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Successful outcome following pneumonectomy in a teenage boy with cystic fibrosis: a case report.

Lookup NU author(s): Dr Zheyi Liew, Professor Kate Gould, Dr Su Bunn, Dr Matthew Thomas, Dr James Lordan, Dr Malcolm Brodlie



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


BACKGROUND: 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.CASE PRESENTATION: 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.CONCLUSIONS: 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.

Publication metadata

Author(s): Liew Z, Mallikarjuna S, Hasan A, Gould FK, Bunn S, Thomas MF, Lordan JL, O'Brien C, Brodlie M

Publication type: Article

Publication status: Published

Journal: BMC Pulmonary Medicine

Year: 2017

Volume: 17

Online publication date: 13/01/2017

Acceptance date: 12/12/2016

Date deposited: 15/01/2017

ISSN (electronic): 1471-2466

Publisher: BioMed Central Ltd.


DOI: 10.1186/s12890-016-0350-x

PubMed id: 28086849


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Funder referenceFunder name
MR/M008797/1Medical Research Council
SRC 003CF Trust