[Pneumothorax in cystic fibrosis]

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.