Survival after spontaneous aortic rupture in a patient with Ehlers-Danlos syndrome.
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.
Dambrin
Camille
C
Department of Cardiovascular Surgery (Pr Cerene), Rangueil Hospital, 1 Av Jean Poulhès, 31403 Toulouse Cedex 4, France. cdambrin@hotmail.com
Marcheix
Bertrand
B
Bîrsan
Tudor
T
Delisle
Marie Bernadette
MB
D000328:Adult; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging / Q000473:pathology; D001019:Aortic Rupture / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008297:Male; D008854:Microscopy, Electron / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods