Review article| Volume 110, P27-38, April 2023

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The role of venous anatomy in guiding treatment approach for dural arteriovenous fistulas of the craniocervical junction; case series & systematic review


      • DAVF of the craniocervical junction can have different venous drainage patterns.
      • The venous drainage can be inferior, superior or both.
      • The anatomy of the DAVF predicts the clinical presentation of the patient. Patients with superior drainage are more likely to present with a hemorrhage whereas patients with inferior drainage tend to present with myelopathy.
      • In our paper, we propose a novel classification system in which DAVF of the craniocervical junction can be grouped into three types and for each type, we present a treatment paradigm.
      • Microsurgery remains an effective treatment option. There is an emerging role of transvenous embolization particularly for superior draining DAVF.



      Dural arteriovenous fistulas (DAVF) of the craniocervical junction (CCF) are an uncommon entity with the following venous drainage pattern: inferior, superior and mixed. Patients may present with subarachnoid hemorrhage, myelopathy or brainstem dysfunction. CCJ DAVF can be treated with microsurgery or with transarterial and transvenous embolization, depending on the venous drainage pattern. We present our institutional experience of treating CCJ DAVFs along with a systematic review of the literature.


      Six patients with CCJ DAVF were treated at our institution over five years. Data was collected using electronic medical record review. Systematic review was performed on CCJ DAVF using the PubMed database from 1990 to 2021. We characterized venous drainage patterns, treatment choices, and outcomes to create a classification system.


      50 case reports, consisting of 115 patients, were included in our review. 61 (53.0 %) patients had inferior drainage while 32 (27.8 %) patients had superior drainage and 22 (19.2 %) patients had mixed venous drainage. Patients with inferior drainage had the fistulous connection at the foramen magnum while patients with superior drainage had a fistulous connection at C1-C2 (p value = 0.026). Patients with inferior drainage were more likely to present with myelopathy while patients with superior drainage presented with hemorrhage (p value = 0.000).


      Classifying the venous drainage pattern is essential in making treatment decision. Transvenous embolization works best with large superior venous drainage. If endovascular treatment is not an option, then surgical clipping can achieve successful cure. Transarterial embolization is a reasonable option in cases with a large arterial feeder.


