Abstract
BackgroundCystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung. This clinical situation is sometimes referred to as “destroyed lung”. Lung resection surgery is seldom indicated in cystic fibrosis and the associated medical literature is relatively sparse.Case presentationA 14 year old boy was referred to our centre for lung transplantation assessment. He had a chronic history of complete collapse and consolidation of his entire right lung. This was causing severe morbidity in terms of a continuous requirement for intravenous antibiotics over the last year, poor exercise tolerance with forced expiratory volume in 1 s of 35–40% predicted and need for home tuition. He also had significant nutritional problems and gastrointestinal symptoms following a Nissen’s fundoplication operation a year earlier. His nutritional status was firstly improved by the institution of jejunal feeding, which also greatly improved his distressing symptoms of nausea and wretching. After thorough multidisciplinary assessment the therapeutic option of performing a right pneumonectomy was considered due to relative sparing of the left lung, which demonstrated only mild bronchiectasis on computed tomography scan. This was performed uneventfully with a smooth peri-operative course. Targeted antimicrobials were used to treat the multiresistant organisms colonising his airways. Subsequently his quality of life, nutritional status and lung function all improved significantly and requirement for lung transplantation has been delayed.ConclusionsWe report a successful outcome following pneumonectomy in a teenage boy with cystic fibrosis referred to our centre for lung transplantation assessment with chronic unilateral collapse and consolidation of his right lung. We believe that improvement of nutritional status pre-operatively and targeted antimicrobial therapy, all contributed to the smooth peri-operative course. Pneumonectomy can be a feasible option in this clinical situation in cystic fibrosis but the associated risks must be considered carefully on a case-by-case basis.
Highlights
Cystic fibrosis lung disease is generally a diffuse process rarely one lung may become damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung
We report a successful outcome following pneumonectomy in a teenage boy with cystic fibrosis referred to our centre for lung transplantation assessment with chronic unilateral collapse and consolidation of his right lung
We believe that improvement of nutritional status pre-operatively and targeted antimicrobial therapy, all contributed to the smooth peri-operative course
Summary
We report a successful outcome following pneumonectomy in a teenage boy with CF and ‘destroyed’ right lung as a strategy to improve his clinical status and quality of life to an extent that it has delayed requirement for. We believe that careful case selection, improvement of nutritional status pre-operatively and targeted antimicrobial therapy all contributed to the smooth peri-operative course and successful outcome. All of these factors must be balanced against the risks of surgery and pneumonectomy should only be considered in patients with severe unilateral problems. It should be noted that in the context of a previous contralateral pneumonectomy future single lung transplantation may be technically more challenging but has been associated with good outcomes [11, 12] The boy in this case experienced significant gastrointestinal symptoms, in the form of gastroesophageal reflux disease pre-fundoplication, and severe problems post-fundoplication likely secondary to gastrointestinal dysmotility. This is likely to involve bronchoscopic clearance of mucus and secretions, targeted antimicrobials, mucolytics and intensive physiotherapy in order to try and avoid more chronic problems developing and irreversible lung damage
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