Lobectomy in patients with cystic fibrosis

Abstract

BACKGROUND: Some patients with cystic fibrosis (CF) develop severe but localized lung disease or recurrent hemoptysis/pneumothorax refractory to conventional medical therapies. METHODS: The outcomes of lung resection in patients with CF and worsening localized lung disease or recurrent hemoptysis/pneumothorax refractory to conventional therapy (n=15) were evaluated by reviewing the medical records of all patients with CF followed at the CF Center at Nationwide Children's Hospital (Columbus, Ohio, USA), who underwent lobectomy over a 15-year period (1998 to 2012). RESULTS: The median age of the 15 patients (93% Caucasian) was 20 years (range two to 41 years) and their mean forced expiratory volume in 1 s (FEV1) was 59.5% of predicted one year before surgery. Three patients died within two years after lobectomy; all three deaths occurred in patients with an FEV1 ≤40% of predicted before surgery. There were no significant changes in mean height, weight, body mass index, hospital admissions or antibiotic use over time. The mean FEV 1 decreased over time. Compared with at surgery, decline in FEV 1 in the year before surgery was -5.4% (P=0.024) and decline in the year after surgery was -1.3% (P=0.513); however, the difference in the rate of decline was not statistically significant. CONCLUSION: In patients with CF and localized worsening bronchiectasis and/or recurrent hemoptysis/pneumothorax, lobectomy carried a significant risk of mortality, especially in patients with FEV1 ≤40% of predicted, and should only be considered when all other measures fail. ©2014 Pulsus Group Inc. All rights reserved.