PMC8010312_01
Back view of the patient showing severe thoracic scoliosis and widespread small neurofibroma and Cafe-au-Lait Spots.
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F1
Physical examination showed numerous cafe-au-lait spots and dermal neurofibroma together with severe thoracic scoliosis (Figure 1).
X-radiograph showing the patient with a severe thoracic scoliosis and barrel chest.
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F2
Chest X-ray confirmed the existence of thoracic scoliosis (Figure 2).
The imaging findings of the patient. (A) Sagittal T2 weighted image showing syringomyelia exists in the C5-T2 spinal cord. (B) A hypointense flow void signal lesion (arrow) extending from C2 to C4 is located in the cervical canal and front of the left cervical spine. (C, D) The coronal and sagittal view of CT angiography showing a complicated AVF at the level of C2-C6. (E) A schematic diagram of the structure of AVF. Star indicates the fistula orifices.
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F3
T2-weighted MRI showed a syringomyelia existed in the spinal cord from C5 to T2 (Figure 3A) and a hypointense signal lesion extended from C2 to C4 which was located in the cervical canal and in front of the left cervical spine, compressing the cervical nerve root (Figure 3B). The flow void signal of the lesion suggested it might be a vascular lesion. Further cervical computed tomography angiography (CTA) illustrated a complicated AVF at the level of C2-C6 with enlargement of the C2-C4 intravertebral foramen (Figures 3C,D). From the original images of CTA, multiple fistula orifices could be identified (Figure 3E).
(A) Left vertebral angiography showing a large AVF located in the left C2-C6 level of the vertebral artery (VA). The fistula is fed by the proximal antegrade flow of the left VA. (B) Right vertebral angiography showing the AVF is fed by distal retrograde flow of right VA. (C) After occlusion of the venous plexus and distal VA, lower orifice (arrow) can be identified. (D) After endovascular embolization, left vertebral angiography showing the AVF together with left VA is occluded. (E) Right vertebral angiography showing a well preserved left posterior inferior cerebellar artery and null retrograde flow into the AVF.
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F4
Angiography showed a large AVF with multiple fistula orifices located in the left C2-C6 level of the vertebral artery (VA) (Figure 4A). The fistulae were fed by the proximal antegrade flow of left VA and distal retrograde flow of right VA (Figure 4B), and it was eventually drained to the external jugular vein through the vertebral and paravertebral venous plexus. After coil embolization step by step, the lower fistula orifice could be identified (Figure 4C). AVF fistulas together with the left VA was occluded (Figure 4D). Angiography through the right VA showed a well preserved left posterior inferior cerebellar artery and null retrograde flow into the AVF (Figure 4E).