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Carotid Cavernous Fistulas And Vein of Galen Malformations

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Introduction to Vascular Neurosurgery

Abstract

Intracranial arteriovenous fistulae involve anomalous connections between cerebral arterial and venous vessels. In this chapter, the authors discuss two of the three forms of fistulae: carotid-cavernous fistulae and vein of Galen malformations. Dural arteriovenous fistula (dAVFs) will be discussed in a separate chapter. For each lesion subtype, the classification, clinical manifestations, imaging, treatment, and prognosis are reviewed.

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Abbreviations

AVM:

Arteriovenous malformation

CCF:

Carotid-cavernous fistulae

CT:

Computed tomography

CTA:

Computed tomography angiogram

ECA:

External carotid artery

EVD:

External ventricular drain

ICA:

Internal carotid artery

MRI:

Magnetic resonance imaging

MRA:

Magnetic resonance angiogram

nBCA:

n-butyl-cyanoacrylate

VOGM:

Vein of Galen malformations

References

  1. Liang W, Xiaofeng Y, Weiguo L, Wusi Q, Gang S, Xuesheng Z. Traumatic carotid cavernous fistula accompanying basilar skull fracture: a study on the incidence of traumatic carotid cavernous fistula in the patients with basilar skull fracture and the prognostic analysis about traumatic carotid cavernous fistula. J Trauma. 2007;63(5):1014–20. – discussion 1020.

    PubMed  Google Scholar 

  2. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg. 1985;62(2):248–56.

    Article  CAS  PubMed  Google Scholar 

  3. Debrun GM, Viñuela F, Fox AJ, Davis KR, Ahn HS. Indications for treatment and classification of 132 carotid-cavernous fistulas. Neurosurgery. 1988;22(2):285–9.

    Article  CAS  PubMed  Google Scholar 

  4. Ellis JA, Goldstein H, Connolly ES, Meyers PM. Carotid-cavernous fistulas. Neurosurg Focus. 2012;32(5):E9.

    Article  PubMed  Google Scholar 

  5. Miller NR. Dural carotid-cavernous fistulas: epidemiology, clinical presentation, and management. Neurosurg Clin N Am. 2012;23(1):179–92.

    Article  PubMed  Google Scholar 

  6. Thomas AJ, Chua M, Fusco M, Ogilvy CS, Tubbs RS, Harrigan MR, et al. Proposal of venous drainage-based classification system for carotid cavernous fistulae with validity assessment in a multicenter cohort. Neurosurgery. 2015;77(3):380–5. –discussion385.

    Article  PubMed  Google Scholar 

  7. Leone G, Renieri L, Enriquez-Marulanda A, Dmytriw AA, Nappini S, Laiso A, et al. Carotid cavernous fistulas and dural arteriovenous fistulas of the cavernous sinus: validation of a new classification according to venous drainage. World Neurosurg. 2019;128:e621–31.

    Article  PubMed  Google Scholar 

  8. Gupta AK, Purkayastha S, Krishnamoorthy T, Bodhey NK, Kapilamoorthy TR, Kesavadas C, et al. Endovascular treatment of direct carotid cavernous fistulae: a pictorial review. Neuroradiology. 2006;48(11):831–9.

    Article  PubMed  Google Scholar 

  9. van Rooij WJ, Sluzewski M, Beute GN. Ruptured cavernous sinus aneurysms causing carotid cavernous fistula: incidence, clinical presentation, treatment, and outcome. Am J Neuroradiol. 2006;27(1):185–9.

    PubMed  PubMed Central  Google Scholar 

  10. Feiner L, Bennett J, Volpe NJ. Cavernous sinus fistulas: carotid cavernous fistulas and dural arteriovenous malformations. Curr Neurol Neurosci Rep. 2003;3(5):415–20.

    Article  PubMed  Google Scholar 

  11. Oishi A, Miyamoto K, Yoshimura N. Etiology of carotid cavernous fistula in Japanese. Jpn J Ophthalmol. 2009;53(1):40–3.

    Article  PubMed  Google Scholar 

  12. Qi Y, Liu H, Jia J, Li J, Liu A, Jiang C. Endovascular treatment for spontaneous carotid cavernous sinus fistula caused by fibromuscular dysplasia: a rare case report and literature review. Clin Neurol Neurosurg. 2020;195:105882.

