Pneumothorax in Cystic Fibrosis

doi:10.1378/chest.123.1.217

Chest

Volume 123, Issue 1, January 2003, Pages 217-221

https://doi.org/10.1378/chest.123.1.217 Get rights and content

Spontaneous pneumothorax is a common complication in patients with cystic fibrosis (CF). It is thought to occur more frequently in patients with more advanced disease. Recommendations on the management of pneumothorax in CF are based on retrospective analyses and reports from CF centers. The following is a review of what has been published regarding the incidence and management of pneumothorax in this population, with some comment on the pathogenesis of the complication.

Section snippets

Epidemiology

The reported lifetime incidence of spontaneous pneumothorax in patients with CF ranges between 2.8% and 18.9%.²³ Combining the results of the publications with larger populations between 1968 and 1989 produces an overall incidence of 6.4%.²³⁴⁵⁶⁷⁸ Rich et al⁶ reported an overall incidence of 6.4%, although it increased to 11% when considering only those patients > 10 years of age. The annual incidence of pneumothorax determined by an analysis of the Cystic Fibrosis Foundation Registry database

Pathogenesis

The cause of spontaneous pneumothorax in CF patients has not been clearly established. One explanation has attributed pneumothoraces to the rupture of subpleural blebs on the visceral pleura. This is supported in one study⁶ by the finding of blebs in 84% of patients who were undergoing pleurodesis by abrasion. However, an examination of the visceral pleura of CF patients, either at autopsy or after they had undergone pleurectomy, found that their visceral pleura were not different from those of

Diagnosis

The patient with spontaneous pneumothorax typically presents with acute onset of chest pain or dyspnea. In one report,⁴ chest pain was a presenting symptom in 50% of patients, while dyspnea occurred in 65%. Interestingly, hemoptysis was a presenting finding in 6 of 32 patients (19%) and was the sole finding in 1 patient. In that same report, 5 of 32 patients (16%) were asymptomatic at the time of diagnosis, 2 of whom had the pneumothorax identified on a routine chest radiograph.

The chest

Management

The methods of managing spontaneous pneumothorax for CF patients has included observation,³⁵⁶⁷¹³ needle aspiration,¹³ closed thoracotomy using a chest tube,³⁵⁶⁷¹³¹⁴ chemical pleurodesis with quinacrine,⁶⁷¹³¹⁶²⁴ silver nitrate,⁷¹³ tetracycline⁵⁷¹³¹⁴ and talc,⁷¹¹¹⁴²⁵ and surgical pleurodesis by either parietal pleurectomy or pleural abrasion.³⁵⁶⁷¹³¹⁶ The combined results of these reports are shown in Table 1. All of these published results are based on the retrospective analysis of charts and not

Outcomes

The prognosis for the patient after a spontaneous pneumothorax has been reported to be very poor, with death occurring approximately 30 months after the occurrence of the first pneumothorax.⁷ There are frequent reports of death occurring at the time of a pneumothorax or shortly thereafter,³⁵¹⁰ and it is thought to coincide with the finding that this complication tends to occur in patients with more severe lung disease. A history of pneumothorax was included on a prognostic scoring system,

Summary

In conclusion, spontaneous pneumothorax is a relatively frequent complication in patients with CF. It is more likely to occur later in the course of airways disease, but there are no factors that will accurately predict those who will experience this complication. Although the current consensus suggests a conservative approach to management, either by observation or tube thoracostomy, the rate of lack of response to treatment or recurrence is so high that most patients will require pleurodesis

ACKNOWLEDGMENT

The author thank Steven Sahn, MD, for his editorial expertise in the preparation of this manuscript.

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ReviewsPneumothorax in Cystic Fibrosis

Section snippets

Epidemiology

Pathogenesis

Diagnosis

Management

Outcomes

Summary

ACKNOWLEDGMENT

J Thorac Cardiovasc Surg

J Pediatr Surg

Ann Thorac Surg

J Pediatr

Chest

Chest

J Pediatr Surg

Hum Pathol

Chest

Ann Thorac Surg

Ann Thorac Surg

Chest

J Pediatr

Pneumothorax: an unusual complication in fibrocystic disease

Am J Dis Child

Reviews
Pneumothorax in Cystic Fibrosis