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        • Maimon S.
        • Luckman Y.
        • Strauss I.
        Spinal dural arteriovenous fistula: a review.
        Adv Tech Stand Neurosurg. 2016; : 111-137
        • Wang J.Y.
        • Molenda J.
        • Bydon A.
        • Colby G.P.
        • Coon A.L.
        • Tamargo R.J.
        • et al.
        Natural history and treatment of craniocervical junction dural arteriovenous fistulas.
        J Clin Neurosci. 2015; 22: 1701-1707
        • Hiramatsu M.
        • Sugiu K.
        • Ishiguro T.
        • Kiyosue H.
        • Sato K.
        • Takai K.
        • et al.
        Angioarchitecture of arteriovenous fistulas at the craniocervical junction: a multicenter cohort study of 54 patients.
        J Neurosurg. 2018; 128: 1839-1849
        • Shinoyama M.
        • Endo T.
        • Takahash T.
        • Shimizu H.
        • Takahashi A.
        • Suzuki M.
        • et al.
        Long-term Outcome of Cervical and Thoracolumbar Dural Arteriovenous Fistulas With Emphasis on Sensory Disturbance and Neuropathic Pain.
        World Neurosurg. 2010; 73: 401-408
        • Zhong W.
        • Zhang J.i.
        • Shen J.
        • Su W.
        • Wang D.
        • Zhang P.
        • et al.
        Dural Arteriovenous Fistulas at the Craniocervical Junction: A Series Case Report.
        World Neurosurg. 2019; 122: e700-e712
        • Cognard C.
        • Gobin Y.P.
        • Pierot L.
        • Bailly A.L.
        • Houdart E.
        • Casasco A.
        • et al.
        Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage.
        Radiology. 1995; 194: 671-680
        • Zhao J.
        • Xu F.
        • Ren J.
        • Manjila S.
        • Bambakidis N.C.
        Dural arteriovenous fistulas at the craniocervical junction: a systematic review.
        J NeuroInterventional Surg. 2016; 8: 648-653
        • Kai Y.
        • Hamada J.
        • Morioka M.
        • Ushio Y.
        • Fujioka S.
        Foramen magnum dural arteriovenous fistulae with repeated subarachnoid haemorrhage.
        Interv Neuroradiol. 1998; 4: 171-176
        • Yoshida S.
        • Oda Y.
        • Kawakami Y.
        • Sato S.
        Progressive Myelopathy Caused by Dural Arteriovenous Fistula at the Craniocervical Junction —Case Report&mdash.
        Neurol Med Chir. 1999; 39: 376-379
        • Liang G.
        • Gao X.u.
        • Li Z.
        • Wang X.
        • Zhang H.
        • Wu Z.
        Endovascular treatment for dural arteriovenous fistula at the foramen magnum: report of five consecutive patients and experience with balloon-augmented transarterial Onyx injection.
        J Neuroradiol. 2013; 40: 134-139
        • Hiramatsu M.
        • et al.
        Results of 1940 embolizations for dural arteriovenous fistulas: Japanese Registry of Neuroendovascular Therapy (JR-NET3).
        J Neurosurg. 2019; 133: 1-8
        • Miyachi S.
        Endovascular treatment for dural arteriovenous fistula.
        Brain Nerve. 2008; 60: 907-914
        • Hiramatsu R.
        • Nozaki K.
        • Kuroiwa T.
        Foramen magnum dural arteriovenous fistula treated by a microsurgical technique combined with a feeder occlusion using transarterial coil embolization.
        Turk Neurosurg. 2015; 25: 971-975
        • Gaensler E.H.
        • Jackson Jr., D.E.
        • Halbach V.V.
        Arteriovenous fistulas of the cervicomedullary junction as a cause of myelopathy: radiographic findings in two cases.
        AJNR Am J Neuroradiol. 1990; 11: 518-521
        • Willinsky R.
        • Terbrugge K.
        • Lasjaunias P.
        • Montanera W.
        The variable presentations of craniocervical and cervical dural arteriovenous malformations.
        Surg Neurol. 1990; 34: 118-123
        • Mascalchi M.
        • Scazzeri F.
        • Prosetti D.
        • Ferrito G.
        • Salvi F.
        • Quilici N.
        Dural arteriovenous fistula at the craniocervical junction with perimedullary venous drainage.
        Am J Neuroradiol. 1996; 17: 1137-1141
        • Niwa J.
        • Matsumura S.
        • Maeda Y.
        • Ohoyama H.
        Transcondylar Approach for Dural Arteriovenous Fistulas of the Cervicomedullary Junction.
        Surg Neurol. 1997; 48: 627-631
        • Kinouchi H.
        • Mizoi K.
        • Takahashi A.
        • Nagamine Y.
        • Koshu K.
        • Yoshimoto T.
        Dural arteriovenous shunts at the craniocervical junction.
        J Neurosurg. 1998; 89: 755-761
        • Do H.M.
        • Jensen M.E.
        • Cloft H.J.
        • Kallmes D.F.
        • Dion J.E.
        Dural arteriovenous fistula of the cervical spine presenting with subarachnoid hemorrhage.
        Am J Neuroradiol. 1999; 20: 348-350
        • Hurst R.W.
        • Bagley L.J.
        • Scanlon M.
        • Flamm E.S.
        Dural arteriovenous fistulas of the craniocervical junction.
        Skull Base Surg. 1999; 9: 1-7
        • Oishi H.
        • Okuda O.
        • Arai H.
        • Maehara T.
        • Iizuka Y.
        Successful surgical treatment of a dural arteriovenous fistula at the craniocervical junction with reference to pre- and postoperative MRI.
        Neuroradiology. 1999; 41: 463-467
        • Hashimoto H.
        • Iida J.-I.
        • Shin Y.
        • Hironaka Y.
        • Sakaki T.
        Iida J-i, Shin Y, Hironaka Y, Sakaki T: Spinal dural arteriovenous fistula with perimesencephalic subarachnoid haemorrhage.
        J Clin Neurosci. 2000; 7: 64-66
        • Reinges M.H.T.
        • Thron A.
        • Mull M.
        • Huffmann B.C.
        • Gilsbach J.M.
        Dural arteriovenous fistulae at the foramen magnum.
        J Neurol. 2001; 248: 197-203
        • Kim M.S.
        • Han D.H.
        • Han M.H.
        • Oh C.-W.
        Posterior fossa hemorrhage caused by dural arteriovenous fistula: case reports.
        Surg Neurol. 2003; 59: 512-516
        • Takami T.
        • Ohata K.
        • Nishio A.
        • Nishikawa M.
        • Goto T.
        • Tsuyuguchi N.
        • et al.
        Microsurgical interruption of dural arteriovenous fistula at the foramen magnum.
        J Clin Neurosci. 2005; 12: 580-583
        • Ansari S.A.
        • Lassig J.P.
        • Nicol E.
        • Thompson B.G.
        • Gemmete J.J.
        • Gandhi D.
        Transarterial embolization of a cervical dural arteriovenous fistula. Presenting with subarachnoid hemorrhage.
        Interv Neuroradiol. 2006; 12: 313-318
        • Hashiguchi A.
        • Mimata C.
        • Ichimura H.
        • Kuratsu J.-I.
        Occipital Neuralgia As a Presenting Symptom of Cervicomedullary Dural Arteriovenous Fistula.
        Headache: J Head Face Pain. 2007; 47: 1095-1097
        • Fassett D.R.
        • Rammos S.K.
        • Patel P.
        • Parikh H.
        • Couldwell W.T.
        Intracranial subarachnoid hemorrhage resulting from cervical spine dural arteriovenous fistulas: literature review and case presentation.
        Neurosurg Focus FOC. 2009; 26: E4
        • Guo L.-M.
        • Zhou H.-Y.
        • Xu J.-W.
        • Wang G.-S.
        • Tian X.
        • Wang Y.
        • et al.
        Dural arteriovenous fistula at the foramen magnum presenting with subarachnoid hemorrhage: case reports and literature review.
        Eur J Neurol. 2010; 17: 684-691
      1. Chen G, Wang Q Fau - Tian Y, Tian Y Fau - Gu Y, et al.: Dural arteriovenous fistulae at the craniocervical junction: the relation between clinical symptom and pattern of venous drainage; 2011.

