Pneumothorax in cystic fibrosis: beyond the guidelines

Paediatr Respir Rev. 2016 Aug;20 Suppl:30-3. doi: 10.1016/j.prrv.2016.06.012. Epub 2016 Jun 15.


Pneumothorax is a serious but common complication in patients with cystic fibrosis (CF). It has adverse prognostic implications as well as associations with subsequent reduction in lung function and significant risk of recurrence. Management dilemmas frequently occur that are beyond current guidelines. We review the evidence and highlight management difficulties in pneumothoraces in CF.

Keywords: Cystic Fibrosis; Persistent air leak; Pleural intervention; Pleurodesis; Pneumothorax.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / microbiology
  • Cystic Fibrosis / physiopathology
  • Diagnostic Imaging
  • Disease Management
  • Humans
  • Male
  • Pneumothorax / diagnostic imaging
  • Pneumothorax / etiology*
  • Pneumothorax / physiopathology
  • Pneumothorax / therapy*
  • Prognosis
  • Respiratory Function Tests
  • Risk Factors