Spontaneous pneumothorax in cystic fibrosis

: The clinical course of 13 cystic fibrosis patients with a total of 24 episodes of pneumothorax was analysed. The study is based on 488 (273/215) patients seen over 20 respectively 10 years at the University Children's Hospitals Frankfurt/Main and Essen. A pneumothorax was observed with a frequency of 2.7% mainly in adolescents of young adults with advanced pulmonary disease (mean age 17.4 years). It was not seen before the age of 10 years. Thus among 255 patients at risk above 10 years a pneumothorax occurred in 5.1%. Presenting symptoms were acute chest pain (n = 17), dyspnea (n = 17) and irritating cough (n = 8). In two patients pneumothorax was an incidental diagnosis. A tension pneumothorax was seen in 7 (= 30%; 3 initial, 4 recurrences of which 3 were ipsilateral). Out of 11 recurrences (n = 6, ipsi- and n = 5, contralateral) 4 occurred only once, one twice and in one patient five times. Two patients died as a consequence of the event (one initially due to tension pneumothorax, one due to heart failure). The therapeutic approach was conservative. Without specific treatment pneumothorax resolved in 12 cases. Ten patients were treated by chest tube drainage and only one patient by pleurodesis with a sclerosing agent. Though the therapeutic results were favorable in the patients presented, the authors suggest more aggressive treatment in view of the high ipsilateral recurrence rate. Detailed recommendations are given.