Abstract
Pseudocoarctation is a rare anomaly in the descending thoracic aorta and had been considered by most as a benign condition, warranting no specific therapy. In this article we present a juvenile patient who had an aortic aneurysm as a complication of pseudocoarctation. The patient was a 13-year-old boy with an asymptomatic mediastinal mass discovered on chest x-ray. Computed tomography and magnetic resonance angiogram demonstrated kinking in the distal aortic arch with a saccular aneurysm of the descending aorta. There was no significant pressure differential between arms and legs, and no evidence of increased collateral circulation (rib notching, etc). At operation, an extremely friable, saccular aneurysm of the descending thoracic aorta was found distal to a buckled and constricted aortic isthmus. The walls of the aneurysm were so thin that the aortic blood flow could be observed through it. With the use of hypothermic circulatory arrest, resection of the aortic aneurysm and buckled aorta just distal to the left subclavian artery, and insertion of a woven Dacron graft was performed. Microscopic examination revealed fragmented smooth muscle fibers in the media and small areas of mucoid degeneration. The patient progressed well without any neurological deficiency.
