title: Airway stenting and tracheobronchoplasty improve respiratory symptoms in Mounier-Kuhn syndrome.
abstract: BACKGROUND
  Mounier-Kuhn syndrome (MKS) is a condition characterized by tracheobronchomegaly resulting from the loss or atrophy of musculoelastic fibers within the airway wall. Concomitant tracheobronchomalacia is seen in most patients with MKS, often leading to significant respiratory compromise due to bronchiectasis, increased dead space, and impaired secretion clearance.
  
  
  METHODS
  We report a series of 12 patients with MKS and tracheobronchomalacia who were evaluated at our institution for significant respiratory problems. Stent trials were conducted in 10 patients, with seven proceeding to operative tracheobronchoplasty (TBP) and one continuing with long-term stent placement. One patient underwent TBP without prior stent placement. Of the remaining three patients, two had no improvement with trials of stent placement, and a stent could not be placed in the third because of a large tracheal diameter.
  
  
  RESULTS
  Compared with baseline values, clinically significant improvements in health-related quality-of-life measures and pulmonary function testing were seen in patients who underwent central airway stabilization (n = 9). Complications of both stent placement and TBP were generally mild. However, one death was reported in the surgical group secondary to an exacerbation of preexisting interstitial pneumonia.
  
  
  CONCLUSIONS
  An aggressive approach that targets central airway stabilization may improve outcomes for patients with MKS.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00550602; URL: www.clinicaltrials.gov.
authors.affiliation: Division of Thoracic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
authors.forename: David D
authors.initials: DD
authors.lastname: Odell
authors.affiliation: Division of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
authors.forename: Archan
authors.initials: A
authors.lastname: Shah
authors.affiliation: Division of Thoracic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; Division of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
authors.forename: Sidhu P
authors.initials: SP
authors.lastname: Gangadharan
authors.affiliation: Division of Interventional Pulmonology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
authors.forename: Adnan
authors.initials: A
authors.lastname: Majid
authors.affiliation: Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
authors.forename: Gaetane
authors.initials: G
authors.lastname: Michaud
authors.affiliation: Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
authors.forename: Felix
authors.initials: F
authors.lastname: Herth
authors.affiliation: Pulmonary, Critical Care and Sleep Medicine, St. Elizabeth's Medical Center, Boston, MA. Electronic address: armin.ernst@caritaschristi.org.
authors.forename: Armin
authors.initials: A
authors.lastname: Ernst
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001980:Bronchi / Q000503:physiopathology*; D001999:Bronchoscopy / Q000295:instrumentation / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D011446:Prospective Studies; D011788:Quality of Life; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D015607:Stents; D014057:Tomography, X-Ray Computed; D014132:Trachea / Q000503:physiopathology*; D055089:Tracheobronchomalacia / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy*; D014137:Tracheobronchomegaly / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy*; D016896:Treatment Outcome