Skip to main content
SpringerLink
Log in

Endovascular embolization of cerebrospinal fluid-venous fistula: a comprehensive systematic review on its efficacy and safety for the management of spontaneous intracranial hypotension

  • Review
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Cerebrospinal fluid (CSF)-venous fistula can cause spontaneous intracranial hypotension (SIH) and poses a significant diagnostic and management challenge. This study aims to provide a comprehensive overview of the clinical and radiological outcomes of endovascular embolization as a novel treatment approach for CSF-venous fistula in patients with SIH. This systematic review adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement. The primary outcome was the efficacy of the embolization procedure in occlusion of the CSF-venous fistula, and secondary outcomes included procedural complications and improvement of clinical symptoms and radiological findings. A total of nine studies consisting of 77 patients met the inclusion criteria. Orthostatic and/or Valsalva headache was the most common symptom. The mean age of the patients was 57 ± 8.9 years, and females accounted for 59.7% (46/77) of the cases. Sixty-five (84.4%) patients reported complete resolution or significant improvement in symptoms. The Bern score, Headache Impact Test-6, and the Patient Global Impression of Change scales demonstrated significant improvements in radiological findings and patients’ quality of life. Following the procedure, 22 patients (28.6%) experienced rebound intracranial hypertension and 27 patients (35.1%) had transient local pain at the site of the embolization. Our study showed that endovascular embolization is a safe and effective treatment for CSF-venous fistula in patients with SIH, providing complete resolution or significant improvement of clinical symptoms and radiological findings, and positive impacts on patients’ quality of life.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Data availability

The data that support the findings of this study are available from the corresponding author, A.O., upon reasonable request.

References

  1. Schievink WI (2013) Stroke and death due to spontaneous intracranial hypotension. Neurocrit Care 18:248–251. https://doi.org/10.1007/s12028-012-9800-3

    Article  PubMed  Google Scholar 

  2. Schievink WI, Maya MM, Moser FG, Jean-Pierre S, Nuño M (2018) Coma: a serious complication of spontaneous intracranial hypotension. Neurology 90:e1638–e1645. https://doi.org/10.1212/WNL.0000000000005477

    Article  PubMed  Google Scholar 

  3. Mokri B (2013) Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks. Headache 53:1034–1053. https://doi.org/10.1111/head.12149

    Article  PubMed  Google Scholar 

  4. Kumar R, Cutsforth-Gregory JK, Brinjikji W (2023) Cerebrospinal fluid leaks, spontaneous intracranial hypotension, and Chiari I malformation. Neurosurg Clin N Am 34:185–192. https://doi.org/10.1016/j.nec.2022.08.012

    Article  PubMed  Google Scholar 

  5. Urbach H, Fung C, Dovi-Akue P, Lützen N, Beck J (2020) Spontaneous intracranial hypotension. Dtsch Arztebl Int 117:480–487. https://doi.org/10.3238/arztebl.2020.0480

    Article  PubMed  PubMed Central  Google Scholar 

  6. Schievink WI, Moser FG, Maya MM (2014) CSF-venous fistula in spontaneous intracranial hypotension. Neurology 83:472–473. https://doi.org/10.1212/WNL.0000000000000639

    Article  PubMed  Google Scholar 

  7. Schievink WI, Maya M, Prasad RS, Wadhwa VS, Cruz RB, Moser FG, Nuno M (2021) Spontaneous spinal cerebrospinal fluid-venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging. Headache 61:387–391. https://doi.org/10.1111/head.14048

    Article  PubMed  Google Scholar 

  8. Berroir S, Loisel B, Ducros A, Boukobza M, Tzourio C, Valade D, Bousser M-G (2004) Early epidural blood patch in spontaneous intracranial hypotension. Neurology 63:1950–1951. https://doi.org/10.1212/01.wnl.0000144339.34733.e9

    Article  PubMed  CAS  Google Scholar 

  9. Lohkamp L-N, Marathe N, Nicholson P, Farb RI, Massicotte EM (2022) Minimally invasive surgery for spinal cerebrospinal fluid-venous fistula ligation: patient series. J Neurosurg Case Lessons 3:CASE21730. https://doi.org/10.3171/CASE21730

  10. Wang TY, Karikari IO, Amrhein TJ, Gray L (2020) Kranz PG (2020) Clinical outcomes following surgical ligation of cerebrospinal fluid-venous fistula in patients with spontaneous intracranial hypotension: a prospective case series. Oper Neurosurg (Hagerstown) 18(3):239–245. https://doi.org/10.1093/ons/opz134