    Article  PubMed  Google Scholar 

  13. Adham S, Trystram D, Albuisson J, Domigo V, Legrand A, Jeunemaitre X, et al. Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review. Orphanet J Rare Dis. 2018;13(1):100–10.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Robert T, Sylvestre P, Blanc R, Botta D, Ciccio G, Smajda S, et al. Thrombosis of venous outflows of the cavernous sinus: possible aetiology of the cortical venous reflux in case of indirect carotid-cavernous fistulas. Acta Neurochir (Wien). 2017;159(5):835–43.

    Article  Google Scholar 

  15. de Keizer R. Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit. 2003;22(2):121–42.

    Article  PubMed  Google Scholar 

  16. Grumann AJ, Boivin-Faure L, Chapot R, Adenis JP, Robert PY. Ophthalmologic outcome of direct and indirect carotid cavernous fistulas. Int Ophthalmol. 2012;32(2):153–9.

    Article  PubMed  Google Scholar 

  17. Sasaki H, Nukui H, Kaneko M, Mitsuka S, Hosaka T, Kakizawa T, et al. Long-term observations in cases with spontaneous carotid-cavernous fistulas. Acta Neurochir (Wien). 1988;90(3–4):117–20.

    Article  CAS  Google Scholar 

  18. Ko JK, Lee SW, Lee TH, Choi CH. Traumatic carotid cavernous fistula with a connection between the supraclinoid internal carotid artery and cavernous sinus via a pseudoaneurysm presenting with delayed life-threatening epistaxis. NMC Case Rep J. 2017;4(2):43–6.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Morton RP, Tariq F, Levitt MR, Nerva JD, Mossa-Basha M, Sekhar LN, et al. Radiographic and clinical outcomes in cavernous carotid fistula with special focus on alternative transvenous access techniques. J Clin Neurosci. 2015;22(5):859–64.

    Article  PubMed  Google Scholar 

  20. Kalsi P, Padmanabhan R, Prasad KSM, Mukerji N. Treatment of low flow, indirect cavernous sinus dural arteriovenous fistulas with external manual carotid compression – the UK experience. Br J Neurosurg. 2020;34(6):701–3.

    Article  PubMed  Google Scholar 

  21. Kai Y, Hamada J-I, Morioka M, Yano S, Kuratsu J-I. Treatment of cavernous sinus dural arteriovenous fistulae by external manual carotid compression. Neurosurgery. 2007;60(2):253–7. –discussion257–8.

    Article  PubMed  Google Scholar 

  22. Higashida RT, Hieshima GB, Halbach VV, Bentson JR, Goto K. Closure of carotid cavernous sinus fistulae by external compression of the carotid artery and jugular vein. Acta Radiol Suppl. 1986;369:580–3.

    CAS  PubMed  Google Scholar 

  23. Rodrigues T, Willinsky R, Agid R, terBrugge K, Krings T. Management of dural carotid cavernous fistulas: a single-centre experience. Eur Radiol. 2014;24(12):3051–8.

    Article  PubMed  Google Scholar 

  24. Dye J, Duckwiler G, Gonzalez N, Kaneko N, Goldberg R, Rootman D, et al. Endovascular approaches to the cavernous sinus in the setting of dural arteriovenous fistula. Brain Sci. 2020;10(8):554.

    Article  PubMed Central  Google Scholar 

  25. de Castro-Afonso LH, Trivelato FP, Rezende MT, Ulhôa AC, Nakiri GS, Monsignore LM, et al. The routes for embolization of dural carotid cavernous fistulas when the endovascular approach is indicated as a first-line strategy. Interv Neuroradiol. 2019;25(1):66–70.

    Article  PubMed  Google Scholar 

  26. Ducruet AF, Albuquerque FC, Crowley RW, McDougall CG. The evolution of endovascular treatment of carotid cavernous fistulas: a single-center experience. World Neurosurg. 2013;80(5):538–48.

    Article  PubMed  Google Scholar 

  27. Meyers PM, Halbach VV, Dowd CF, Lempert TE, Malek AM, Phatouros CC, et al. Dural carotid cavernous fistula: definitive endovascular management and long-term follow-up. Am J Ophthalmol. 2002;134(1):85–92.

    Article  PubMed  Google Scholar 

  28. Hassan T, Rashad S, Aziz W, Sultan A, Ibrahim T. Endovascular modalities for the treatment of cavernous sinus arteriovenous fistulas: a single-center experience. J Stroke Cerebrovasc Dis. 2015;24(12):2824–38.

    Article  PubMed  Google Scholar 

  29. Sumdani H, Aguilar-Salinas P, Avila MJ, El-Ghanem M, Dumont TM. Carotid cavernous fistula treatment via flow diversion: a systematic review of the literature. World Neurosurg. 2021;149:e369.

