Ortner's Syndrome Revealing an Aortic Arch Aneurysm in Behçet's Disease: A Case Report

Background: Behçet’s disease is a systemic vasculitis that can affect vessels of any size. Arterial involvement is uncommon but may lead to severe complications. Ortner’s syndrome, defined as hoarseness caused by recurrent laryngeal nerve compression from cardiovascular structures, is an exceptional mode of presentation.

Case Presentation: We report the case of a 34-year-old man with progressive hoarseness, fever, weight loss, arthralgias, and scrotal ulcers. Laboratory findings showed elevated inflammatory markers. CT angiography revealed a large saccular aneurysm of the aortic arch with mural thrombus compressing the left pulmonary artery. The diagnosis of Behçet’s disease was retained. Because of active vascular inflammation, surgical repair was postponed. The patient was treated with high-dose corticosteroids followed by cyclophosphamide, leading to clinical and biological improvement.

Conclusion: This case illustrates the rare occurrence of Ortner’s syndrome as the first manifestation of Behçet’s disease. It underlines the importance of considering large-vessel vasculitis in young patients with unexplained hoarseness.