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Natural history, angiographic presentation and outcomes of anterior cranial fossa dural arteriovenous fistulas
  1. Sebastian Sanchez1,
  2. Ashrita Raghuram1,
  3. Linder Wendt2,
  4. Minako Hayakawa3,
  5. Ching-Jen Chen4,
  6. Jason P Sheehan5,
  7. Louis J Kim6,
  8. Isaac Josh Abecassis6,
  9. Michael R Levitt6,
  10. R Michael Meyer6,
  11. Ridhima Guniganti7,
  12. Akash P Kansagra8,
  13. Giuseppe Lanzino9,
  14. Enrico Giordan9,
  15. Waleed Brinjikji10,
  16. Diederik O Bulters11,
  17. Andrew Durnford11,
  18. W Christopher Fox12,
  19. Jessica Smith13,
  20. Adam J Polifka13,
  21. Bradley Gross14,
  22. Sepideh Amin-Hanjani15,
  23. Ali Alaraj15,
  24. Amanda Kwasnicki15,
  25. Robert M Starke16,
  26. Stephanie H Chen16,
  27. J Marc C van Dijk17,
  28. Adriaan R E Potgieser17,
  29. Junichiro Satomi18,
  30. Yoshiteru Tada18,
  31. Ryan Phelps19,
  32. Adib Abla19,
  33. Ethan Winkler19,
  34. Rose Du20,
  35. Pui Man Rosalind Lai20,
  36. Gregory J Zipfel7,
  37. Colin Derdeyn3,
  38. Edgar A Samaniego21
  1. 1Department of Neurology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
  2. 2Institute for Clinical and Translational Science, The University of Iowa, Iowa City, Iowa, USA
  3. 3Department of Radiology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
  4. 4Department of Neurosurgery, The University of Texas Health Science Center at Houston, Houston, Texas, USA
  5. 5Department of Neurosurgery, University of Virginia Health System, Charlottesville, Virginia, USA
  6. 6Department of Neurosurgery, University of Washington, Seattle, Washington, USA
  7. 7Department of Neurosurgery, Washington University School of Medicine in Saint Louis, St Louis, Missouri, USA
  8. 8Mallinckrodt Institute of Radiology, Washington University School of Medicine in Saint Louis, St Louis, Missouri, USA
  9. 9Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA
  10. 10Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA
  11. 11Department of Neurosurgery, University Hospital Southampton NHS Foundation Trust, Southampton, Southampton, UK
  12. 12Department of Neurosurgery, Mayo Clinic Jacksonville Campus, Jacksonville, Florida, USA
  13. 13Department of Neurosurgery, University of Florida, Gainesville, Florida, USA
  14. 14Department of Neurosurgery, University of Pittsburgh Medical Center Health System, Pittsburgh, Pennsylvania, USA
  15. 15Department of Neurosurgery, University of Illinois Chicago, Chicago, Illinois, USA
  16. 16Department of Neurosurgery, University of Miami, Coral Gables, Florida, USA
  17. 17Department of Neurosurgery, University of Groningen, Groningen, Groningen, Netherlands
  18. 18Department of Neurosurgery, Tokushima University Hospital, Tokushima, Tokushima, Japan
  19. 19Department of Neurosurgery, University of California San Francisco, San Francisco, California, USA
  20. 20Department of Neurosurgery, Brigham and Women's Hospital, Boston, Massachusetts, USA
  21. 21Departments of Neurology, Radiology and Neurosurgery, The University of Iowa, Iowa City, Iowa, USA
  1. Correspondence to Dr Edgar A Samaniego, Departments of Neurology, Radiology and Neurosurgery, The University of Iowa, Iowa City, IA 52242, USA; edgarsama{at}


Background Anterior cranial fossa dural arteriovenous fistulas (ACF-dAVFs) are aggressive vascular lesions. The pattern of venous drainage is the most important determinant of symptoms. Due to the absence of a venous sinus in the anterior cranial fossa, most ACF-dAVFs have some degree of drainage through small cortical veins. We describe the natural history, angiographic presentation and outcomes of the largest cohort of ACF-dAVFs.

Methods The CONDOR consortium includes data from 12 international centers. Patients included in the study were diagnosed with an arteriovenous fistula between 1990–2017. ACF-dAVFs were selected from a cohort of 1077 arteriovenous fistulas. The presentation, angioarchitecture and treatment outcomes of ACF-dAVF were extracted and analyzed.

Results 60 ACF-dAVFs were included in the analysis. Most ACF-dAVFs were symptomatic (38/60, 63%). The most common symptomatic presentation was intracranial hemorrhage (22/38, 57%). Most ACF-dAVFs drained through cortical veins (85%, 51/60), which in most instances drained into the superior sagittal sinus (63%, 32/51). The presence of cortical venous drainage predicted symptomatic presentation (OR 9.4, CI 1.98 to 69.1, p=0.01). Microsurgery was the most effective modality of treatment. 56% (19/34) of symptomatic patients who were treated had complete resolution of symptoms. Improvement of symptoms was not observed in untreated symptomatic ACF-dAVFs.

Conclusion Most ACF-dAVFs have a symptomatic presentation. Drainage through cortical veins is a key angiographic feature of ACF-dAVFs that accounts for their malignant course. Microsurgery is the most effective treatment. Due to the high risk of bleeding, closure of ACF-dAVFs is indicated regardless of presentation.

  • Fistula
  • Hemorrhage
  • Intervention

Data availability statement

Data are available upon reasonable request.

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Data availability statement

Data are available upon reasonable request.

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  • Contributors Study conception and design: EAS. Acquisition of data: all authors. Analysis and interpretation of data: EAS, SS, and LW. Critically revising the article: all authors. EAS is the guarantor of the study.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests LJK is a co-founder of Spi Surgical and reports compensation for consultant services from Microvention. IJA reports stock options in Remedy Robotics and compensation from Remedy Robotics and InNeuroCo for consultant services. MRL reports equity interest in Propio, Cerebrotech and Synchron, and grants from Stryker, Volcano Philips and Medtronic. MRL is part of the editorial board of JNIS. APK reports compensation from Penumbra and Microvention for consultant services. WB reports compensation for consultant services from Johnson & Johnson, Stryker, Medtronic Vascular and Microvention; compensation from MIVI Neurovascular for data and safety monitoring services; stock holdings from Marblehead Medical LLC. AJP reports compensation from DePuy Synthes Spine for consultant services. BG reports compensation from Microvention and Medtronic for consultant services. AA reports compensation from Johnson & Johnson and Cerenovous for consultant services. RMS reports consulting and teaching agreements with Penumbra, Abbott, Medtronic, InNeuroCo and Cerenovous. RD reports compensation from Grand Rounds for consultant services and compensation from NIH for other services. CD reports stock options from Euphrates Vascular; compensation from noNO and Penumbra, Inc for data and safety monitoring services. EAS is a proctor with Microvention and reports compensation from Medtronic and Rapid Medical for consultant services.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.