title: Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF.
abstract: BACKGROUND
  Tracheomalacia (TM) occurs in approximately 1 in 2,100 children. Because the trachea develops abnormally in animal models of cystic fibrosis (CF), we hypothesized this may also occur in children with CF, increasing their risk of TM.
  
  
  OBJECTIVE
  To examine the prevalence and clinical consequences of TM in children with CF.
  
  
  METHODS
  We studied children with CF born between 1995 and 2012. TM was defined as dynamic collapse of the trachea, and the severity was recorded as described in the chart. The effect of TM on patient outcomes, including FEV1 , CT changes, and acquisition of CF pathogens, was assessed using a longitudinal patient dataset.
  
  
  RESULTS
  Eighty-nine percent of children with CF had at least one bronchoscopy (n = 97/109). Fifteen percent of these children had TM described in any bronchoscopy report (n = 15/97). Of the patients with TM, eight had meconium ileus (P = 0.003) and all were pancreatic insufficient. Pseudomonas aeruginosa infection occurred 1.3 years earlier among children with TM (P = 0.01). Starting FEV1 values by age 8 were diminished by over 18% of predicted for patients with TM. Life-threatening episodes of airway obstruction occurred in 3 of 15 patients with CF and TM, including one leading to death. Gender, prematurity, and hepatic disease were not associated with TM. No difference was observed in the frequency of bronchiectasis.
  
  
  CONCLUSIONS
  TM is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction and earlier Pseudomonas acquisition.
authors.affiliation: Department of Pediatrics, University of Iowa, Iowa City, Iowa.
authors.forename: Anthony J
authors.initials: AJ
authors.lastname: Fischer
authors.forename: Sachinkumar B
authors.initials: SB
authors.lastname: Singh
authors.forename: Ryan J
authors.initials: RJ
authors.lastname: Adam
authors.forename: David A
authors.initials: DA
authors.lastname: Stoltz
authors.forename: Christopher F
authors.initials: CF
authors.lastname: Baranano
authors.forename: Simon
authors.initials: S
authors.lastname: Kao
authors.forename: Miles M
authors.initials: MM
authors.lastname: Weinberger
authors.forename: Paul B
authors.initials: PB
authors.lastname: McCray
authors.forename: Timothy D
authors.initials: TD
authors.lastname: Starner
mesh_terms: D000402:Airway Obstruction / Q000150:complications; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007223:Infant; D008297:Male; D011552:Pseudomonas Infections / Q000150:complications*; D012189:Retrospective Studies; D055090:Tracheomalacia / Q000150:complications*
keywords: airway; bronchoscopy; cystic fibrosis; infant; meconium ileus; obstructive lung disease; pediatric; tracheomalacia