    Article  PubMed  Google Scholar 

  30. Goelz L, Kreißl L, Gutowski P, Sparenberg P, Kirsch M, Mutze S. Unique indications for covered stent grafts in neuroradiology departments of level 1 trauma centers. J Neurol Surg A Cent Eur Neurosurg. 2020;81(3):243–52.

    Article  PubMed  Google Scholar 

  31. Lin L-M, Colby GP, Jiang B, Pero G, Boccardi E, Coon AL. Transvenous approach for the treatment of direct carotid cavernous fistula following Pipeline embolization of cavernous carotid aneurysm: a report of two cases and review of the literature. BMJ Case Rep. 2014;2014:bcr2014011235.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Dashti SR, Fiorella D, Spetzler RF, Albuquerque FC, McDougall CG. Transorbital endovascular embolization of dural carotid-cavernous fistula: access to cavernous sinus through direct puncture: case examples and technical report. Neurosurgery. 2011;68(1 Suppl Operative):75–83. –discussion83.

    PubMed  Google Scholar 

  33. Tang C-L, Liao C-H, Chen W-H, Shen S-C, Lee C-H, Lee H-T, et al. Endoscope-assisted transsphenoidal puncture of the cavernous sinus for embolization of carotid-cavernous fistula in a neurosurgical hybrid operating suite. J Neurosurg. 2017;127(2):327–31.

    Article  PubMed  Google Scholar 

  34. Sur S, Menaker SA, Alvarez C, Chen S, Shah SS, Peterson EC, et al. Multimodal Management of Carotid-Cavernous Fistulas. World Neurosurg. 2020;133:e796–803.

    Article  PubMed  Google Scholar 

  35. Dye JA, Buchanan CC, Gonzalez NR. Integrated open surgical and endovascular embolization treatment of a paracavernous venous plexus fistula: case report. J Neurosurg. 2015;122(4):933–8.

    Article  PubMed  Google Scholar 

  36. Muram S, Khader Eliyas J, Eesa M, Mitha AP. Combined surgical and endovascular approach to treat a carotid cavernous fistula with associated brainstem venous congestion. BMJ Case Rep. 2017;2017:bcr–2017–013248.

    Article  PubMed  Google Scholar 

  37. Starke RM, McCarthy D, Sheinberg D, Connolly ES. Genetic drivers of vein of Galen malformations. Neurosurgery. 2019;85(2):E205–6.

    Article  PubMed  Google Scholar 

  38. Briggs RG, Bonney PA, Algan O, Patel AD, Sughrue ME. Bilateral carotid-cavernous fistulas treated with partial embolization and radiosurgery. Cureus. 2019;11(10):e5886.

    PubMed  PubMed Central  Google Scholar 

  39. Wu C-A, Yang H-C, Hu Y-S, Wu H-M, Lin C-J, Luo C-B, et al. Venous outflow restriction as a predictor of cavernous sinus dural arteriovenous fistula obliteration after gamma knife surgery. J Neurosurg. 2019;132(1):1–8.

    Google Scholar 

  40. Park S-H, Park K-S, Kang D-H, Hwang J-H, Hwang S-K. Stereotactic radiosurgery for dural carotid cavernous sinus fistulas. World Neurosurg. 2017;106:836–43.

    Article  PubMed  Google Scholar 

  41. Pan H-C, Sun M-H, Sheehan J, Sheu M-L, Chen CC-C, Lee H-T, et al. Radiosurgery for dural carotid-cavernous sinus fistulas: Gamma Knife compared with XKnife radiosurgery. J Neurosurg. 2010;113 Suppl(Special_Supplement):9–20.

    Google Scholar 

  42. Morshed RA, Winkler EA, Kim H, Braunstein S, Cooke DL, Hetts SW, et al. High-flow vascular malformations in children. Semin Neurol. 2020;40(3):303–14.

    Article  PubMed  Google Scholar 

  43. Bhattacharya JJ, Thammaroj J. Vein of galen malformations. J Neurol Neurosurg Psychiatr. 2003;74 Suppl 1(90001):i42–4.

    Google Scholar 

  44. Recinos PF, Rahmathulla G, Pearl M, Recinos VR, Jallo GI, Gailloud P, et al. Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 2012;23(1):165–77.

    Article  PubMed  Google Scholar 

  45. Long DM, Seljeskog EL, Chou SN, French LA. Giant arteriovenous malformations of infancy and childhood. J Neurosurg. 1974;40(3):304–12.

