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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery
DOI: 10.1055/s-0044-1788603
Case Report

Spinal Dural Arteriovenous Fistula, a Rare and Frequently Misdiagnosed Cause of Mielopathy – Case Report and Review of the Literature[*]

Fístula arteriovenosa dural espinhal, uma causa de mielopatia rara e frequentemente não diagnosticada – Relato de caso e revisão da literatura
Thiago Magalhães de Souza 
1   Division of Neurosurgery, Department of Neurology, Universidade Estadual de Campinas, Campinas, SP, Brazil
,
Paulo Gabriel Sacramento da Silva 
1   Division of Neurosurgery, Department of Neurology, Universidade Estadual de Campinas, Campinas, SP, Brazil
,
Fellipe de Paula 
1   Division of Neurosurgery, Department of Neurology, Universidade Estadual de Campinas, Campinas, SP, Brazil
,
Enrico Ghizoni 
1   Division of Neurosurgery, Department of Neurology, Universidade Estadual de Campinas, Campinas, SP, Brazil
,
Andrei Fernandes Joaquim 
1   Division of Neurosurgery, Department of Neurology, Universidade Estadual de Campinas, Campinas, SP, Brazil
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Abstract

Spinal dural arteriovenous fistulas (SDAVFs) are rare vascular malformations with unspecific clinical manifestations, which often lead to misdiagnosis and delay in the establishment of effective therapies. Since the neurological prognosis depends on symptom duration and pretreatment neurological status, rapid identification and obliteration of the fistula are key to provide patients with a better quality of life. We herein describe an illustrative case of an elderly patient presenting with progressive myelopathy, with a definitive diagnosis of SDAVF after 18 months of the initial symptoms. We also review the core concepts regarding this condition and discuss strategies to prevent misdiagnosis and worsening of the neurological outcome.

Resumo

Fístulas arteriovenosas durais espinhais (FADEs) são malformações vasculares raras e com manifestações clínicas pouco específicas, o que frequentemente leva a erros diagnósticos e ao atraso no estabelecimento de terapias efetivas. Considerando que o prognóstico neurológico depende da duração dos sintomas e do status neurológico prévio, a agilidade na identificação e obliteração da fístula são essenciais para prover melhor qualidade de vida aos pacientes. Neste estudo, descrevemos um caso ilustrativo de um paciente idoso com quadro de mielopatia progressiva, com diagnóstico definitivo de FADE após 18 meses do início dos sintomas. Realizamos ainda uma revisão sobre os conceitos principais da doença e discutimos estratégias para prevenir atrasos diagnósticos e piora neurológica.

Keywords

arteriovenous fistulas - dural - myelopathy - spinal cord disease

Palavras-chave

fístulas arteriovenosas - dural - mielopatia - doença da medula espinal

* Study conducted at the Teaching Hospital of Universidade Estadual de Campinas, Campinas, SP, Brazil.




