Takazawa A, Hashimoto A, Aomi S, Yamaki F, Sakahashi H, Koyanagi H
Department of Cardiovascular Surgery, Tokyo Women's Medical College, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1995 Nov;43(11):1850-3.
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.
我们曾为一名患有埃勒斯-丹洛斯综合征的42岁男性进行胸主动脉瘤的移植物置换术。该患者在此次胸段手术前1年接受了腹主动脉瘤的移植物置换术，但其外表并无异常。胸部CT扫描显示最大直径为80mm的胸主动脉瘤。我们采用深低温及逆行脑灌注技术，从升主动脉至胸降主动脉近端进行了胸主动脉移植物置换术。主动脉壁非常薄，以至于我们在主动脉残端外壁使用特氟龙毡来加强移植物吻合。切除的主动脉壁的III型胶原染色显示III型胶原缺乏，这与埃勒斯-丹洛斯综合征（IV型）相符。术后过程顺利，患者恢复了正常生活。
