Aortic arch aneurysm of Takayasu arteritis associated with entero-Behçet disease

We report a case of a ruptured aortic arch aneurysm due to Takayasu arteritis concomitant with entero-Behçet disease. A 53-year-old woman with total left lung atelectasis underwent emergency total aortic arch replacement with a modified Bentall operation and elephant trunk procedure. The postoperative course was highly eventful. A pseudoaneurysm of the left coronary button occurred with mediastinitis due to fistula of the left bronchus into the remnant of the aneurysmal wall. The left main trunk was reconstructed with a saphenous vein graft. The left bronchial fistula into the esophagus was exposed and an esophageal stent was placed. Finally, the saphenous vein graft ruptured and the patient expired. The autopsy diagnosis was Takayasu arteritis. This is the first reported case of concomitant Takayasu arteritis and entero-Behçet disease.