Mounier-Kuhn Syndrome (MKS) is a rare disorder characterized by central airway dilation, diverticulosis and tracheobronchomalacia (TBM). Symptoms include recurrent respiratory infections and bronchiectasis due to ineffective secretion clearance. Our objective is to evaluate effects of airway stenting and/or airway reconstruction surgery as therapeutic options.
Prospective observational study with retrospective review of medical records of consecutive patients with MKS evaluated for TBM. Patients underwent baseline and post intervention assessments of respiratory symptoms, health-related quality of life (HRQOL) and lung function.
11 men were included (median age 61) were identified. Co-morbidities included COPD (8), bronchiectasis (2) and UIP (1). Symptoms included dyspnea, productive cough and recurrent lower respiratory tract infections and respiratory failure (3). Mean tracheal diameters in the anteroposterior and sagital planes were 26.7 mm and 33.9 mm respectively. Mean right and left bronchial anteroposterior diameters were 20.5 mm and 20.4 mm respectively. All had severe and diffuse TBM. Of the 11 patients: 7 (63%) had a successful stent trial followed by surgery. 1 had surgery without a stent trial and 1 has a permanent stent. 1 elected not to have any intervention and 1 is awaiting surgery.Of 10 patients with airway stabilization, 9 (90%) reported improvement in respiratory symptoms. There was improvement in HRQOL (5 0f 6, 83%), dyspnea scores (4 of 5, 80%), FEV1 (6 of 7, 85%).
Central airway stabilization with silicone stents followed by reconstructive airway surgery is a therapeutic option for patients with MKS, improving respiratory symptoms, dyspnea scores, HRQOL and lung functions.
In this largest cohort of patients with MKS, central airway stabilization in an experienced center may improve symptoms.
Archan Shah, No Financial Disclosure Information; No Product/Research Disclosure Information