Distal aortic arch aneurysm in an adult case of PHACE syndrome†

A rare case of adult PHACE syndrome with a distal aortic arch aneurysm was treated surgically. The patient had several anomalies including uneven cerebral blood flow, a small left common carotid artery and a narrowed transverse aorta. Under deep hypothermic circulatory arrest, aortic replacement with reconstruction of the left subclavian artery was performed uneventfully. Cystic degeneration of the tunica media was observed in the aneurysm wall.