Bronchopleural fistula (BPF) is an abnormal communication between the bronchus and the pleural space,1,2 commonly occurring after pulmonary resection or due to a spontaneous pneumothorax secondary to an underlying lung disease; the latter occasionally referred to as alveolopleural fistula.3 The incidence of BPF is estimated to be 2 to 6 per 100,000 per year following primary spontaneous pneumothorax and 26 per 100,000 per year following secondary pneumothorax.3
Granulomatous polyangitis (GPA) is a small vessel necrotizing granulomatous vasculitis affecting upper and lower respiratory tract.4 BPF can occur in the setting of GPA, typically with necrotizing vasculitis or due to rupture of cavitary nodules into the pleural space.5–7 We present a case of BPF in the setting of GPA treated with immunosupressive therapy and endobronchial valves (EBVs) with a longitudinal follow-up.
A 62-year-old man with chronic obstructive pulmonary disease and significant smoking history presented initially with hemoptysis and sinus congestion. Computed tomography of the chest revealed bilateral infiltrates suggestive of diffuse alveolar hemorrhage. Structurally, centrilobular emphysema with paraseptal blebs was also present. He was intubated for hypoxic respiratory failure. Bronchoscopy demonstrated friable airways and bronchoalveolar lavage was consistent with diffuse alveolar hemorrhage. Erythrocyte sedimentation rate, C-reactive protein, cytoplasmic antineutrophil cytoplasmic antibodies, and proteinase 3 antibodies were found to be in the abnormal range, further supporting the diagnosis of GPA. Cyclophosphamide and steroid therapy were initiated.
Eleven days after the presentation, the patient developed spontaneous bilateral pneumothoraces requiring chest tubes placement. The left lung air leak resolved after thoracostomy tube only and was subsequently removed. However, the air leak from the right lung persisted (Fig. 1). Chemical pleurodesis with doxycycline was performed twice (51 and 56 d after thoracostomy) but was unsuccessful. Because of his debilitated state after prolonged hospitalization, invasive surgical options were not favored.
The patient underwent intrabronchial valve (Spiration IBV) placement 66 days after initial pneumothorax and thoracostomy (Fig. 2). Intraoperatively, individual airway segments of the right upper lobe were occluded sequentially to localize the fistula and improvement was noted, however, the air leak persisted even after the right upper lobe was occluded entirely although qualitatively lower leak by visual estimate. A total of 5 IBV valves were deployed into the anterior, apical, and posterior subsegments of the right upper lobe (7-mm valves in RB1a, RB1b, RB2, RB3b; 6-mm valve in RB3a).
The patient was converted to a Heimlich valve 4 days later, and after a prolonged hospitalization was discharged 7 days after IBV placement. Time to air resolution was 54 days, and time to chest tube removal was 106 days after EBV placement. Valves were removed 62 days later according to standard management (Table 1).
At the time of writing, the patient is currently 7 months out from EBV placement. He has had an excellent recovery, has increased exercise tolerance, and has achieved a quality of life equaling that before his hospitalization.
BPFs can be problematic, leading to increased hospital length of stay, morbidity, and mortality. Management options for BPFs include thoracostomy tube, blood patch, pleurodesis, and surgery. Less invasive approaches, such as EBVs employed here, have shown promise.2,8–10 The use of these valves is Food and Drug Administration approved for BPF following thoracic surgery (ie, lung volume reduction surgery or lung resection) but this indication was considered “off label” as treatment of medical lung disease. However, several cases have been reported wherein this method of bronchial occlusion has led to successful resolution of fistulas in the setting of fungal infections, tuberculous, and nontuberculous empyema.11,12
These 1-way valves allow air and mucus to exit the diseased segment of lung during expiration, but prevent the reentry of air upon inspiration. The targeted segment may undergo atelectasis, achieving nonsurgical lung volume reduction, and allowing the remaining lung to compensate for the loss of volume.2,8,9 The use of these valves has shown to decrease hospitalization, morbidity, and mortality in these patients. In this case, the patient endured a prolonged hospitalization (82 d) and was able to be discharged only 7 days after EBV placement. This facilitated engagement in a pulmonary rehabilitation program, increased physical activity, and ultimately resumption of normal activity for the patient. To our knowledge, this is the first case of EBV used to treat BPF in the setting of underlying GPA. This underscores the point that in appropriate settings, EBVs can decrease morbidity and mortality, and significantly improve the quality of life.
1. Lois M, Noppen M. Bronchopleural fistula
: an overview of the problem with special focus on endoscopic management. Chest. 2005;128:3955–3965
2. Alexander ES, Healey TT, Martin DW, et al. Use of endobronchial valves for the treatment of bronchopleural fistula
after thermal ablation of lung neoplasms. J Vasc Interv Radiol. 2010;23:1236–1240
3. Wood DE, Cerfolio RJ, Gonzalez X, et al. Bronchoscopic management of prolonged air leak. Clin Chest Med. 2010;31:127–133
4. Hansell DM. Idiopathic interstitial pneumonias and immunological diseases of the lungs. Imaging of Diseases of the Chest. 20095th ed London, UK Mosby
5. Jaspan T, Davison AM, Walker WC. Spontaneous pneumothorax in Wegener’s granulomatosis. Thorax. 1982;37:774–775
6. Koyoma S, Murakami K, Sakakibara T. Massive pleural effusion and bronchopleural fistula
in Wegener’s granulomatosis. Sarcoidosis Vasc Diffuse Lung Dis. 2010;27:76–79
7. Storelli E, Casali C, Natali P. Unusual pathogenesis of spontaneous pneumothorax secondary to Wegener’s granulomatosis. Ann Thorac Surg. 2007;84:288–290
8. Chung SC, Peters MJ, Chen S, et al. Effect of unilateral endobronchial valve
insertion on pulmonary ventilation and perfusion: a pilot study. Respirology. 2010;15:1079–1083
9. Travaline JM, McKenna RJ, DeGiacomo T, et al. Treatment of persistent pulmonary air leaks using endobronchial valves. Chest. 2009;136:355–360
10. Gillespie CT, Sterman DH, Cerfolio RJ. Endobronchial valve
treatment for prolonged air leaks of the lung: a case series. Ann Thorac Surg. 2011;91:270–273
11. Ferguson JS, Sprenger K, Van Natta T. Closure of bronchopleural fistula
using bronchoscopic placement of an endobronchial valve
designed for the treatment of emphysema. Chest. 2006;129:479–481
12. Snell GI, Holsworth L, Fowler S, et al. Occlusion of a broncho-cutaneous fistula with endobronchial one-way valves. Ann Thorac Surg. 2005;80:1930–1932