Pneumothorax in cystic fibrosis: Prevalence and outcomes in Scotland

BackgroundPneumothorax 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.MethodsCase review of patients from the three Scottish adult CF centres.ResultsA total of 22 episodes of pneumothorax occurred in 20 patients over a 12 year 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 1 year.ConclusionPneumothorax 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.