Thoracoabdominal aortic repair in a patient with Ehlers-Danlos syndrome.

Fukui, Toshihiro; Hiraoka, Daisuke; Uchimuro, Tomoya; Shimokawa, Tomoki; Takanashi, Shuichiro; Takahashi, Yukihiro · Ann Thorac Surg · 2014

case_report · Level V

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Abstract

Type IV Ehlers-Danlos syndrome is a life-threatening inherited disorder of connective tissue associated with multiple aneurysm formation. Thoracoabdominal aortic repair in these patients has rarely been performed. We report the case of a 13-year-old patient with Ehlers-Danlos syndrome who had multiple aortic operations from the ascending aorta to the thoracoabdominal aorta.

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