Background: Recent data on the yield of bronchoscopy in pediatric cystic fibrosis (CF) patients are lacking. Therapeutic bronchoscopic lavage with the mucolytic recombinant human deoxyribonuclease (rhDNase) has been used during CF bronchoscopies, but efficacy data are scarce.
Methods: A retrospective review of all bronchoscopies performed in pediatric CF patients in our hospital in the past 15 years.
Aims of the Study: To evaluate indications for and safety of bronchoscopy in pediatric CF patients, to describe the findings of bronchoscopy and the contribution of these findings to clinical management, and to evaluate the application of bronchoscopic lavage with rhDNase.
Results: Between 1992 and 2007, 66 bronchoscopies were performed in 48 CF patients (25 males) at a median (range) age of 8.3 (0.1 to 20.4) years. Indications for bronchoscopy were persistent atelectasis (42%), refractory symptoms (29%), need for microbiologic culture (11%), suspected anatomic abnormality (11%), and bronchial toilet (7%). Relevant new information with therapeutic consequences was obtained in 28 (42%) bronchoscopies, including a first Pseudomonas aeruginosa infection (n=3), infection with atypical mycobacteria (n=3), or Aspergillus fumigatus (n=5), and severe tracheo(broncho)malacia (n=4). In patients with atelectasis, rhDNase lavage was associated with improved chest radiograph scores and a transient decline in forced vital capacity. In 7 of 11 patients with refractory symptoms, lung function tended to improve after rhDNase lavage. No serious complications were observed after bronchoscopy and rhDNase lavage.
Conclusions: Bronchoscopy provides clinically relevant information in about 40% of these pediatric CF patients. Lavage with rhDNase seemed safe, and was associated with improved chest radiographs in patients with therapy resistant atelectasis.