[Successful graft replacement of a thoracic aortic aneurysm in a patient with Ehlers-Danlos syndrome].

We have experienced graft replacement of a thoracic aortic aneurysm in a 42-year-old man with Ehlers-Danlos syndrome. The patient received graft replacement of the abdominal aortic aneurysm 1 year before this thoracic operation but had no abnormality in his outside appearance. Thoracic CT scan revealed a thoracic aortic aneurysm of 80 mm in maximal diameter. We performed a graft replacement of the thoracic aorta from the ascending aorta to the proximal descending thoracic aorta using deep hypothermia and retrograde cerebral perfusion. The aortic wall was so thin that we used Teflon felt for reinforcement of graft anastomosis at the outside wall of the aortic stump. Type III collagen stain of the resected aortic wall showed deficiency of type III collage, which was consistent with Ehlers-Danlos syndrome (type IV). Postoperative course was uneventful, and the patient returned to his ordinary life.