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Pneumothorax in cystic fibrosis

Ioannis P Kioumis1, Konstantinos Zarogoulidis1, Haidong Huang1

  • 11 Pulmonary Department, "G. Papanikolaou" General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece ; 2 Department of Respiratory Diseases Shanghai Hospital, II Military University Hospital, Shanghai 200438, China ; 3 Hematology Department, "Laiko" University General Hospital, Athens, Greece ; 4 Obstetric-Gynecology Department, "Thriassio" General Hospital of Athens, Athens, Greece ; 5 Surgery Department, University General Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece ; 6 Institute for Pulmonary Diseases of Vojvodina, Clinic for Thoracic Oncology, Faculty of Medicine, University of Novi Sad, Serbia; 7 Oncology Department, "Biomedicine" Private Hospital, Thessaloniki, Greece ; 8 Oncology Department, "Papageorgiou" General Hospital, Thessaloniki, Greece.

Journal of Thoracic Disease
|October 23, 2014
PubMed

Insights

Pneumothorax is a serious risk for cystic fibrosis (CF) patients, particularly those with lung infections or compromised respiratory function. Management is similar to non-CF patients, but outcomes worsen quality of life.

Area of Science:

  • Pulmonology
  • Critical Care Medicine

Background:

  • Pneumothorax is a frequent and dangerous complication in cystic fibrosis (CF) patients.
  • Risk factors include specific infections (P. aeruginosa, B. cepacia, Aspergillus), allergic bronchopulmonary aspergillosis (ABPA), hemoptysis, enteral feeding, and compromised respiratory function.
  • Structural lung damage, altered airflow, inhaled medications, and non-invasive positive pressure ventilation (NIPPV) also contribute to pneumothorax risk.

Purpose of the Study:

  • To review the predisposing factors, clinical presentation, and management of pneumothorax in cystic fibrosis patients.
  • To highlight the impact of pneumothorax on morbidity, mortality, and quality of life in CF.

Main Methods:

  • Review of existing literature on pneumothorax in CF patients.
  • Analysis of predisposing factors, clinical manifestations, and treatment strategies.

Main Results:

  • Pneumothorax presentation varies from severe to mild.
  • Management strategies are comparable to those for non-CF patients, including tube drainage, VATS, and pleurodesis.
  • Pneumothorax significantly increases short- and long-term morbidity and mortality in CF patients.

Conclusions:

  • Pneumothorax is a significant complication that worsens outcomes and quality of life for individuals with cystic fibrosis.
  • Prompt and appropriate management is crucial to mitigate the adverse effects of pneumothorax in CF.

Keywords:
Cystic fibrosis (CF)antibiotics, macrolidespneumothorax

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