        • Beynon C.
        • Herweh C.
        • Rohde S.
        • Unterberg A.W.
        • Sakowitz O.W.
        Intraoperative indocyanine green angiography for microsurgical treatment of a craniocervical dural arteriovenous fistula.
        Clin Neurol Neurosurg. 2012; 114: 696-698
        • Kulwin C.
        • Bohnstedt B.N.
        • Scott J.A.
        • Cohen-Gadol A.
        Dural arteriovenous fistulas presenting with brainstem dysfunction: diagnosis and surgical treatment.
        Neurosurgical Focus FOC. 2012; 32: E10
        • Suda S.
        • Katsura K.-I.
        • Okubo S.
        • Abe A.
        • Kanamaru T.
        • Ueda M.
        • et al.
        A Case of Dural Arteriovenous Fistulas at the Craniocervical Junction Presenting with Occipital/Neck Pain Associated with Sleep.
        Intern Med. 2012; 51: 925-928
        • Salamon E.
        • Patsalides A.
        • Gobin Y.P.
        • Santillan A.
        • Fink M.E.
        Dural Arteriovenous Fistula at the Craniocervical Junction Mimicking Acute Brainstem and Spinal Cord Infarction.
        JAMA Neurol. 2013; 70: 796
        • Wu Q.
        • Wang H.-D.
        • Shin Y.S.
        • Zhang X.
        Brainstem Congestion due to Dural Ateriovenous Fistula at the Craniocervical Junction.
        J Korean Neurosurg Soc. 2014; 55: 152-155
        • Pop R.
        • Manisor M.
        • Aloraini Z.
        • et al.
        Foramen magnum dural arteriovenous fistula presenting with epilepsy.
        Interv Neuroradiol. 2015; 21: 724-727
        • Kasliwal M.K.
        • Moftakhar R.
        • O'Toole J.E.
        • Lopes D.K.
        High cervical spinal subdural hemorrhage as a harbinger of craniocervical arteriovenous fistula: an unusual clinical presentation.
        Spine J. 2015; 15: e13-e17
        • Inamasu J.
        • Tanaka R.
        • Nakahara I.
        • Hirose Y.
        Dural arteriovenous fistula of the craniocervical junction manifesting as cerebellar haemorrhage.
        Neuroradiol J. 2016; 29: 356-360
        • Do A.-S.-M.
        • Kapurch J.
        • Kumar R.
        • Port J.
        • Miller J.W.
        • Van Gompel J.J.
        The Long and Winding Road: Thoracic Myelopathy Associated With Occipitocervical Dural Arteriovenous Fistula.
        World Neurosurg. 2017; 108: 998
        • Liu X.
        • Ogata A.
        • Masuoka J.
        • et al.
        Dural arteriovenous fistula manifesting as pontine hemorrhage at the craniocervical junction.
        Acta Neurochir. 2017; 159: 831-834
        • Lang M.J.
        • Atallah E.
        • Tjoumakaris S.
        • Rosenwasser R.H.
        • Jabbour P.
        Remote Thoracic Myelopathy From a Spinal Dural Arteriovenous Fistula at the Craniocervical Junction: Case Report and Review of Literature.
        World Neurosurg. 2017; 108: 992.e991-992.e994
        • Enokizono M.
        • Sato N.
        • Morikawa M.
        • et al.
        “Black butterfly” sign on T2*-weighted and susceptibility-weighted imaging: A novel finding of chronic venous congestion of the brain stem and spinal cord associated with dural arteriovenous fistulas.
        J Neurol Sci. 2017; 379: 64-68
        • Takahashi H.
        • Ueshima T.
        • Goto D.
        • et al.
        Acute Tetraparesis with Respiratory Failure after Steroid Administration in a Patient with a Dural Arteriovenous Fistula at the Craniocervical Junction.
        Intern Med. 2018; 57: 591-594
        • Wang X.-c.
        • Du Y.-y.
        • Tan Y.
        • et al.
        Brainstem Congestion Due to Dural Arteriovenous Fistula at the Craniocervical Junction: Case Report and Review of the Literature.
        World Neurosurg. 2018; 118: 181
        • Fujimoto S.
        • Takai K.
        • Nakatomi H.
        • Kin T.
        • Saito N.
        Three-dimensional angioarchitecture and microsurgical treatment of arteriovenous fistulas at the craniocervical junction.
        J Clin Neurosci. 2018; 53: 140-146
      2. Firsching R, Kohl J, Skalej M, Beuing O: Resolution of Brainstem Edema after Neurosurgical Occlusion of Dural Arteriovenous Fistulas of the Craniocervical Junction: Report of Three Cases and Review; 2019.