    Article  PubMed  Google Scholar 

  11. Mehta D, Cheema S, Davagnanam I, Matharu M (2023) Diagnosis and treatment evaluation in patients with spontaneous intracranial hypotension. Front Neurol 14:1145949. https://doi.org/10.3389/fneur.2023.1145949

    Article  PubMed  PubMed Central  Google Scholar 

  12. Orscelik A, Cutsforth-Gregory JK, Madhavan A, Senol YC, Kobeissi H, Bilgin GB, Bilgin C, Kallmes DF, Brinjikji W. Endovascular embolization techniques for cerebrospinal fluid-venous fistula in the treatment of spontaneous intracranial hypotension. Radiol Clin N Am. https://doi.org/10.1016/j.rcl.2023.10.006

  13. Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, Shamseer L, Tetzlaff JM, Akl EA, Brennan SE, Chou R, Glanville J, Grimshaw JM, Hróbjartsson A, Lalu MM, Li T, Loder EW, Mayo-Wilson E, McDonald S, McGuinness LA, Stewart LA, Thomas J, Tricco AC, Welch VA, Whiting P, Moher D (2021) The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ 372:n71. https://doi.org/10.1136/bmj.n71

    Article  PubMed  PubMed Central  Google Scholar 

  14. Sterne JA, Hernán MA, Reeves BC, Savović J, Berkman ND, Viswanathan M, Henry D, Altman DG, Ansari MT, Boutron I, Carpenter JR, Chan A-W, Churchill R, Deeks JJ, Hróbjartsson A, Kirkham J, Jüni P, Loke YK, Pigott TD, Ramsay CR, Regidor D, Rothstein HR, Sandhu L, Santaguida PL, Schünemann HJ, Shea B, Shrier I, Tugwell P, Turner L, Valentine JC, Waddington H, Waters E, Wells GA, Whiting PF, Higgins JP (2016) ROBINS-I: a tool for assessing the risk of bias in non-randomised studies of interventions. BMJ 355:i4919. https://doi.org/10.1136/bmj.i4919

    Article  PubMed  PubMed Central  Google Scholar 

  15. Brinjikji W, Savastano LE, Atkinson JLD, Garza I, Farb R, Cutsforth-Gregory JK (2021) A novel endovascular therapy for CSF hypotension secondary to CSF-venous fistulas. AJNR Am J Neuroradiol 42:882–887. https://doi.org/10.3174/ajnr.A7014

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  16. Aljuboori Z, McGrath M, Essibayi MA, Zaidi S, Hallam D (2021) Ghodke B (2021) Spontaneous spinal cerebrospinal fluid venous-fistula treated with transvenous embolization: a case report. Surg Neurol Int 12:492. https://doi.org/10.25259/SNI_878_2021

    Article  PubMed  PubMed Central  Google Scholar 

  17. Borg N, Oushy S, Savastano L, Brinjikji W (2022) Transvenous embolization of a cerebrospinal fluid-venous fistula for the treatment of spontaneous intracranial hypotension. J Neurointerv Surg 14:948. https://doi.org/10.1136/neurintsurg-2021-018160

    Article  PubMed  Google Scholar 

  18. Brinjikji W, Garza I, Whealy M, Kissoon N, Atkinson JLD, Savastano L, Madhavan A, Cutsforth-Gregory J (2022) Clinical and imaging outcomes of cerebrospinal fluid-venous fistula embolization. J Neurointerv Surg 14:953–956. https://doi.org/10.1136/neurintsurg-2021-018466

    Article  PubMed  Google Scholar 

  19. Bergui M, Mistretta F, Bosco G, Cester G, Chioffi F, Gambino A, Molinaro S, Russo R, Sorarù G, Causin F (2022) CSF-venous leak responsible for spontaneous intracranial hypotension treated by endovascular venous route: first cases in Italy. Interv Neuroradiol 15910199221116012. https://doi.org/10.1177/15910199221116011

  20. Parizadeh D, Vasconcelos AHC, Miller DA, Fermo OP, Huynh TJ (2022) Dual microcatheter and coil/balloon pressure cooker technique for transvenous embolization of cerebrospinal fluid-venous fistulas. J Neurointerv Surg neurintsurg-2022–019005. https://doi.org/10.1136/neurintsurg-2022-019005

  21. Madhavan AA, Cutsforth-Gregory JK, Oushy SH, Borg N, Brinjikji W (2022) Combined CSF-venous fistula and middle meningeal artery embolization for treatment of spontaneous intracranial hypotension. Interv Neuroradiol 15910199221109044. https://doi.org/10.1177/15910199221109044

  22. Noufal M, Liang CW, Negus J (2022) Transvenous embolization for cerebrospinal fluid-venous fistula. A case series from a single community-academic center. World Neurosurg 168:e613–e620. https://doi.org/10.1016/j.wneu.2022.10.050

