Pulmonary resection for complications of cystic fibrosis

Abstract

Pulmonary sequelae account for a large proportion of the morbidity and mortality of cystic fibrosis. Bronchiectasis, hemoptysis, and abscess formation are often not responsive to conservative medical therapy. Pulmonary resection in selected cystic fibrosis patients is safe and therapeutically rewarding. Eleven pulmonary resections in ten patients with cystic fibrosis were performed. Patients ranged from 2.5 to 19 years of age. Indications for resection were: (1) abscess and bronchiectasis (nine patients), (2) atelectasis and mass (one patient), and (3) life-threatening hemorrhage (one patient). Surgical resection was employed only for medically refractory lesions which were life-threatening or contaminated otherwise functional lungs. Resection was limited to the most severely diseased areas, sparing functional lung parenchyma when possible. In this series, 9 lobectomies, 1 pneumonectomy, and 1 segmentectomy were performed. Preoperative management included aggressive chest physiotherapy and postural drainage, rigid bronchoscopic lavage, and broad-spectrum parenteral antibiotics. All patients were extubated in the operating room immediately postoperatively. Tracheostomy was not employed. There were no perioperative complications. All patients experienced subjective improvement. Objectively, improvement following surgical resection included: decreased cough and sputum production, and decreased incidence of exacerbations of pulmonary infections. Limited pulmonary resection when combined with intensive preoperative pulmonary toilet is a safe adjunct in the treatment of bronchiectasis and hemoptysis secondary to cystic fibrosis. Pulmonary resection should be limited to only severely destroyed lung parenchyma which is refractory to medical management. In contradistinction to other authors we have not found tracheostomy a necessary adjunct in surgical management.