Related Experiment Videos
[Pneumothorax in cystic fibrosis]
1Medizinische Universitäts-Kinderklinik Bern.
Insights
Increased pneumothorax (PT) in cystic fibrosis (CF) patients necessitates effective treatment. Quinacrine sclerosis offers a successful, less invasive option for preventing recurrent PT after initial chest tube drainage.
Area of Science:
- Pulmonology
- Pediatric Medicine
- Thoracic Surgery
Context:
- Rising life expectancy in cystic fibrosis (CF) patients leads to more advanced lung disease.
- Pneumothorax (PT) is an increasingly common complication in advanced CF stages.
- This study retrospectively analyzed PT events in pediatric CF patients over 15 years.
Purpose:
- To evaluate the long-term efficacy of different therapeutic modalities for pneumothorax in pediatric cystic fibrosis patients.
- To compare complication rates and hospital stays between treatment options.
- To identify optimal prophylactic strategies for recurrent pneumothorax in this population.
Summary:
- 23 pediatric CF patients experienced 51 PT events, including 18 tension pneumothoraces, with a mean age of 15.6 years at first episode.
- Therapeutic success rates varied: observation (24%), pleural suction drainage (9%), quinacrine sclerosis (75%), and open thoracotomy (90%).
- Quinacrine sclerosis demonstrated comparable efficacy to thoracotomy with fewer complications and shorter hospitalizations.
Impact:
- Quinacrine sclerosis is recommended as a primary prophylactic treatment for recurrent PT in CF patients post-chest tube evacuation.
- This approach may improve patient outcomes and reduce the burden of recurrent pneumothorax.
- The study found no CF disease deterioration or PT-related deaths, suggesting manageable risks with appropriate treatment.
Abstract:
Longer life expectancy for patients with cystic fibrosis (CF) has resulted in an increased incidence of pneumothorax (PT), which occurs in advanced stages of CF. This study is based on 276 patients with CF followed up between 1974 and 1989 at the Department of Pediatrics, University of Berne, Switzerland. 23 children or adolescents (10 boys and 13 girls) experienced 51 events of PT, 18 of which were tension pneumothoraces. The average age at first episode was 15.6 years. The long-term success rates of the four different therapeutic modalities used were 24% for observation, 9% for pleural suction drainage only, 75% for quinacrine sclerosis and 90% for open thoracotomy. Sclerosis was associated with fewer complications and shorter hospital stays than thoracotomy. We therefore conclude that, following initial evacuation of the PT using a chest tube, quinacrine sclerosis should be considered as primary prophylaxis for recurrent PT. Only in 3 of the 23 patients did the course of the basic disease deteriorate substantially following PT, and there was no death related to the PT episodes.