Pneumothorax in cystic fibrosis: prevalence and outcomes in Scotland

J Cyst Fibros. 2010 Jul;9(4):246-9. doi: 10.1016/j.jcf.2010.04.005. Epub 2010 May 23.


Background: Pneumothorax is a feared complication of cystic fibrosis. With improved survival into adult life the incidence of pneumothorax is expected to increase. The optimal management of these patients is unclear.

Methods: Case review of patients from the three Scottish adult CF centres.

Results: A total of 22 episodes of pneumothorax occurred in 20 patients over a 12year period. 2 patients died as a result of the pneumothorax. 16 pneumothoraces were treated by insertion of an intercostal drain and 8 by observation. 8 patients suffered a prolonged air leak. 5 patients were treated with pleurodesis. Pneumothorax was associated with a small decline in lung function which persisted for at least 1year.

Conclusion: Pneumothorax can present a challenge to treat in adult CF. However successful outcomes can be achieved even in cases of prolonged air leaks. Current national guidelines help in selecting optimal pleural interventions.

Publication types

  • Multicenter Study

MeSH terms

  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / epidemiology
  • Cystic Fibrosis / therapy
  • Drainage
  • Female
  • Humans
  • Male
  • Pleurodesis
  • Pneumothorax / etiology*
  • Pneumothorax / mortality*
  • Prevalence
  • Scotland / epidemiology
  • Young Adult