Korean J Thorac Cardiovasc Surg.  2000 Nov;33(11):910-914.

Surgical Treatment of Acute Type A Aortic Dissection in Ehlers-Danlos Syndrome

Affiliations
  • 1Department of Thoracic and Cardiovascular Surgery, Seoul National University College of Medicine, Seoul, Korea.

Abstract

Ehlers-Danlos syndrome type IV(the arterial-ecchymotic type) which has skin fragility, easy bruisability, and j oint hyperextensibility is occasionally combined with large vessel involve-ment and spontaneous catastrophic bleeding. As even a small inj ury can cause profound vascular tearing and damage, surgical management is hazardous and often unrewarding. We report a successful surgical treatment of an acute type A aortic dissection associated with Ehlers-Danlos syndrome.

Keyword

Ehlers-Danlos syndrome; Aortic dissection; Aortic aneurysm, arcending

MeSH Terms

Ehlers-Danlos Syndrome*
Hemorrhage
Skin
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