Perforation of the ascending aorta with a hematoma extending into the left-side upper extrapleural cavity.

We herein present an extremely rare case of a perforation of the ascending aorta with a hematoma extending into the left-side upper extrapleural cavity. A 62-year-old male with a sudden onset of severe chest pain was referred to our institution because of an abnormal shadow in the left-side upper lung field. Computed tomography revealed a small fusiform aortic arch aneurysm and a hematoma extending to the left-side upper extrapleural cavity. We diagnosed the patient to have acute aortic syndrome and urgent surgery was thus performed. Major bleeding which might be caused by a progression of the perforation was seen during a dissection of the aorta. The aortic arch was transected and a total arch replacement was performed with a 26 mm Dacron graft. No findings of a rupture of the aortic arch aneurysm or dissection were observed. The histopathology of the aorta revealed a severe atheromatous lesion with calcification and thinning disarrayed elastic fibers. The postoperative course was essentially good except for the development of pericardial effusion which required drainage.