Lobectomy in patients with cystic fibrosis

Some patients with cystic fibrosis (CF) develop severe but localized lung disease or recurrent hemoptysis⁄pneumothorax refractory to conventional medical therapies.

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).

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 FEV1 decreased over time. Compared with at surgery, decline in FEV1 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.

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.