Abstract
We report the follow-up of a previously published case (Forlodou et al. Neuroradiology 38:595–597, 1996) of carotido-cavernous fistulas (CCFs) in a patient presenting with type IV Ehlers–Danlos syndrome (EDS 4) that were successfully treated twice by an endovascular approach. Initial treatment with a detachable balloon was in 1994 for a right CCF, and, 8 years later, a left CCF was treated by selective transarterial occlusion of the cavernous sinus with coils. Unfortunately, the patient suffered from a spontaneous post-operative intracranial haemorrhage in the left hemisphere and died. Review of the literature, technical considerations for bilateral CCF and complication are discussed.
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References
Pepin M, Schwarze U, Superti-Furga A, Byers PH (2000) Clinical and genetic features of Ehlers–Danlos syndrome type IV, the vascular type. N Engl J Med 342:673–680
Forlodou P, de Kersaint-Gilly A, Pizzanelli J, Viarouge MP, Auffray-Calvier E (1996) Ehlers–Danlos syndrome with a spontaneous carotidocavernous fistula occluded by detachable balloon: case report and review of the literature. Neuroradiology 38:595–597
Barabas AP (1967) Heterogeneity of the Elhers–Danlos syndrome: description of three clinical types and a hypothesis to explain the basic defect. BMJ 2:612–613
Debrun GM, Aletich VA, Miller NR, DeKeiser RJW (1996) Three cases of spontaneous direct carotid cavernous fistulas associated with Elhers–Danlos syndrome type IV. Surg Neurol 46:247–252
Halbach VV, Higashida RT, Dowd CF, Barnwell SL, Hieshima GB (1990) Treatment of carotid cavernous fistulas associated with Elhers–Danlos syndrome. Neurosurgery 26:1021–1027
Horowitz MB, Purdy PD, Valentine RJ, Morrill K (2000) Remote vascular catastrophes after neurovascular interventional therapy for type 4 Ehlers–Danlos Syndrome. AJNR Am J Neuroradiol 21:974–976
Chuman H, Trobe JD, Petty EM, Schwarze U, Pepin M, Byers PH, Deveikis JP (2002) Spontaneous direct carotid–cavernous fistula in Elhers–Danlos syndrome type IV: two case reports and a review of the literature. J Neuroophthalmol 22:75–81
Kanner AA, Maimon S, Rappaport ZH (2000) Treatment of spontaneous carotid–cavernous fistula in Elhers–Danlos syndrome by transvenous occlusion with Guglielmi detachable coils. Case report and review of the literature. J Neurosurg 93:689–692
Desal H, Leaute F, Auffray-Calvier E, Martin S, Guillon B, Robert R, de Kersaint-Gilly A (1997) Direct carotido-cavernous fistula. Clinical, radiological and therapeutic studies in 49 cases. J Neuroradiol 24:141–154
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Desal, H.A., Toulgoat, F., Raoul, S. et al. Ehlers–Danlos syndrome type IV and recurrent carotid-cavernous fistula: review of the literature, endovascular approach, technique and difficulties. Neuroradiology 47, 300–304 (2005). https://doi.org/10.1007/s00234-005-1378-4
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DOI: https://doi.org/10.1007/s00234-005-1378-4