BACKGROUND:

A small proportion of cystic fibrosis patients develop severe localised bronchiectasis. When this persists despite maximal medical therapy it presents a difficult management problem. Lung transplantation cannot be justified. We report encouraging results in six patients with severe localised bronchiectasis and cystic fibrosis who underwent pulmonary resection.

METHODS:

Each child had severe localised bronchiectasis despite maximal medical therapy. Intensive preoperative toilet was instituted and pulmonary resection undertaken when lung function was optimal.

RESULTS:

There was a marked improvement in symptoms in every case. No significant long-standing morbidity was associated with the resection. There was no significant decrease in pulmonary function following resection.

CONCLUSION:

Pulmonary resection should be considered in the management of severe localised bronchiectasis unresponsive to maximal medical therapy in cystic fibrosis patients.