Abstract
We present a patient with Ehlers-Danlos syndrome type IV (EDS IV) with a carotid dissecting pseudoaneurysm causing severe carotid stenosis. This lesion was treated endovascularly. Unfortunately, the patient died of remote vascular catastrophes (intracranial hemorrhage and abdominal aortic rupture). This unique case illustrates the perils of endovascular treatment of EDS IV patients and the need for preoperative screening for concomitant lesions. It also shows that a dissecting pseudoaneurysm can feasibly be treated with a covered stent and that closure is effective using Angioseal in patients with EDS IV.
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Smith LT, Schwarze U, Goldstein J, et al. (1997) Mutations in the COL3A1 gene result in the Ehlers-Danlos syndrome type IV and alterations in the size and distribution of the major collagen fibrils of the dermis. J Invest Dermatol 108(3):241–247
Boutouyrie P, Germain DP, Fiessinger JN, et al. (2004) Increased carotid wall stress in vascular Ehlers-Danlos syndrome. Circulation 109(12):1530–1535
Oderich GS, Panneton JM, Bower TC, et al. (2005) The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience. J Vasc Surg 42(1):98–106
Beighton P, De Paepe A, Steinmann B, et al. (1998) Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). Am J Med Genet 77(1):31–37
Tseng A, Ramaiah V, Rodriguez-Lopez JA, et al. (2003) Emergent endovascular treatment of a spontaneous internal carotid artery dissection with pseudoaneurysm. J Endovasc Ther 10(3):643–646
Scavee V, De Wispelaere JF, Mormont E, et al. (2001) Pseudoaneurysm of the internal carotid artery: treatment with a covered stent. Cardiovasc Interv Radiol 24(4):283–285
Horowitz MB, Purdy PD, Valentine RJ, et al. (2000) Remote vascular catastrophes after neurovascular interventional therapy for type 4 Ehlers-Danlos syndrome. Am J Neuroradiol 21(5):974–976
Usinskiene J, Mazighi M, Bisdorff A, et al. (2006) Fatal peritoneal bleeding following embolization of a carotid-cavernous fistula in Ehlers-Danlos syndrome type IV. Cardiovasc Interv Radiol 29(6):1104–1106
Kurata A, Oka H, Ohmomo T, et al. (2003) Successful stent placement for cervical artery dissection associated with the Ehlers-Danlos syndrome. Case report and review of the literature. J Neurosurg 99(6):1077–1081
D’Souza ES, Williams DM, Deeb GM, et al. (2006) Resolution of large azygos vein aneurysm following stent-graft shunt placement in a patient with Ehlers-Danlos syndrome type IV. Cardiovasc Interv Radiol 29(5): 915–919
Freeman RK, Swegle J, Sise MJ (1996) The surgical complications of Ehlers-Danlos syndrome. Am Surg 62(10):869–873
Schievink WI, Piepgras DG, Earnest F, et al. (1991) Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome type IV. Case report. J Neurosurg 74(6):991–998
Cikrit DF, Miles JH, Silver D (1987) Spontaneous arterial perforation: the Ehlers-Danlos specter. J Vasc Surg 5(2):248–255
Guillon B, Brunerear l, Biousse V, et al. (1999) Long term follow-up of aneurysms developed during extracranial internal carotid artery dissection. Neurology 53(1):117–122
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The authors thank David Parker for his assistance in preparation of the graphic files.
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Lim, S.P., Duddy, M.J. Endovascular Treatment of a Carotid Dissecting Pseudoaneurysm in a Patient with Ehlers-Danlos Syndrome Type IV with Fatal Outcome. Cardiovasc Intervent Radiol 31, 201–204 (2008). https://doi.org/10.1007/s00270-007-9135-1
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DOI: https://doi.org/10.1007/s00270-007-9135-1