        • Sorenson T.J.
        • De Maria L.
        • Rangel-Castilla L.
        • Lanzino G.
        Surgical treatment of previously embolized craniocervical junction dural arteriovenous fistula.
        Neurosurg Focus FOC. 2019; 46: V2
        • Sun L.
        • Ren J.
        • Zhang H.
        Application of the selective indocyanine green videoangiography in microsurgical treatment of a craniocervical junction dural arteriovenous fistula.
        Neurosurg Focus FOC. 2019; 46: V5
        • Hayashi N.
        • Tomura N.
        • Okada H.
        • et al.
        Usefulness of preoperative cone beam computed tomography and intraoperative digital subtraction angiography for dural arteriovenous fistula at craniocervical junction: Technical case report.
        Surg Neurol Int. 2019; 10: 5
        • Kanemaru K.
        • Yoshioka H.
        • Hashimoto K.
        • et al.
        Efficacy of Intraarterial Fluorescence Video Angiography in Surgery for Dural and Perimedullary Arteriovenous Fistula at Craniocervical Junction.
        World Neurosurg. 2019; 126: e573-e579
        • Goto Y.
        • Hino A.
        • Shigeomi Y.
        • Oka H.
        Surgical Management for Craniocervical Junction Arteriovenous Fistula Targeting the Intradural Feeder.
        World Neurosurg. 2020; 144: e685-e692
      3. Patel K, Olijnyk LD, Tsang ACO, Pereira VM, Radovanovic I. Craniocervical Junction Vertebral Artery Dural Arteriovenous Fistula With Cranial and Spinal Venous Reflux: 2-Dimensional Operative Video; 2020.

