The patient was a 37 year-old man. We diagnosed Loeys-Dietz syndrome based on his physical characteristics that were widely spaced eyes and brachycephaly etc. Since he developed De Bakey III b aortic dissection 3 months later, he needed surgical repair for saccular-shaped distal arch aortic aneurysm. We performed total aortic arch replacement for the aneurysm and valve-sparing aortic root reconstruction for dilatation of the Valsalva sinus. Furthermore we performed the frozen elephant trunk technique for residual aortic dissection at the same time. After 18 months from the operation, we were able to recognize by computed tomography that the false lumen of the aorta next to the stent graft was thrombosed and absorbed and finally disappeared. The stent graft treatment for patients with connective tissue disease might be an effective method and deserves more attention.

A 54-year-old woman underwent abdominal aortic replacement for abdominal aortic aneurysm in March 2012. Approximately 6 months after surgery, she was taken by ambulance to hospital due to thoracodorsal pain, lower limb paralysis and pain. Emergency computed tomography indicated acute aortic dissection involving the ascending aorta, aortic arch, and descending aorta. The outline of the prosthesis implanted in the abdominal aorta was absent, and emergency surgery was performed immediately by median sternotomy to treat suspected complete obstruction. Following confirmation of brachiocephalic artery dissection, extracorporeal circulation was started with drainage of blood from the vena cava and the return via left axillary artery, plus perfusion in both lower limbs. However, the level of regional oxygen saturation declined as the flow of extracorporeal circulation increased. To solve this problem, an incision was made in the ascending aorta, and an aortic cannula was inserted directly into the true lumen. Aortic arch replacement was then performed, but this central repair failed to improve blood flow in both the left and right femoral artery. Proximal thrombectomy successfully removed a large amount of thrombi, but did not improve blood circulation. Left axillobifemoral bypass was subsequently performed, and improved lower limb blood circulation, but with residual motor impairment. Since the patient regained somatosensory sensation and was able to perform simple exercises, rehabilitation was started. Hemodialysis was required after abnormal increases in muscle enzyme levels and white blood cell count, but this was later discontinued following improvement of renal function. The patient was transferred to a rehabilitation clinic 54 days after surgery.