhead).

Figures 10 and 11

Immediate post embolization angiogram in the right Thl2 and left Thll arteries after embolization showing total oclusion of both posterior SCAVF (compare with figures 7 and 9 respectively).

Figures 10 and 11

Immediate post embolization angiogram in the right Thl2 and left Thll arteries after embolization showing total oclusion of both posterior SCAVF (compare with figures 7 and 9 respectively).

Figure 12

Control angiogram in left Th6 six years after embolization with total occlusion of the A-V shunt, preserving the anterior spinal axis.