      4. Matsubara S, Toi H, Takai H, et al. Variations and management for patients with craniocervical junction arteriovenous fistulas: Comparison of dural, radicular, and epidural arteriovenous fistulas. Surg Neurol Int 2021;12:411-411. doi:10.25259/SNI_557_2021.

        • Okamoto T.
        • Nanto M.
        • Hasegawa Y.
        • et al.
        Early rebleeding of a foramen magnum dural arteriovenous fistula: A case report and review of the literature.
        Radiol Case Rep. 2021; 16: 3499-3503
        • Yoo D.H.
        • Cho Y.D.
        • Boonchai T.
        • et al.
        Endovascular treatment of medullary bridging vein-draining dural arteriovenous fistulas: foramen magnum vs. craniocervical junction lesions.
        Neuroradiology. 2022; 64: 333
        • Lam S.K.S.
        • Chu S.L.
        • Yuen S.C.
        • Yam K.Y.
        Far Lateral Approach for Disconnection of Craniocervical Junction Dural Arteriovenous Fistula Presented with Myelopathy and Hydrocephalus.
        J Neurol Surg B Skull Base. 2021; 82: S45-S47
        • Takamatsu S.
        • Suzuki K.
        • Murakami Y.
        • Nomura K.
        • Yamamoto J.
        • Nishizawa S.
        Usefulness of arterial spin labeling in the evaluation for dural arteriovenous fistula of the craniocervical junction.
        Radiol Case Rep. 2021; 16: 1655-1659
        • Naylor R.M.
        • Topinka B.
        • Rinaldo L.
        • et al.
        Progressive Myelopathy From a Craniocervical Junction Dural Arteriovenous Fistula.
        Stroke. 2021; 52: e278-e281
        • Agnoletto G.
        • Fredrickson V.
        • Hollon T.
        • Couldwell W.
        Far Lateral Craniotomy for Obliteration of High-Risk Craniocervical Junction Arteriovenous Fistula.
        Neurol India. 2021; 69: 1554-1556
      5. Gadot R Auid- Orcid: --- Fau - Gopakumar S, Gopakumar S Auid- Orcid: --- Fau - Wagner K, Wagner K Auid- Orcid: --- Fau - Xu DS, et al.: Foramen Magnum Dural Arteriovenous Fistula Presenting With Thoracic Myelopathy: Technical Case Report With 2-Dimensional Operative Video. 2021.

        • Krishnan D.
        • Viswanathan S.
        • Rose N.
        • Benjamin H.S.N.
        • Ong A.M.
        • Hiew F.L.
        Clinical heterogeneity of low flow spinal arteriovenous fistulas; a case series.
        BMC Neurol. 2021; 21: 366