    Article  CAS  PubMed  Google Scholar 

  46. Raybaud C. Normal and abnormal embryology and development of the intracranial vascular system. Neurosurg Clin N Am. 2010;21(3):399–426.

    Article  PubMed  Google Scholar 

  47. Bhatia K, Mendes-Pereira V, Krings T, Brugge Ter K, Kortman H, Dirks P, et al. Factors contributing to major neurological complications from vein of Galen malformation embolization. JAMA Neurol. 2020;77(8):992–9.

    Article  PubMed  Google Scholar 

  48. Raybaud CA, Strother CM, Hald JK. Aneurysms of the vein of Galen: embryonic considerations and anatomical features relating to the pathogenesis of the malformation. Neuroradiology. 2nd ed. Springer-Verlag. 1989;31(2):109–28.

    CAS  Google Scholar 

  49. Duran D, Zeng X, Jin SC, Choi J, Nelson-Williams C, Yatsula B, et al. Mutations in chromatin modifier and ephrin signaling genes in vein of Galen malformation. Neuron. 2019;101(3):429–443.e4.

    Article  CAS  PubMed  Google Scholar 

  50. Chida A, Shintani M, Wakamatsu H, Tsutsumi Y, Iizuka Y, Kawaguchi N, et al. ACVRL1 gene variant in a patient with vein of Galen aneurysmal malformation. J Pediatr Genet. 2013;2(4):181–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  51. Tsutsumi Y, Kosaki R, Itoh Y, Tsukamoto K, Matsuoka R, Shintani M, et al. Vein of Galen aneurysmal malformation associated with an endoglin gene mutation. Pediatrics. 2011;128(5):e1307–10.

    Article  PubMed  Google Scholar 

  52. Mortazavi MM, Griessenauer CJ, Foreman P, Bavarsad Shahripour R, Shoja MM, Rozzelle CJ, et al. Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr. 2nd ed. American Association of Neurological Surgeons. 2013;12(3):293–306.

    Google Scholar 

  53. Yaşargil MG, Antic J, Laciga R, Jain KK, Boone SC. Arteriovenous malformations of vein of Galen: microsurgical treatment. Surg Neurol. 1976;3:195–200.

    Google Scholar 

  54. Lasjaunias P, Rodesch G, Pruvost P, Laroche FG, Landrieu P. Treatment of vein of Galen aneurysmal malformation. J Neurosurg. 1989;70(5):746–50.

    Article  CAS  PubMed  Google Scholar 

  55. Geibprasert S, Krings T, Armstrong D, TerBrugge KG, Raybaud CA. Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations. Childs Nerv Syst. 2010;26(1):35–46.

    Article  PubMed  Google Scholar 

  56. Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery. 2006;59(5 Suppl 3):S184–94. –discussionS3–13.

    PubMed  Google Scholar 

  57. Hoang S, Choudhri O, Edwards M, Guzman R. Vein of Galen malformation. Neurosurg Focus. 2009;27(5):E8.

    Article  PubMed  Google Scholar 

  58. Yan J, Gopaul R, Wen J, Li X-S, Tang J-F. The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis. J Neurointerv Surg. 2017;9(6):564–70.

    Article  PubMed  Google Scholar 

  59. Chow ML, Cooke DL, Fullerton HJ, Amans MR, Narvid J, Dowd CF, et al. Radiological and clinical features of vein of Galen malformations. J Neurointerv Surg. 2015;7(6):443–8.

    Article  PubMed  Google Scholar 

  60. Paúl L, Casasco A, Gil A, Aleu A, Guimaraens L. Arteriovenous fistulas of the vein of Galen region in adults: endovascular treatment. Interv Neuroradiol. 2020;26(6):757–66.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Brinjikji W, Krings T, Murad MH, Rouchaud A, Meila D. Endovascular treatment of vein of galen malformations: a systematic review and meta-analysis. AJNR Am J Neuroradiol. 2017;38(12):2308–14.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Fullerton HJ, Aminoff AR, Ferriero DM, Gupta N, Dowd CF. Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations. Neurology. Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology; 2003;61(10):1386–90.

    Google Scholar 

  63. Payne BR, Prasad D, Steiner M, Bunge H, Steiner L. Gamma surgery for vein of Galen malformations. J Neurosurg. 2000;93(2):229–36.

    Article  CAS  PubMed  Google Scholar 

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Srienc, A.I., Huguenard, A.L., Gupta, V.P., Osbun, J.W. (2022). Carotid Cavernous Fistulas And Vein of Galen Malformations. In: Mascitelli, J.R., Binning, M.J. (eds) Introduction to Vascular Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-030-88196-2_22

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