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Pulmonary Procedures |

Mounier-Kuhn Syndrome:Tracheobronchomegaly Presenting as Persistent Dyspnea FREE TO VIEW

Audra Fuller, MD; Menfil Orellana-Barrios, MD; Gaurav Patel, MD; Tashfeen Mahmood, MD; Raed Alalawi, MD
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TTUHSC, Lubbock, TX


Chest. 2015;148(4_MeetingAbstracts):844A. doi:10.1378/chest.2264548
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Abstract

SESSION TITLE: Procedures Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare condition generally seen in middle aged men, mostly smokers1.

CASE PRESENTATION: A 61 year old man presented with persistent shortness of breath (SOB) for one week, particularly on exertion. He had a cough with scarce sputum production, a hoarse voice and constant wheezing. He was treated with nebulized albuterol, antibiotics and steroids for three days. He improved moderately but quickly deteriorated again after his discharge. He had history of hypertension, asthma and COPD from >30 pack years smoking. He was intubated once in his life and had multiple recent admissions for presumed COPD exacerbations. On admission he was found in moderate distress with an O2 sat 93% on 3L NC. He had loud, harsh inspiratory stridor audible over the neck, supraclavicular and upper sternal areas. He had decreased breath sounds in bilateral lung fields with mild scattered expiratory wheezing. Cardiac auscultation revealed tachycardia and a 4/6 systolic murmur over the left superior sternal border. Initial lab results were within normal limits except a mild respiratory alkalosis. Given his recurrent SOB, dyspnea on exertion and stridor, CT imaging of the neck and chest were performed (Figure 1). Bronchoscopy revealed increased tracheal and bronchial diameters, 91-100% dynamic collapsibility of the airway and copious clear bronchial secretions. Numerous saccular diverticula where noted within the main bronchi (Figure 2). Tracheal diameter measured 3.4cm, R main 3.2cm and L main 2.5cm. An intra-bronchial Y-stent (15 mm main diameter, 12 mm stem diameter) was placed 2cm below cricoid region. He had significant relief of his SOB and breath sounds improved bilaterally.

DISCUSSION: Although the etiology is unclear, the histopathology of MKS includes thinning of tracheal muscular mucosa with atrophy of longitudinal muscle and elastic fibers.2 MKS usually presents as chronic nonspecific respiratory symptoms and diagnosis requires a high clinical suspicion. CT scan is currently the main modality for diagnosis. Reported treatment options include: mucolytics, physical therapy, non-invasive CPAP ventilation, tracheobronchoplasty, lung transplantation, endoscopic laser and airway stenting. 3

CONCLUSIONS: Our case demonstrates the clinical, radiological and bronchoscopic findings of MKS. This case shows the similarity of MKS with more common pulmonary conditions like COPD and the also the feasibility of tracheobronchial stenting with good clinical outcome.

Reference #1: Krustins E. Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years. Clin Respir J. 2014.

Reference #2: Katz I, Levine M, Herman P. Tracheobronchiomegaly. The Mounier-Kuhn syndrome. The American journal of roentgenology, radium therapy, and nuclear medicine. 1962;88:1084-1094.

Reference #3: Krustins E, Kravale Z, Buts A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: A literature review. Respiratory Medicine. 2013;107(12):1822-1828.

DISCLOSURE: The following authors have nothing to disclose: Audra Fuller, Menfil Orellana-Barrios, Gaurav Patel, Tashfeen Mahmood, Raed Alalawi

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