Recurrent lobar atelectasis in a child with cystic fibrosis

Abstract

Cystic fibrosis pulmonary exacerbations in children usually manifest with increased cough and a fall in lung function. We report a challenging case where an 8-year-old girl has had frequent prolonged exacerbations over the last 3 years needing aggressive management, associated with multi-lobar collapse. She was born at term and presented with meconium ileus needing bowel resection and stoma formation which was reversed during first year of life. Cystic fibrosis was diagnosed on sweat testing, with a ΔF508 homozygous genotype. She has a younger brother aged 5 years, who also has cystic fibrosis. She was admitted as an infant with respiratory syncytial virus bronchiolitis at 7 months of age, requiring CPAP for few days. She was later admitted at 15 months of age with a pulmonary exacerbation, and a chest X-Ray showed left lower lobe collapse. Pseudomonas aeruginosa was isolated for the first time from bronchoalveolar lavage and she was treated with intravenous and nebulized antibiotics; she had a normal chest X-ray few weeks later. Nebulized dornase alpha (DNase) was started at 3 years of age and a gastrostomy inserted at age 6. A port-a-cath was inserted at age 7. Over the last 3 years she has had 2 to 3 pulmonary exacerbations per year requiring prolonged hospital admissions for up to 3 weeks. During these admissions she is treated with intravenous antibiotics, intensive physiotherapy, up to 4 times per day of nebulised hypertonic saline and twice daily DNase. In 2011 she had 5 hospital admissions for pulmonary exacerbations. Pulmonary exacerbations are preceded by trivial viral upper respiratory tract infection followed a few days later by acute onset of tachypnoea, dyspnoea and profound hypoxemia (oxygen saturations of 80–82% on admission). The chest X-ray shows lobar collapse with the left lower lobe most frequently involved as well as involvement of other lobes. Our approach is to treat her with humidified high flow oxygen, intravenous antibiotics, intensive physiotherapy and at least one early therapeutic bronchoscopy to re-inflate the collapsed lobe. Bronchoscopy is usually performed on the first or second day of admission and reveals very thick tenacious mucus, which is difficult to clear. We instill DNase during the procedure, and on some occasions she has chest physiotherapy under general anesthesia. Large mucus plugs can be removed, sometimes as bronchial casts. We believe that early bronchoscopy results in clinical and radiological improvement and she can be discharged home after 2 weeks with no respiratory distress and normal oxygen saturations (Figure 1). Open in a separate window Figure 1 Serial chest X-rays in a typical pulmonary exacerbation. a) X-ray on day of admission showing left, right lower & middle lobe collapse; b) X-ray 36 hr post bronchoscopy showing good inflation of right lung; c) X-ray 6 weeks later showing no lobar collapse