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Eur J Cardiothorac Surg 2005;28:650-652
© 2005 Elsevier Science NL

Case report

Survival after spontaneous aortic rupture in a patient with Ehlers–Danlos syndrome

^a Department of Cardiovascular Surgery (Pr Cerene), Rangueil Hospital, 1 Av Jean Poulhès, 31403 Toulouse Cedex 4, France
^b Department of Surgery, Vienna General Hospital, Vienna, Austria
^c Department of Pathology, Rangueil Hospital, Toulouse, France

Received 23 December 2004; received in revised form 21 February 2005; accepted 24 February 2005.

^* Corresponding author. Tel.: +33 5 61 32 26 52; fax: +33 5 61 32 23 15. (Email: cdambrin{at}hotmail.com).

Ehlers–Danlos syndrome (EDS) is a rare inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and abnormalities of the cardiovascular system. Most patients are unaware of their disease until a catastrophic event such as arterial rupture or bowel perforation occurs. Aortic disruption accounts for many of the deaths in EDS type IV cases and only two cases of survival after spontaneous aortic rupture have previously been reported. We report on a third case of a survivor of spontaneous abdominal aortic rupture in EDS type IV.

Key Words: Ehlers–Danlos • Aorta • Dissection • Vascular graft