Case Presentation
An eight-year-old girl with cystic fibrosis (genotype Delta F508/G542X), presented with productive cough, associated with impaired exercise tolerance. Examination revealed decreased air-entry on the left upper zone, with deterioration in lung function. Chest X-RAY showed collapse of the left upper lobe. She was treated with an empirical course of oral antibiotics, pending microbiological results, as per cystic fibrosis guidelines. She failed to respond, with persistence of clinical and radiological findings. A non-tuberculous Mycobacterium was isolated from her sputum, which was subsequently verified to be, Mycobacterium abscessus complex , detected by Mycobacterium DNA probe test and polymerase chain reaction by a reference lab.
She was admitted for three weeks of intravenous antibiotics (Amikacin, Meropenem, Cefoxitin), oral Azithromycin and nebulised Tobramycin, as well as intensive chest physiotherapy.
Initial Bronchoscopy revealed thick viscous mucus plugging of the left upper lobe bronchus with complete obliteration, making the prospects of clearance by conservative measures highly unlikely. Therapeutic bronchoalveolar lavage was attempted using normal saline (total volume of 50ml (2ml/kg). Dornase alfa 2.5mg was instilled at the opening of the left upper lobe bronchus at the end of the procedure. The patient underwent a total of 3 therapeutic bronchoscopies. Progress was slow but consistent with complete clearance of the bronchus with the final procedure.