title: Successful outcome following pneumonectomy in a teenage boy with cystic fibrosis: a case report.
abstract: Cystic 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.
  
  
  
  A 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.
  
  
  
  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. 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.
authors.affiliation: Department of Paediatric Respiratory Medicine, Great North Children's Hospital, Newcastle upon Tyne, UK.
authors.forename: Zheyi
authors.initials: Z
authors.lastname: Liew
authors.affiliation: Department of Paediatric Respiratory Medicine, Great North Children's Hospital, Newcastle upon Tyne, UK.
authors.forename: Santosh
authors.initials: S
authors.lastname: Mallikarjuna
authors.affiliation: Department of Paediatric Cardiothoracic Surgery, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: Asif
authors.initials: A
authors.lastname: Hasan
authors.affiliation: Institute of Transplantation, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: F Kate
authors.initials: FK
authors.lastname: Gould
authors.affiliation: Department of Paediatric Gastroenterology, Great North Children's Hospital, Newcastle upon Tyne, UK.
authors.forename: Su
authors.initials: S
authors.lastname: Bunn
authors.affiliation: Department of Paediatric Respiratory Medicine, Great North Children's Hospital, Newcastle upon Tyne, UK.
authors.forename: Matthew F
authors.initials: MF
authors.lastname: Thomas
authors.affiliation: Institute of Transplantation, Freeman Hospital, Newcastle upon Tyne, UK.
authors.forename: Jim L
authors.initials: JL
authors.lastname: Lordan
authors.affiliation: Department of Paediatric Respiratory Medicine, Great North Children's Hospital, Newcastle upon Tyne, UK.
authors.forename: Christopher
authors.initials: C
authors.lastname: O'Brien
authors.affiliation: Institute of Cellular Medicine, Newcastle University and Department of Paediatric Respiratory Medicine, Great North Children's Hospital, Level 3 Clinical Resource Building, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK. malcolm.brodlie@ncl.ac.uk.
authors.forename: Malcolm
authors.identifier: 0000-0003-4591-8299
authors.initials: M
authors.lastname: Brodlie
mesh_terms: D000293:Adolescent; D001987:Bronchiectasis / Q000000981:diagnostic imaging; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D011013:Pneumonectomy; D011788:Quality of Life; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed
keywords: Case report; Cystic fibrosis; Destroyed lung; Paediatrics; Pneumonectomy