The external carotid artery as a rare feeder of a spinal dural arteriovenous fistula causing cervical myelopathy: A review of the literature

Senne Broekx; Rik Houben; Luc Stockx; Thierry Boulanger; Geert Gelin; Frank Weyns; Tom De Beule

doi:10.1016/j.bas.2021.100299

The external carotid artery as a rare feeder of a spinal dural arteriovenous fistula causing cervical myelopathy: A review of the literature

Brain Spine. 2021 Sep 28:1:100299. doi: 10.1016/j.bas.2021.100299. eCollection 2021.

Authors

Senne Broekx^{1

2}, Rik Houben², Luc Stockx³, Thierry Boulanger³, Geert Gelin⁴, Frank Weyns^{1

5}, Tom De Beule³

Affiliations

¹ Department of Neurosurgery, Ziekenhuis Oost-Limburg, 3600, Genk, Belgium.
² Department of Neurology, Ziekenhuis Oost-Limburg, 3600, Genk, Belgium.
³ Department of Interventional Radiology, Ziekenhuis Oost-Limburg, 3600, Genk, Belgium.
⁴ Department of Neuroradiology, Ziekenhuis Oost-Limburg, 3600, Genk, Belgium.
⁵ Faculty of Medicine and Life Sciences, Hasselt University, 3500, Hasselt, Belgium.

Abstract

Introduction: A causal relationship between SDAVF's and cervical myelopathy is exceedingly rare. 1-2% of these lesions are located at the craniocervical junction of which 12% are caused by arterial feeders from the external carotid artery. A correct diagnosis can be challenging with a high rate of initial misdiagnosis.

Research question: Which aspects constitute the most important potential pitfalls in the diagnostic workup and treatment of SDAVF's with feeders from the external carotid artery causing cervical myelopathy?

Material and methods: We performed a PRISMA-guided review of the literature in which fourteen articles were included. We illustrate the diagnostic hazards through one of our own cases.

Results: SDAVF's at the cervical segment contain unique clinical and radiographic characteristics which differ from those elsewhere. Cervical myelopathy is caused by a SDAVF in 2.3% of cases. Pitfalls are numerous and diagnosis can be challenging, due to a broad differential diagnosis, potential isolated lower extremity involvement and absence of spinal cord edema on MRI. MR-alterations not always correlate with fistula localization.

Discussion and conclusion: A SDAVF should be part of the differential diagnosis in patients with subacute tetraparesis. When MRI shows signal alterations in combination with enlarged perimedullary vessels, a SDAVF should be suspected. Spinal angiography should include the vertebrobasilar system, as well as the internal and external carotid arteries. Early and adequate occlusion by means of an endovascular or neurosurgical approach of the draining radicular veins should be pursued. A multidisciplinary approach is key in the diagnostic workup and treatment of these patients.

Keywords: Ascending pharyngeal artery; CASPR2, Contactin associated protein 2; CLIPPERS, Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids; CRP, C-reactive protein; Cervical myelopathy; DPPX, Dipeptidyl-peptidase-like protein; EMG, Electromyogram; External carotid artery; GABAb, Gamma aminobutyric acid; LETM, Longitudinally extensive transverse myelitis; LGLI1, Glioma-associated oncogene 1; MEP, Motor-evoked potential; MOG, Myelin oligodendrocyte glycoprotein; MRC, Medical research council; NMO, Neuromyelitis optica; Occipital artery; PCR, Polymerase chain reaction; POEMS, Polyneuropathy organomegaly endocrinopathy monoclonal gammopathy skin changes; PRISMA, Preferred reporting items for systematic reviews and meta-analyses; QUORUM, Quality of reporting of meta-analyses; Spinal dural arteriovenous fistula; mGLUR1, Metabotropic glutamate receptor 1.

Publication types

Review