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Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF

Anthony J Fischer1, Sachinkumar B Singh, Ryan J Adam

  • 1Department of Pediatrics, University of Iowa, Iowa City, Iowa.

Pediatric Pulmonology
|October 30, 2013
PubMed

Insights

Tracheomalacia (TM) is more common in children with cystic fibrosis (CF) than the general population. This condition increases the risk of airway obstruction and earlier Pseudomonas aeruginosa infection in CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Genetics and Genomics

Background:

  • Tracheomalacia (TM) affects approximately 1 in 2,100 children.
  • Abnormal tracheal development in cystic fibrosis (CF) animal models suggests a potential link to TM in children with CF.

Purpose of the Study:

  • To determine the prevalence of TM in children with CF.
  • To investigate the clinical impact and consequences of TM on CF patient outcomes.

Main Methods:

  • A longitudinal study of children with CF born between 1995 and 2012.
  • TM diagnosis based on dynamic tracheal collapse during bronchoscopy.
  • Assessment of TM's effect on FEV1, CT scan findings, and pathogen acquisition.

Main Results:

  • 15% of children with CF (15/97) showed TM on bronchoscopy.
  • TM was associated with meconium ileus (P=0.003) and pancreatic insufficiency.
  • Pseudomonas aeruginosa infection occurred 1.3 years earlier in children with TM (P=0.01).
  • FEV1 at age 8 was over 18% lower in patients with TM.
  • 3 of 15 patients with CF and TM experienced life-threatening airway obstruction, including one fatality.

Conclusions:

  • TM is significantly more prevalent in children with CF compared to the general population.
  • TM is linked to increased risk of airway obstruction and earlier acquisition of Pseudomonas aeruginosa in CF patients.
Abstract

Keywords:
airwaybronchoscopycystic fibrosisinfantmeconium ileusobstructive lung diseasepediatrictracheomalacia

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