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 perforatio...

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Bibliographic Details
Published in:European journal of cardio-thoracic surgery 2005-10, Vol.28 (4), p.650-652
Main Authors: Dambrin, Camille, Marcheix, Bertrand, Bîrsan, Tudor, Delisle, Marie Bernadette
Format: Article
Language:English
Subjects:
Adult
Aorta
Aorta, Abdominal - diagnostic imaging
Aorta, Abdominal - pathology
Aortic Rupture - diagnostic imaging
Aortic Rupture - etiology
Aortic Rupture - pathology
Biological and medical sciences
Cardiology. Vascular system
Dissection
Ehlers-Danlos Syndrome - complications
Ehlers-Danlos Syndrome - diagnostic imaging
Ehlers-Danlos Syndrome - pathology
Ehlers–Danlos
Humans
Male
Medical sciences
Microscopy, Electron - methods
Pneumology
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the heart
Tomography, X-Ray Computed - methods
Vascular graft
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Description
Summary: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.
ISSN:1010-7940
1873-734X
DOI:10.1016/j.ejcts.2005.02.050