Anesthetic Considerations for Bilateral Lung Transplantation in Mounier-Kuhn Syndrome

Abstract

OUNIER-KUHN SYNDROME (MKS), or tracheobronchomegaly, is a rare disorder of unknown etiology that is characterized by marked dilation of the trachea and major bronchi, due to severe atrophy and thinning, as well as absence of elastic fibers and smooth muscular fibers of the large airways. 1 This syndrome commonly presents in men in the third or fourth decade of life. It is marked by weakness of the membranous and cartilaginous parts of the trachea and main bronchi leading to an insufficient cough, mucous retention, and tracheobronchial wall collapse during respiration. Symptoms can range from well-preserved lung function to refractory respiratory failure. There are fewer than 100 case reports in the literature with only 2 reports of successful lung transplantation for patients with this syndrome. 1–3 Herein, the authors present a case of a patient diagnosed with MKS presenting for bilateral lung transplantation and describe the one-lung ventilation (OLV) strategies that were attempted and utilized.