    Article  PubMed  Google Scholar 

  23. Parizadeh D, Fermo O, Vibhute P, Gupta V, Arturo Larco JL, Grewal SS, Quinones-Hinojosa A, Erben YM, Clendenen S, Rozen TD, Huynh TJ (2023) Transvenous embolization of cerebrospinal fluid-venous fistulas: independent validation and feasibility of upper-extremity approach and using dual-microcatheter and balloon pressure cooker technique. J Neurointerv Surg jnis-2022–019946. https://doi.org/10.1136/jnis-2022-019946

  24. Borg N, Cutsforth-Gregory J, Oushy S, Huynh T, Savastano LE, Cloft HJ, Lanzino G, Brinjikji W (2022) Anatomy of spinal venous drainage for the neurointerventionalist: from puncture site to intervertebral foramen. AJNR Am J Neuroradiol 43:517–525. https://doi.org/10.3174/ajnr.A7409

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  25. Houk JL, Dennison JV, Kranz PG, Amrhein TJ (2022) Spontaneous intracranial hypotension: a review of pathogenesis, presentation, diagnosis, and treatment. Adv Clin Radiol 4:231–241. https://doi.org/10.1016/j.yacr.2022.04.015

    Article  Google Scholar 

  26. D’Antona L, Jaime Merchan MA, Vassiliou A, Watkins LD, Davagnanam I, Toma AK, Matharu MS (2021) Clinical presentation, investigation findings, and treatment outcomes of spontaneous intracranial hypotension syndrome: a systematic review and meta-analysis. JAMA Neurol 78:329–337. https://doi.org/10.1001/jamaneurol.2020.4799

    Article  PubMed  Google Scholar 

  27. Schievink WI, Maya M, Barnard Z, Taché RB, Prasad RS, Wadhwa VS, Moser FG, Nuño M (2022) The reversible impairment of behavioral variant frontotemporal brain sagging syndrome: challenges and opportunities. Alzheimers Dement (N Y) 8:e12367. https://doi.org/10.1002/trc2.12367

    Article  PubMed  Google Scholar 

  28. Timoney R, Bethanabatla R, Reddy M, Taha A (2023) Surgical resolution of severe headache and ataxia caused by spontaneous spinal cerebrospinal fluid leak secondary to cerebrospinal fluid venous fistula—case report. SN Compr Clin Med 5:45. https://doi.org/10.1007/s42399-022-01381-0

    Article  Google Scholar 

  29. Schievink WI, Maya MM, Moser FG, Tuchman A, Cruz RB, Farb RI, Rebello R, Reddy K, Prasad RS (2019) Spontaneous spinal CSF-venous fistulas associated with venous/venolymphatic vascular malformations: report of 3 cases. J Neurosurg Spine 32:305–310. https://doi.org/10.3171/2019.8.SPINE19716

    Article  PubMed  Google Scholar 

  30. Schievink WI (2021) Spontaneous intracranial hypotension. N Engl J Med 385:2173–2178. https://doi.org/10.1056/NEJMra2101561

    Article  PubMed  Google Scholar 

  31. Farnsworth PJ, Madhavan AA, Verdoorn JT, Shlapak DP, Johnson DR, Cutsforth-Gregory JK, Brinjikji W, Lehman VT (2023) Spontaneous intracranial hypotension: updates from diagnosis to treatment. Neuroradiology 65:233–243. https://doi.org/10.1007/s00234-022-03079-5

    Article  PubMed  Google Scholar 

  32. Kranz PG, Gray L, Malinzak MD, Houk JL, Kim DK, Amrhein TJ (2021) CSF-venous fistulas: anatomy and diagnostic imaging. AJR Am J Roentgenol 217:1418–1429. https://doi.org/10.2214/AJR.21.26182

    Article  PubMed  Google Scholar 

  33. Shlobin NA, Shah VN, Chin CT, Dillon WP, Tan LA (2021) Cerebrospinal fluid-venous fistulas: a systematic review and examination of individual patient data. Neurosurgery 88:931–941. https://doi.org/10.1093/neuros/nyaa558

    Article  PubMed  Google Scholar 

  34. Schievink WI, Maya MM, Moser FG, Prasad RS, Cruz RB, Nuño M, Farb RI (2019) Lateral decubitus digital subtraction myelography to identify spinal CSF-venous fistulas in spontaneous intracranial hypotension. J Neurosurg Spine 1–4. https://doi.org/10.3171/2019.6.SPINE19487

