Abstract
Objectives: Spontaneous pneumothorax (PTx) is a well recognised complication in CF and is associated with recurrence and increased morbidity and mortality. To inform future management, we looked at the treatment and outcome of PTx in CF patients attending our large adult centre. Method: We reviewed 64 episodes of spontaneous PTx in 28 CF patients (mean FEV1 32% predicted [range 16−68%], 36% male), focussing on modalities of treatment and ultimate outcomes. Results: Immediate treatments are shown in the table. Nine patients (32%) sustained bilateral PTx and 50% had ipsilateral recurrence (mean interval 4 months). Twenty four have died (63% at 1 year, 80% at 2 years, median survival 7 months [0−68]) after first PTx, with the remaining 4 alive at 6−34 months post PTx. Conclusions: We have confirmed that spontaneous PTx is a poor prognostic factor in CF with poor survival rates and a recurrence or a contralateral PTx increases mortality. A multidisciplinary approach towards the treatment modalities for the management of recurrent or persistent PTx is essential when dealing with this complication.
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