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Research ArticleNeurointervention

Dural Arteriovenous Fistulas of the Foramen Magnum Region: Clinical Features and Angioarchitectural Phenotypes

M.T. Caton, K.H. Narsinh, A. Baker, C.F. Dowd, R.T. Higashida, D.L. Cooke, S.W. Hetts, V.V. Halbach and M.R. Amans
American Journal of Neuroradiology August 2021, 42 (8) 1486-1491; DOI: https://doi.org/10.3174/ajnr.A7152
M.T. Caton
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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K.H. Narsinh
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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  • ORCID record for K.H. Narsinh
A. Baker
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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C.F. Dowd
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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R.T. Higashida
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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D.L. Cooke
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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S.W. Hetts
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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V.V. Halbach
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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M.R. Amans
aFrom the Department of Radiology and Biomedical Imaging, Interventional Neuroradiology Section, University of California, San Francisco, San Francisco, California
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  • FIG 1.
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    FIG 1.

    Venous anatomy of the FMR from above. The marginal sinus (MS) lines the margin the foramen magnum and connects to the basilar plexus (BP) anteriorly, the anterior condylar vein (ACV) laterally via the hypoglossal canal (HGC), and the suboccipital cavernous sinus inferiorly (not shown). The anterior condylar vein (AVC) connects with the anterior condylar confluence (ACC), which, in turn, communicates with the inferior petrosal sinus (IPS) and inferior petroclival vein (IPCV) and posteriorly with the lateral condylar vein (LCV), and the jugular bulb and internal jugular vein (JB/IJV). The posterior condylar (emissary) vein (PCV) exits via the posterior condylar canal (PCC).

  • FIG 2.
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    FIG 2.

    Modified grading system for FMR-AVFs shown in 4 patients having undergone lateral occipital artery (OA) injection DSA. Type 1 shows unrestricted drainage via the IJV (curved arrow) without reflux. Type 2 shows both antegrade drainage (curved arrow) and sinus reflux (dashed arrow, inferior petrosal sinus). Type 3 lesions are differentiated by the presence of cortical venous reflux, in this case, pontine perforating veins (black arrow) in addition to sinus drainage (white arrow). Type 4 lesions have restricted, exclusive drainage via cortical veins (arrowheads) without a coexisting sinus drainage pathway.

  • FIG 3.
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    FIG 3.

    Arterial anatomy of a typical type 1 FMR AVF. TOF-MRA (A) of the skull base shows abnormal flow-related enhancement in the right hypoglossal canal, corresponding to a fistula (asterisk). Arterial supply from the contralateral ascending pharyngeal artery (hypoglossal branch) crosses the foramen magnum and drains via the IJV. Relative anatomy: an anterior-posterior DSA with injection of the left ascending pharyngeal artery (B) shows hypertrophied arterial channels (double-sided arrow). Lateral DSA of the right ICA (C) shows supply to the fistula (asterisk) from the meningohyposphyseal trunk (MHT). D, Lateral DSA of the right vertebral artery (V3) shows direct contribution to fistula (asterisk) and drainage via the IJV. NMT indicates neuromeningeal trunk; MS, marginal sinus.

  • FIG 4.
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    FIG 4.

    A type 3 FMR-AVF with clinical features mimicking a carotid cavernous fistula. MRA of the FMR (A) shows flow-related enhancement in the right hypoglossal canal, corresponding to an anterior condylar vein fistula site. Coronal MRA (B) shows asymmetric flow-related enhancement of the right (white arrowhead) relative to left (clear arrowhead) cavernous sinuses. Lateral-projection DSA of the right ascending pharyngeal artery (C) shows the fistula (asterisk) with antegrade drainage via the IJV (straight arrow) and reflux via the inferior petrosal sinus (IPS, curved arrow) extending to the cavernous sinus (CS) and superior ophthalmic vein (SOV), resulting in proptosis and chemosis. Anterior-posterior DSA of the right vertebral artery (D) shows reflux via the IPS and CS, which continues to the middle cerebral vein (MCV), making this a high-risk type 3 fistula. The asterisk represents the fistulous shunt site. BA indicates the basilar artery.

Tables

  • Figures
  • Venous drainage patterns in FMR AVF

    Venous Angioarchitectural TypeClinical Presentation (% of Cases)
    Pulsatile Tinnitus and/or BruitOrbital SymptomsCN XII PalsyMyelopathyHemorrhageHeadache
    Type 1 (n = 11)90.9%9.1%0.0%0.0%0.0%27.3%
    Type 2 (n = 9)100.0%44.4%22.2%0.0%0.0%44.4%
    Type 3 (n = 6)66.7%33.3%0.0%0.0%16.7%50.0%
    Type 4 (n = 3)33.3%33.3%0.0%33.3%66.7%66.7%
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American Journal of Neuroradiology: 42 (8)
American Journal of Neuroradiology
Vol. 42, Issue 8
1 Aug 2021
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M.T. Caton, K.H. Narsinh, A. Baker, C.F. Dowd, R.T. Higashida, D.L. Cooke, S.W. Hetts, V.V. Halbach, M.R. Amans
Dural Arteriovenous Fistulas of the Foramen Magnum Region: Clinical Features and Angioarchitectural Phenotypes
American Journal of Neuroradiology Aug 2021, 42 (8) 1486-1491; DOI: 10.3174/ajnr.A7152

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Dural Arteriovenous Fistulas of the Foramen Magnum Region: Clinical Features and Angioarchitectural Phenotypes
M.T. Caton, K.H. Narsinh, A. Baker, C.F. Dowd, R.T. Higashida, D.L. Cooke, S.W. Hetts, V.V. Halbach, M.R. Amans
American Journal of Neuroradiology Aug 2021, 42 (8) 1486-1491; DOI: 10.3174/ajnr.A7152
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