RSS-Feed abonnieren
DOI: 10.1055/s-0032-1322595
Endovascular Treatment of Bilateral Multiple Carotid-Cavernous Fistulas in a Patient with Ehlers-Danlos Syndrome
Publikationsverlauf
15. Juli 2011
02. März 2012
Publikationsdatum:
30. Juli 2012 (online)
![](https://www.thieme-connect.de/media/jnlsa/2013S01/lookinside/thumbnails/10.1055-s-0032-1322595-1.jpg)
Abstract
Introduction In this article, we report the first patient with Ehlers-Danlos syndrome type IV (EDS IV) who developed multiple spontaneous contralateral direct carotid-cavernous fistulas (CCFs) after permanent occlusion of left internal carotid artery CCF 2 years before. We occluded the multiple fistulas and preserved the internal carotid artery (ICA) by using transarterial balloon-assisted embolization with coils and Onyx.
Case Report A 39-year-old woman presented with severe sudden-onset periorbital pain, increasing swelling, and proptosis of her left eye. Digital subtraction angiography (DSA) demonstrated direct left CCF. This patient underwent successful occlusion of left ICA and CCF by using detachable balloons. Two years later, she complained of a pulsatile intracranial bruit and then swelling and proptosis of her right eye.
Intervention The right carotid artery angiogram revealed direct high-flow CCF with two fistulas in the cavernous segment of right ICA. Taking a transarterial approach, we occluded the two fistulas with GDC coils and Onyx-18 by an assisted balloon.
Conclusion In the management of multiple CCFs in EDS IV patients, we are able to occlude the fistulas and leave the ICA intact by using transarterial balloon-assisted embolization. Intensive follow-up of treated arteries is mandatory, because EDS IV is widespread and can occur on contralateral vessels as well.
-
References
- 1 Horowitz MBPP, Purdy PD, Valentine RJ, Morrill K. Remote vascular catastrophes after neurovascular interventional therapy for type 4 Ehlers-Danlos syndrome. AJNR Am J Neuroradiol 2000; 21 (5) 974-976
- 2 Forlodou P, de Kersaint-Gilly A, Pizzanelli J, Viarouge MP, Auffray-Calvier E. Ehlers-Danlos syndrome with a spontaneous caroticocavernous fistula occluded by detachable balloon: case report and review of literature. Neuroradiology 1996; 38 (6) 595-597
- 3 Pepin MSU, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 2000; 342 (10) 673-680
- 4 Germain DPH-GY, Herrera-Guzman Y. Vascular Ehlers-Danlos syndrome. Ann Genet 2004; 47 (1) 1-9
- 5 Kanner AAMS, Maimon S, Rappaport ZH. Treatment of spontaneous carotid-cavernous fistula in Ehlers-Danlos syndrome by transvenous occlusion with Guglielmi detachable coils. Case report and review of the literature. J Neurosurg 2000; 93 (4) 689-692
- 6 Halbach VVHR, Higashida RT, Hieshima GB, Hardin CW, Yang PJ. Transvenous embolization of direct carotid cavernous fistulas. AJNR Am J Neuroradiol 1988; 9 (4) 741-747
- 7 Mitsuhashi TMM, Miyajima M, Saitoh R, Nakao Y, Hishii M, Arai H. Spontaneous carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV—case report. Neurol Med Chir (Tokyo) 2004; 44 (10) 548-553
- 8 Hollands JKST, Santarius T, Kirkpatrick PJ, Higgins JN. Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome: a novel approach. Neuroradiology 2006; 48 (7) 491-494
- 9 Bing F, Albrieux M, Vinh Moreau-Gaudry V, Vasdev A. Cavernous sinus fistula treated through the transvenous approach: report of four cases. J Neuroradiol 2009; 36 (5) 265-269
- 10 Burrows NP. The molecular genetics of the Ehlers-Danlos syndrome. Clin Exp Dermatol 1999; 24 (2) 99-106
- 11 Forlodou P, de Kersaint-Gilly A, Pizzanelli J, Viarouge MP, Auffray-Calvier E. Ehlers-Danlos syndrome with a spontaneous caroticocavernous fistula occluded by detachable balloon: case report and review of literature. Neuroradiology 1996; 38 (6) 595-597
- 12 Kwon BJ, Han MH, Kang HS, Chang KH. Endovascular occlusion of direct carotid cavernous fistula with detachable balloons: usefulness of 3D angiography. Neuroradiology 2005; 47 (4) 271-281
- 13 Baccin CE, Campos CM, Abicalaf R , et al. Traumatic carotid-cavernous fistula: endovascular treatment with onyx and coils. Interv Neuroradiol 2005; 11 (4) 363-367
- 14 Arat A, Cekirge S, Saatci I, Ozgen B. Transvenous injection of Onyx for casting of the cavernous sinus for the treatment of a carotid-cavernous fistula. Neuroradiology 2004; 46 (12) 1012-1015
- 15 Zenteno M, Santos-Franco J, Rodríguez-Parra V , et al. Management of direct carotid-cavernous sinus fistulas with the use of ethylene-vinyl alcohol (Onyx) only: preliminary results. J Neurosurg 2010; 112 (3) 595-602