Abstract

Five cases of cystic fibrosis complicated by eight pneumothoraces are described. These cases come from a group of 49 patients who have attended the Brompton Hospital between 1964 and 1969 for management of their cystic fibrosis. Three of the patients, who had a total of six pneumothoraces, were managed by pleurectomy. Spontaneous pneumothoraces were a terminal event in a fourth patient, and an incidental finding which required no specific management in a fifth patient. The possible aetiology of the pneumothorax in cystic fibrosis is discussed with particular consideration of the known alveolar hypoplasia and overdistension and the abnormality of the visceral pleura. The results of pleurectomy in three patients are discussed both in relation to the successful avoidance of further pneumothoraces and in the notable absence of a restrictive ventilatory defect. In view of the relative success of pleurectomy we suggest that this treatment should be considered for any patient with cystic fibrosis who develops a spontaneous pneumothorax.