[A case report of tracheal stenosis due to true aortic arch aneurysm of retroesophageal right aortic arch associated with so called vascular ring-facial syndrome].

A 63-year-old man admitted to our hospital because of dyspnea and inspiratory stridor. The X-ray computed tomography and angiogram revealed tracheal stenosis due to compression by aortic arch aneurysm of retroesophageal right aortic arch. His face was congenitally asymmetrical, and he also showed anotia, and meatal atresia. In the operation, we approached the aneurysm via median sternotomy and left thoracotomy by the 3rd intercostal space, and found atretic left aortic arch in front of trachea. So, operative diagnosis was Edwards Ib complete vascular ring associated with right aortic arch aneurysm. The aneurysm was incised and the arch and its branches were reconstructed with vascular prosthesis under ECC using selective cerebral perfusion. Postoperatively, until 5th postoperative day his condition was uneventful, and he was neurologically almost normal. But on the 5th postoperative day, his hemodynamics suddenly deteriorated because of severe pneumonia and septicemia. On the 6th postoperative day, he died in spite of earnest resuscitation. We could not find any previous reports about this rare combination of diseases.