  35. Shlapak DP, Mark IT, Kim DK, Benson JC, Diehn FE, Kissoon NR, Liebo GB, Madhavan AA, Morris JM, Morris PP, Oien MP, Verdoorn JT, Carr CM (2023) Incremental diagnostic yield and clinical outcomes of lateral decubitus CT myelogram immediately following negative lateral decubitus digital subtraction myelogram. Neuroradiol J 19714009231173110. https://doi.org/10.1177/19714009231173110

  36. Kranz PG, Amrhein TJ, Schievink WI, Karikari IO, Gray L (2016) The “hyperdense paraspinal vein” sign: a marker of CSF-venous fistula. AJNR Am J Neuroradiol 37:1379–1381. https://doi.org/10.3174/ajnr.A4682

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  37. Shukla D, Sadashiva N, Saini J, Kamath S (2021) Spontaneous intracranial hypotension—a dilemma. Neurol India 69:S456–S462. https://doi.org/10.4103/0028-3886.332255

    Article  PubMed  Google Scholar 

  38. Mamlouk MD, Shen PY, Sedrak MF, Dillon WP (2021) CT-guided fibrin glue occlusion of cerebrospinal fluid-venous fistulas. Radiology 299:409–418. https://doi.org/10.1148/radiol.2021204231

    Article  PubMed  Google Scholar 

  39. Majeed K, Hanz SZ, Roytman M, Chazen JL, Greenfield JP (2021) Identification and surgical ligation of spinal CSF-venous fistula. Surg Neurol Int 12:514. https://doi.org/10.25259/SNI_539_2021

    Article  PubMed  PubMed Central  Google Scholar 

  40. Konovalov A, Gadzhiagaev V, Vinogradov E, Nikitin N, Eliava S, Konovalov N (2022) Surgical treatment efficacy of CSF-venous fistulas: systematic review. World Neurosurg 161:91–96. https://doi.org/10.1016/j.wneu.2022.02.036

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, Mayo Clinic, 200 First St. SW, Rochester, MN, USA

    Atakan Orscelik, Basel Musmar, Hassan Kobeissi, Gokce Belge Bilgin, Cem Bilgin, Madona Pakkam & Waleed Brinjikji

  2. Department of Neurosurgery, Division of Neuroendovascular Surgery, Medical University of South Carolina, Charleston, SC, USA

    Atakan Orscelik & Sara Zandpazandi

  3. Department of Neurosurgery, University of California, San Francisco, CA, USA

    Yigit Can Senol

Authors
  1. Atakan Orscelik
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yigit Can Senol
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Basel Musmar
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hassan Kobeissi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Gokce Belge Bilgin
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Sara Zandpazandi
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Cem Bilgin
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Madona Pakkam
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Waleed Brinjikji
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

A.O. designed the concept, collected the data, analyzed and interpreted the data, and drafted the paper. Y.C.S. contributed to the scanning process, study selection, and data extraction, and reviewed submitted version of manuscript. B.M. contributed to the scanning process, study selection, and data extraction, and reviewed submitted version of manuscript. H.K. contributed to administrative, technical, and material support, and reviewed submitted version of manuscript. G.B.B. contributed to administrative and technical support, and reviewed submitted version of manuscript. S.Z. contributed to technical support, and reviewed submitted version of manuscript. C.B. reviewed submitted version of manuscript. M.P. reviewed submitted version of manuscript. W.B. designed the concept, interpreted the data, critically revised the paper, supervised, and reviewed submitted version of manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Atakan Orscelik.

Ethics declarations

Ethics approval

This systematic review involved the analysis and synthesis of previously published data and information from electronic databases. The study did not involve direct interaction with human participants, the collection of new primary data, or the use of sensitive personal information. As a result, ethical approval was not deemed necessary for this study.

Competing interests

W.B. holds equity in Nested Knowledge, Superior Medical Editors, Piraeus Medical, Sonoris Medical, and MIVI Neurovascular. He receives royalties from Medtronic and Balloon Guide Catheter Technology. He receives consulting fees from Medtronic, Stryker, Imperative Care, MicroVention, MIVI Neurovascular, Cerenovus, Asahi, and Balt. He serves in a leadership or fiduciary role for MIVI Neurovascular, Marblehead Medical LLC, Interventional Neuroradiology (Editor-in-Chief), Piraeus Medical, and WFITN. The remaining authors have no conflicts of interest to declare.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Orscelik, A., Senol, Y.C., Musmar, B. et al. Endovascular embolization of cerebrospinal fluid-venous fistula: a comprehensive systematic review on its efficacy and safety for the management of spontaneous intracranial hypotension. Neurosurg Rev 47, 28 (2024). https://doi.org/10.1007/s10143-023-02264-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s10143-023-02264-1

Keywords

Search

Navigation