Pneumothorax in cystic fibrosis: prevalence and outcomes in Scotland.
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.