Abstract
Introduction
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.
DECLARATIONS
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Parental consent obtained
PN
All authors contributed equally
She was born at term and presented with meco-nium ileus needing bowel resection and stoma formation which was reversed during first year of life. Cystic fibrosis was diagnosed on sweat testing, with a ?F508 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. Pseu-domonas 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).
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
Investigations
Airway microbiology shows one isolate of Pseudo-monas aeruginosa 2 years ago, no mycobacterial growth and 3 isolates of Aspergillus fumigatus over the last 12 months but no allergic broncho pulmonary aspergillosis (normal serum IgE, eosi-nophils and negative tests for aspergillus precipitating antibodies). The bronchoalveolar lavage cytology usually shows predominantly macro-phages and no significant neutrophils/eosino-phils/lymphocytes. She has no tracheo/ bronchomalacia or pulmonary hypertension. Immune function tests (total immunoglobulins, IgGsubsets, lymphocyte subsets, antibodies to Hib, tetanus) are normal. Allergy tests to common aeroallergens are negative. Chest CT 2 years ago showed no bronchiectasis or small airway disease. Her best FEV1 over last 12 months is 84% predicted.
Other management
Her compliance to treatment at home is good. Alongside twice daily nebulized hypertonic saline and DNase, three times a week azithromycin, montelukast, a minimum of twice daily physiotherapy, she has also had prolonged courses of oral steroids with no appreciable benefits. She is currently on oral itraconazole. During her pulmonary exacerbations she was also trialled on intravenous bronchodilators, and bi-level ventilation via facemask with no objective benefit.
Discussion
Lobar collapse is common in patients with cystic fibrosis. 1 Patients usually respond to intravenous antibiotics, physiotherapy and use of muco-kinetic agents. Very few patients do not respond to the above measures, especially those with bronchiectasis or structural airway abnormalities like bronchomalacia. The use of flexible broncho-scopy as a ‘secondary’ treatment along with installation of DNase is described in patients not responding to usual treatment in small case series,2,3 but no literature is available on the use of early therapeutic bronchoscopy in cystic fibrosis. The cause for recurrent lobar atelectasis in our patient is yet to be determined. Her compliance to treatment appears to be good, and visits by our specialist cystic fibrosis nurses have not yielded any allergic triggers at home. Our patient has profound hypoxemia disproportionate to clinical signs and X-ray changes suggesting more diffuse mucus plugging in multiple distal airways. Airway microbiology is repeatedly negative for bacteriology suggesting the possibility of abnormal airway mucus hyper secretion triggered by viral respiratory infection. Bronchial casts are removed on a couple of occasions suggesting the possibility of plastic bronchitis which is described in cystic fibrosis, 4 although we do not have histology results. However, it is not clear why she has such thick mucus, and why she has repeated lobar collapse, despite conventional therapy.
In conclusion, early therapeutic bronchoscopy appears to reduce the severity and duration of pulmonary exacerbations although the cause for these repeated episodes are yet to be identified.
Footnotes
Acknowledgements
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