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A case involving a giant aberrant craniocervical arteriovenous malformation
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Accepted: ,
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Abstract
Background:
Spinal cord arteriovenous malformations (AVMs) comprise about 3%–4% of primary intraspinal masses and are only rarely found external to the C2–C7 cervical vertebral foramen.
Case Description:
A 21-year-old female presented with neck pain and a spastic quadriparesis of 1 year duration. The cervical magnetic resonance imaging and three-dimensional computed tomography angiograms documented an AVM/dural arteriovenous fistula on the right fed by multiple arteries located in the C5–C6 and C6–C7 foramen intervertebralis; utilizing a laminectomy, the large feeding arteries were double-clipped. This allowed for devascularization of the AVM and facilitated resection while preserving the aberrant vertebral artery. The patient was discharged within 1 week and, 2 months later, was able to ambulate to the outpatient clinic.
Conclusion:
Double clipping of the two main right-sided arterial feeders at the C5–C6 and C6–C7 levels allowed for devascularization and resection of this large cervical AVM while carefully preserving the aberrant vertebral artery.
Keywords
Aberrant vertebral artery
Craniocervical junction
Dural arteriovenous fistulas
Spinal cord arteriovenous malformation
INTRODUCTION
Spinal arteriovenous malformations (AVMs) may be variously treated with microsurgery, endovascular embolization, and radiation therapy. However, surgery may be associated with considerable morbidity. Here, we present a type 1 AVM found in a 21-year-old female treated with both microsurgery and endovascular embolization.[1-4]
CASE REPORT
History and examination
A 21-year-old female presented with a progressive spastic cervical quadriparesis of 1 year duration. This was accompanied by significant bilateral upper and lower extremity spasticity and urinary retention.
Imaging studies
The craniocervical magnetic resonance imaging/magnetic resonance angiography/digital subtraction angiography revealed a large C1–C7 right-sided cervical spinal dural AVM; the main two feeders were coming from the C5–C6 and C6–C7 neural foramen [Figures 1 and 2]. Smaller feeders were also coming from the C2–C3 foramen, in conjunction with huge draining veins. Three-dimensional computed tomography angiography further elucidated the location of the AVM and its feeders [Figure 3].
Surgery and post-operative course
Using a midline C1–C7 cervical exposure, a laminectomy was performed at the C5 and C6 levels. On the right, huge feeding arteries were identified entering the respective vertebral foramina of C5–C6 and C6–C7; utilizing extensive foraminotomies, double curved permanent clips were placed on the major feeders at each respective level. This allowed for decompression and resection of the AVM and was followed by lateral screws/rod fusion [Figure 4]. Her quadriparesis continued to improve over 6 post operative days, and she was discharged on day 7. 2 months later, she was able to ambulate (e.g., Karnofsky Performance Status score 100) [Figure 5].
DISCUSSION
Vascular malformations of the spinal cord and dura account for approximately 3%–4% of spinal cord lesions; they are the least common in the cervical region.[6] Our patient, with a 1-year onset of a spastic quadriparesis, had a spinal type I AVM with multiple feeders that originated predominantly from the right vertebral artery (C5–C6 and C6–C7 neural foramina).
Reports of Other Aberrant VA Most vertebral arteries enter the transverse foramina at the C6 level (93%, 855 cases of 920 cases).[7,5] Ulusoy et al. reported a 15-year-old female with a Klippel–Feil syndrome who showed an extraforaminal, cranially ascending aberrant right VA that originated from the ipsilateral carotid bulb. Shin et al. also reported a similar aberrant VA. In our case, the feeding arteries originated from the right C5–C6 nd C6–C7 transverse foramen; laminectomy and foraminal decompression allowed for double clipping of the feeders, facilitating decompression, and subsequent complete resection of the AVM while preserving the aberrant right vertebral artery. The patient then underwent C5–C6 lateral mass screw fixation/fusion [Figure 4]. Within 2 post operative months, her spastic quadriparesis markedly improved [Figure 5].
CONCLUSION
For patients with cervical AVM, preserving the aberrant VA while double clipping other AVM feeders is critical to maintain neurological function.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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