Publication History

Received: 01 August 2023

Accepted: 16 May 2024

Article published online:
23 July 2024

© 2024. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Day AL, Turkmani AH, Chen PR. Spinal arteriovenous fistulae: surgical management [Internet]. 1st ed.. Vol. 143. Handbook of Clinical Neurology. Elsevier B.V.; 2017: 189-198 . Available from: http://dx.doi.org/10.1016/B978-0-444-63640-9.00018-7
    CrossrefGoogle Scholar
  • 2 Maimon S, Luckman Y, Strauss I. Spinal Dural Arteriovenous Fistula: A Review. Adv Tech Stand Neurosurg 2016; 43 (43) 111-137
    CrossrefPubMedGoogle Scholar
  • 3 McCutcheon IE, Doppman JL, Oldfield EH. Microvascular anatomy of dural arteriovenous abnormalities of the spine: a microangiographic study. J Neurosurg 1996; 84 (02) 215-220
    CrossrefPubMedGoogle Scholar
  • 4 Donghai W, Ning Y, Peng Z. et al. The diagnosis of spinal dural arteriovenous fistulas. Spine 2013; 38 (09) E546-E553
    CrossrefPubMedGoogle Scholar
  • 5 Ehresman J, Catapano JS, Baranoski JF, Jadhav AP, Ducruet AF, Albuquerque FC. Treatment of Spinal Arteriovenous Malformation and Fistula. Neurosurg Clin N Am 2022; 33 (02) 193-206 DOI: 10.1016/j.nec.2021.11.005. [Internet]
    CrossrefPubMedGoogle Scholar
  • 6 Takai K. Spinal arteriovenous shunts: Angioarchitecture and historical changes in classification. Neurol Med Chir (Tokyo) 2017; 57 (07) 356-365
    CrossrefPubMedGoogle Scholar
  • 7 Di Chiro G. Angiography of obstructive vascular disease of the spinal cord. Radiology 1971; 100 (03) 607-614
    CrossrefPubMedGoogle Scholar
  • 8 Rosenblum B, Oldfield EH, Doppman JL, Di Chiro G. Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM's in 81 patients. J Neurosurg 1987; 67 (06) 795-802
    CrossrefPubMedGoogle Scholar
  • 9 Spetzler RF, Detwiler PW, Riina HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg 2002; 96 (2, Suppl) 145-156
    PubMedGoogle Scholar
  • 10 Rodesch G, Hurth M, Alvarez H. et al. Classification of spinal cord arteriovenous shunts: proposal for a reappraisal—the Bicêtre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery 2002; 51 (02) 374-379
    PubMedGoogle Scholar
  • 11 Kona MP, Buch K, Singh J, Rohatgi S. Spinal vascular shunts: A patterned approach. AJNR Am J Neuroradiol 2021; 42 (12) 2110-2118
    CrossrefPubMedGoogle Scholar
  • 12 Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder. Brain 2006; 129 (Pt 12): 3150-3164
    CrossrefPubMedGoogle Scholar
  • 13 Ronald AA, Yao B, Winkelman RD, Piraino D, Masaryk TJ, Krishnaney AA. Spinal Dural Arteriovenous Fistula: Diagnosis, Outcomes, and Prognostic Factors. World Neurosurg 2020; 144: e306-e315 DOI: 10.1016/j.wneu.2020.08.126. [Internet]
    CrossrefPubMedGoogle Scholar
  • 14 Muralidharan R, Saladino A, Lanzino G, Atkinson JL, Rabinstein AA. The clinical and radiological presentation of spinal dural arteriovenous fistula. Spine 2011; 36 (25) E1641-E1647
    CrossrefPubMedGoogle Scholar
  • 15 Takai K, Endo T, Yasuhara T. et al. Neurosurgical versus endovascular treatment of spinal dural arteriovenous fistulas: a multicenter study of 195 patients. J Neurosurg Spine 2020; 34 (03) 514-521
    CrossrefPubMedGoogle Scholar
  • 16 Saladino A, Atkinson JLD, Rabinstein AA. et al. Surgical treatment of spinal dural arteriovenous fistulae: a consecutive series of 154 patients. Neurosurgery 2010; 67 (05) 1350-1357 , discussion 1357–1358
    CrossrefPubMedGoogle Scholar
  • 17 Yang B, Lu T, He X, Li H. Spinal dural arteriovenous fistula: A rare but treatable disease that should not be missed by orthopedic surgeons. Front Neurol 2022; 13: 938342
    CrossrefPubMedGoogle Scholar
  • 18 Fox S, Hnenny L, Ahmed U, Meguro K, Kelly ME. Spinal dural arteriovenous fistula: a case series and review of imaging findings. Spinal Cord Ser Cases 2017; 3 (01) 17024
    CrossrefPubMedGoogle Scholar
  • 19 Gemmete JJ, Chaudhary N, Elias AE. et al. Spinal dural arteriovenous fistulas: clinical experience with endovascular treatment as a primary therapy at 2 academic referral centers. AJNR Am J Neuroradiol 2013; 34 (10) 1974-1979
    CrossrefPubMedGoogle Scholar
  • 20 Dowd EC, Frank MJ, Collins A, Goldd JM, Barch DM. Kuschner. HHS Public Access. Physiol Behav 2017; 176 (03) 139-148 . Available from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3856065/pdf/ijms-14-22274.pdf%0Ahttp://www.mdpi.com/1422-0067/14/11/22274 [Internet]
    PubMedGoogle Scholar
  • 21 Jiang J, Trundle P, Ren J. et al. We are IntechOpen, the world ' s leading publisher of Open Access books Built by scientists, for scientists TOP 1%. Intech [Internet] 2010; 34 (08) 57-67 DOI: 10.1007/s12559-021-09926-6%0A. . https://www.intechopen.com/books/advanced-biometric-technologies/liveness-detection-in-biometrics%0Ahttp://dx.doi.org/10.1016/j.compmedimag.2010.07.003
    CrossrefPubMedGoogle Scholar
  • 22 Condette-Auliac S, Boulin A, Roccatagliata L. et al. MRI and MRA of spinal cord arteriovenous shunts. J Magn Reson Imaging 2014; 40 (06) 1253-1266
    CrossrefPubMedGoogle Scholar
  • 23 Durnford AJ, Hempenstall J, Sadek AR. et al. Degree and Duration of Functional Improvement on Long-Term Follow-Up of Spinal Dural Arteriovenous Fistulae Occluded by Endovascular and Surgical Treatment. World Neurosurg 2017; 107: 488-494 DOI: 10.1016/j.wneu.2017.07.140. [Internet]
    CrossrefPubMedGoogle Scholar
  • 24 Bretonnier M, Hénaux PL, Gaberel T. et al. Spinal Dural Arteriovenous Fistulas: Clinical Outcome After Surgery Versus Embolization: A Retrospective Study. World Neurosurg 2019; 127: e943-e949
    CrossrefPubMedGoogle Scholar
  • 25 Goyal A, Cesare J, Lu VM. et al. Outcomes following surgical versus endovascular treatment of spinal dural arteriovenous fistula: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry 2019; 90 (10) 1139-1146
    CrossrefPubMedGoogle Scholar
  • 26 Steinmetz MP, Chow MM, Krishnaney AA. et al. Outcome after the treatment of spinal dural arteriovenous fistulae: a contemporary single-institution series and meta-analysis. Neurosurgery 2004; 55 (01) 77-87 , discussion 87–88
    CrossrefPubMedGoogle Scholar