Pulmonary Procedures |

Mounier-Kuhn Syndrome: Management of a Patient for 13 Years With This Rare Diagnosis FREE TO VIEW

Jason Stienecker, DO; Craig Shapiro, MD
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Parkview Medical Center, Department of Pulmonary and Critical Care Medicine, Pueblo, CO

Chest. 2015;148(4_MeetingAbstracts):801A. doi:10.1378/chest.2269870
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SESSION TITLE: Interventional Pulmonary Cases

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Sunday, October 25, 2015 at 10:45 AM - 11:45 AM

INTRODUCTION: Mounier-Kuhn Syndrome (MKS) is a rare disease that is represented by abnormal enlargement of the central airways. The pathophysiology behind the disease is related to atrophy or absence of smooth muscle and elastic fibers. There are three major subtypes of disease: type 1 shows symmetric dilation of trachea and major bronchi; type 2 has dilation with diverticula in the trachea; type 3 has diverticula extending to the distal bronchi1. We present a case of type 3 MKS and our management experience over the last 13 years.

CASE PRESENTATION: A 56-year-old male patient presented to our service originally April of 2002 with the established diagnosis of MKS previously undergoing lung volume reduction surgery, two failed tracheal stents, and a bronchial stent. The patient was admitted for worsening shortness of breath. Imaging revealed dilated trachea and bronchi, diverticula, multiple cysts, and bronchiectasis all consistent with MKS (Fig 1). The patient underwent diagnostic bronchoscopy showing tracheal and bronchial dilation, thick mucous occluding his stent, and collapse of the trachea upon exhalation (Fig 2). The obstructing mucous was removed and the patient was treated with antibiotics and physiotherapy, recovering well. This patient has continued under our care and has scheduled broncoscopies every 2-3 months for removal of mucous obstructions, the patient and his wife have open-access to the office for any signs or symptoms of reoccurrence of infection. The patient is compliant with percussion vest, and NIPPV at night. Through this open-access and aggressive home therapy the patient has maintained minimal decline in his FEV1 from 1.87 L to 1.68 L over the past 13 years. The patient has only required 5 admissions since 2002, never requiring intubation.

DISCUSSION: No long-term studies have been conducted for treatment of MKS. Through our treatment protocol it can be recognized that current recommendations for chest physiotherapy, early antibiotics2, and airway stents reduce hospitalizations. The addition of mucous clearance using bronchoscopy also decreases hospitalization when performed early.

CONCLUSIONS: Therapeutic bronchoscopy should be recommended for prevention of respiratory failure, hospitalization and pulmonary function decline for patients with Mounier-Kuhn Syndrome, in addition to standard of care.

Reference #1: Schwartz M, Rossoff L. Tracheobronchomegaly. Chest 1994;106:1589-1590

Reference #2: Himalstein M.R., Gallagher J.C. Tracheobronchiomegaly. Annals of otology, Rhinology and Laryngology, 82 (1973), pp. 223-227

DISCLOSURE: The following authors have nothing to disclose: Jason Stienecker, Craig Shapiro

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