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Long term results of lung resection in cystic fibrosis patients with localised lung disease
  1. J S A Lucas1,
  2. G J Connett1,
  3. J Fairhurst2
  1. 1Department of Paediatrics, Southampton General Hospital, Tremona Road, Southampton SO16 6YD, UK; garyconnett{at}suht.freeserve.co.uk
  2. 2Department of Paediatric Radiology, Southampton General Hospital, Tremona Road, Southampton SO16 6YD, UK

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We have previously reported favourable short term outcomes following lobectomy in six children with cystic fibrosis and severe localised bronchiectasis (range 6 months to 6 years post-operation).1 Prior to surgery all had significant respiratory symptoms despite aggressive conventional treatment, including frequent courses of intravenous antibiotics. Computerised tomography and ventilation scans showed severe localised disease with little or no evidence for bronchiectasis elsewhere. Lung function was maintained or improved in all but one case from six months post-surgery, and all had improved symptoms.

All children have now been reassessed at least four years postoperatively (table 1). Three remain much improved, with few symptoms and minimal need for intravenous antibiotic therapy. One child remains better than prior to surgery, but has recently required increased intervention to maintain wellbeing (case 5). Two children require antibiotics as frequently as prior to surgery with chronic signs (cases 3 and 6). There were no preoperative risk factors predictive of a less favourable outcome in these patients. Lung function has been maintained in all except one (case 6).

Follow up chest x rays were assessed by a consultant paediatric radiologist, using the Chrispin Norman Scoring system.2 New radiological changes have tended to occur in the zones previously occupied by the resected lobe (table 2). One of the patients has had a bronchoscopy following right upper lobectomy (case 3). Upwards displacement of the right middle lobe bronchus appeared to be causing airway narrowing. Such distortion of the lung anatomy may predispose to bronchiectasis in lobes that have shifted to occupy the spaces previously occupied by the resected lobe.

Our long term results suggest that surgical resection is a worthwhile option in selected children with severe localised symptomatic bronchiectasis. Detailed preoperative assessment is essential to exclude patients with more extensive lung damage. While there is a good long term improvement of symptoms and preservation of lung function in the majority of patients, there is a tendency for new radiological abnormalities to occur in the zones previously occupied by resected lobes.

Table 1

Lung function data: simple spirometry after bronchodilator inhalation

Table 2

Chest x ray score

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