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

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

• Jeong DS
• Kim KH
• Ahn H

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