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.