Hypoglossal canal dural arteriovenous fistula: incidence and the relationship between symptoms and drainage pattern

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The purpose of this study was to evaluate the incidence, radiographic findings, relationship between presenting symptoms for treatment and drainage pattern, and treatment outcomes of hypoglossal canal dural arteriovenous fistula (HC-dAVF).


During a 16-year period, 238 patients underwent endovascular treatment for cranial dAVF at a single center. The incidence, radiographic findings, relationship between presenting symptoms for treatment and drainage pattern, and treatment outcomes of HC-dAVF were retrospectively evaluated.


The incidence of HC-dAVF was 4.2% (n = 10). Initial symptoms were tinnitus with headache (n = 6), tinnitus only (n = 1), ocular symptoms (n = 1), otalgia (n = 1), and congestive myelopathy (n = 1). Presenting symptoms requiring treatment included ocular symptoms (n = 4), hypoglossal nerve palsy (n = 4), aggravation of myelopathy (n = 1), and aggravation of tinnitus with headache (n = 1). While the affected HC was widened in 4 of 10 patients, hypersignal intensity on source images was conspicuous in all 7 patients who underwent MR angiography (MRA). All ocular symptoms and congestive myelopathy were associated with predominant drainage to superior ophthalmic or perimedullary veins due to antegrade drainage restriction. All patients who underwent transvenous coil embolization (n = 8) or transarterial N-butyl cyanoacrylate (NBCA) embolization (n = 1) improved without recurrence. One patient who underwent transarterial particle embolization had a recurrence 12 months posttreatment and was retreated with transvenous embolization.


The incidence of HC-dAVF was 4.2% of all cranial dAVF patients who underwent endovascular treatment. Source images of MRA helped to accurately diagnose HC-dAVF. More aggressive symptoms may develop as a result of a change in the predominant drainage route due to the development of venous stenosis or obstruction over time. Transvenous coil embolization appears to be the first treatment of choice.

Abbreviations used in this paper:dAVF = dural arteriovenous fistula; DSA = digital subtraction angiography; HC = hypoglossal canal; HC-dAVF = hypoglossal canal dural arteriovenous fistula; MRA = MR angiography; NBCA = N-butyl cyanoacrylate; 3D-TOF = 3D time-of-flight.

Article Information

Address correspondence to: Byung Moon Kim, M.D., Ph.D., Division of Interventional Neuroradiology, Department of Radiology, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, 120-752, Seodaemun-gu, Seoul, South Korea. email: bmoon21@hanmail.net.

Please include this information when citing this paper: published online May 31, 2013; DOI: 10.3171/2013.4.JNS121974.

© AANS, except where prohibited by US copyright law.



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    Case 1. A 68-year-old man presenting with headache and tinnitus for 2 months after right exophthalmos and conjunctival injection for 2 weeks. A: Cranial CT with bone window shows widening of the left HC. B: Right external carotid artery angiogram well delineates a left HC-dAVF (short arrow) with retrograde venous drainage due to stenosis of the antegrade drainage route through the left jugular vein (long arrow). Arrowhead indicates cortical venous reflux through the right sylvian vein. C: Right external carotid artery angiogram after transvenous coil embolization shows complete occlusion of the fistula.

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    Case 6. A 47-year-old woman presenting with right hypoglossal palsy and leg weakness for 1 week after headache with tinnitus for 3 months. A: Source image of MRA shows high signal intensity on the right HC. B and C: Anteroposterior (B) and lateral (C) views of the left external carotid artery angiogram show the fistula site (arrow, B) and predominant drainage to the vertebral venous plexus (arrowheads, B and C) without visible drainage either antegrade to the jugular vein or retrograde to the cavernous sinus. D: Postembolization angiogram shows near-complete obliteration of the fistula.

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    Case 8. A 62-year-old woman presenting with left blurred vision and diplopia for 1 week after left exophthalmos for 18 months. A: Left external carotid artery angiogram shows the fistula between the neuromeningeal branch (arrowheads) of the left ascending pharyngeal artery and the left anterior condylar vein (short arrow) in the HC and a stenotic junction (long arrow). B and C: Oblique sagittal reconstruction image of an angiographic CT scan (B) and its schematic diagram (C) show a well-delineated feeding artery (arrowheads, B), anterior condylar vein in the HC (short arrow, B), stenotic junction (long arrow, B) between the anterior condylar vein and inferior petrosal sinus, the cavernous sinus and superior ophthalmic vein, and their relationship with surrounding bone structures. D: Oblique sagittal reconstruction image of angiographic CT scan after transarterial embolization reveals lipiodol-NBCA mixture filling from feeding artery (arrowheads) to the junction (long arrow) between the anterior condylar vein (short arrow) and the inferior petrosal sinus. Note that some lipiodol-NBCA mixture migrated to the cavernous sinus. E: Postembolization angiogram shows complete obliteration of the fistula. ACV = anterior condylar vein; APA = ascending pharyngeal artery; IPS = inferior petrosal sinus; SOV = superior ophthalmic vein.

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    Case 10. A 51-year-old man presenting with aggravating congestive cervical myelopathy over 2 months. A: T2-weighted sagittal cervical spine image showing engorged anterior spinal and pial veins along with the surface of cervical spinal cord with mild signal change. B: Right external carotid artery angiogram shows an HC-dAVF that was mainly fed by branches of the ascending pharyngeal artery (white arrow). Note retrograde drainage through markedly enlarged perimedullary veins to the anterior spinal vein (arrowheads). C and D: Oblique sagittal reconstruction of an angiographic CT scan (C) and its schematic diagram (D) disclose the retrograde drainage via the bridging vein to perimedullary veins (arrowheads, C) and the severe stenosis (long black arrow, C), where the anterior condylar confluent drained to the jugular vein, and also well delineate the surrounding structures, including the inferior petrosal sinus (short black arrow, C) and feeding branches (white arrows, C) from the ascending pharyngeal artery. E: Angiographic CT scan also helps to accurately guide the microcatheter in the fistula site. F: Postembolization angiogram showing complete obliteration of the fistula. G: The 6-month follow-up T2-weighted sagittal cervical spine image shows complete regression of the engorged veins. ICA = internal carotid artery; IJV = internal jugular vein.



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