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SPONTANEOUS PNEUMOTHORAX IN PATIENTS WITH CYSTIC FIBROSIS (CF): 10 YEARS EXPERIENCE
  1. R A Khan1,
  2. P Greally1,
  3. B Elnazir1
  1. 1Department of Respiratory Medicine, National Children Hospital, Tallaght, Dublin, Ireland

Abstract

Spontaneous pneumothorax is a major cause of morbidity and mortality in cystic fibrosis (CF) patients.

Aims To look for spontaneous pneumothorax in patients with CF over a 10 year period.

Methods Retrospective study was done over 10 years to look for the incidence of spontaneous pneumothorax. Different variables included age, sex, age of diagnosis of cystic fibrosis, age at the time of pneumothorax, pulmonary function test pre and post pneumothorax, treatment, incidence of death, and time of death.

Results 7 patients were identified who had developed pneumothorax. 57% were female and 43% were male. The median age of diagnosis was 105 months. The median age of pneumothorax was 11.75 years. Chest pain, cough, and dyspnoea were found in all the patients as common symptoms. All patients needed oxygen. 64% had left sided and 36% had right sided pneumothorax. 3 patients were tried on nitrogen wash out which was unsuccessful. Chest drain was put in all 7 patients. Average time of insertion of chest drain was 2.2 days ranging from 1–8 days. No patient had needle aspiration. 100% had close thoracotomy with chest drain and 14% had blood pleurodesis following this. 28% had recurrent pneumothorax. Average length of time of recurrence was 36 days (22–50 days). Mortality rate post pneumothorax was 28% between 30–52 days.

Conclusion Spontaneous pneumothorax is an important and common problem and late complication in chronic cystic fibrosis patients. Patients with any respiratory signs should consult the medical team in view of higher rate of both unilateral and contralateral pneumothorax. A multicentre study is needed to determine the optimum timing and method of treatment.

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