CORRESPONDENCE 15. Berlinger B, Benker N, Weinbruch S, L’Vov B, Ebert M, Koch W, Ellingsen DG, Thomassen Y. Physicochemical characterisation of different welding aerosols. Anal Bioanal Chem 2011;399:1773–1780.
Copyright © 2014 by the American Thoracic Society
Successful Management of a Chronic, Refractory Bronchopleural Fistula with Endobronchial Valves followed by Talc Pleurodesis To the Editor: Lymphangioleiomyomatosis (LAM) is a rare, neoplastic lung disease that affects women more often than men, and is associated with cystic destruction of the lung (1, 2). Over 65% of patients with LAM develop pneumothorax during the course of their illness, followed by two or more recurrences, on average (3, 4). An attempt at pleural symphysis with the first pneumothorax is therefore recommended, despite pleurodesis failure rates that exceed those of
most other chronic lung diseases (3). The following case describes the use of one-way endobronchial valves to treat a persistent bronchopleural fistula that had failed to resolve after multiple prior medical and surgical management approaches. The patient was a 39-year-old nonsmoking female physician who developed a spontaneous right pneumothorax. Computed tomography (CT) scanning of the chest revealed a large collection of air in the right anterior hemithorax, and numerous large round cysts varying in size from 3 mm to 4.6 cm (Figure 1A). Because of the unusual cyst dimensions, and an atypical basilar and peripheral distribution, the patient was initially thought to have Birt-HoggDub´e syndrome, but genetic testing for folliculin mutations was negative. Her pulmonary function tests were normal, and the pneumothorax was treated with simple chest tube drainage. She developed a recurrent right-sided pneumothorax 6 months later, treated initially for 2 days with small-bore chest tube drainage as an inpatient and continuing for 5 days after discharge with Heimlich valve–regulated drainage. She developed shortness of breath and decreased exercise tolerance 3 days after the chest tube was removed. She was found to have a right-sided pneumothorax and
Figure 1. (A) Large right-sided pneumothorax (arrow) and multiple cystic lesions (arrow) were later confirmed to be due to lymphangioleiomyomatosis. Recurrent right-sided pneumothorax presented with dyspnea on exertion and reduction in forced vital capacity. Posteroanterior (B) and lateral (C) chest radiograph showed a right pleural effusion (arrow), but pneuomothorax was not readily apparent. Coronal (D) and sagittal (E) images from a computed tomography (CT) scan of the chest revealed a large anterior pleural air collection (D, arrow), and leftward mediastinal shift consistent with tension pneumothorax (E, arrow). (F) CT scan showing complete resolution of right-sided pneuomothorax 3 months after second endobronchial valve replacement and talc pleurodesis. A collection of talc within the fissure is visible (arrow).
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American Journal of Respiratory and Critical Care Medicine Volume 189 Number 4 | February 15 2014
CORRESPONDENCE underwent right video-assisted thoracotomy with biopsy, bleb resection, mechanical pleurodesis, and subtotal pleurectomy. Pathology was consistent with LAM, including typical smooth muscle cell infiltration and positive HMB-45 staining. She was discharged with good control of the pneumothorax, but was admitted to the hospital on three subsequent occasions over a 3-month period with recurrent right-sided pneumothorax, managed on each occasion with chest tube drainage. One month after the last chest tube was removed, she complained of reduced exercise tolerance, and her pulmonary function tests revealed a 50% reduction in FVC compared with a study completed 7 months before. Although a chest radiograph suggested that her lungs were fully expanded, a CT image of the chest showed a large anterior pleural air collection on the right with subtle leftward mediastinal displacement, consistent with a mild degree of tension (Figures 1B–1E). A chest tube was placed by interventional radiology, and she was found to have a large air leak. Repeat CT of the chest showed a persistent right-sided pneumothorax despite drainage under suction. The patient was taken to the operating room and intubated. A balloon occlusion catheter was deployed through bronchoscope into each subsegmental bronchus while monitoring the intensity of the air leak through the water chamber in the pleural drainage device to localize the airways subtending the leak. The leak was markedly attenuated by obstruction of superior segment of the right lower lobe and the medial basilar segment of the right lower lobe, the sites of her prior lung biopsies. One-way endobronchial valves were deployed into a subsegment of the superior segment of the right lower lobe. After the procedure, only rare bubbles appeared in the water seal chamber. A chest radiograph suggested good pleural apposition, and talc was delivered through the chest tube to provoke pleural symphysis. Repeat chest CT, however, revealed a residual hydropneumothorax. She was taken again to the operating room, due to continued air leak and failed talc pleurodesis. The endobronchal valve was removed and new valves were placed in the right lower lobe, including the posterior and anterior subsegments of the superior segment, and in the most proximal portion of the medial basilar segment. Postoperatively, the leak resolved, and repeat CT showed resolution of the right-sided pneumothorax. Chemical pleurodesis with talc was again delivered through the chest tube, and the patient was discharged home on Day 4 after the second pleurodesis attempt. At a follow-up visit 3 months later, the patient was asymptomatic, pulmonary function tests were normal, and the CT scan of the chest showed complete resolution of the pneumothorax (Figure 1F). The endobronchial valves were removed without complications. This case illustrates the use of one-way endobronchial valves to treat a chronic pneumothorax that had recurred and persisted despite multiple prior chest tube drainage procedures, and thoracic surgical interventions including mechanical pleurodesis, bleb resection, and pleurectomy. It was clear that the pneumothorax was under tension, and had not resolved because of a persistent bronchopleural fistula. The use of positive pressure and balloon occlusion to localize the airways subtending the fistula, followed by deployment of endobronchial valves, was successful in reducing the rate of air leak to levels that allowed for pleural apposition and fusion upon pleurodesis with talc. Although the use of endobronchial valves has been described as an alternative to lung Correspondence
volume reduction surgery (5), as a bridge to transplant in patients with cystic fibrosis with bronchopleural fistula (6), for management of pneumothorax due to lung volume reduction surgery (7), and for spontaneous pneumothorax in patients with emphysema (8), this is the first report of use of the technology to facilitate successful pleurodesis in a patient with chronic pneumothorax. Early use of removable endobronchial valves may be a viable strategy to reduce the morbidity associated with persistent bronchopleural fistulas in patients with LAM and other conditions. n Author disclosures are available with the text of this letter at www.atsjournals.org. William B. Cundiff, D.O. Francis X. McCormack, M.D. University of Cincinnati College of Medicine Cincinnati, Ohio Kathryn Wikenheiser-Brokamp, M.D. Cincinnati Children’s Hospital Medical Center and University of Cincinnati Cincinnati, Ohio Sandra Starnes, M.D. University of Cincinnati Cincinnati, Ohio Robert Kotloff, M.D. University of Pennsylvania Philadelphia, Pennsylvania Sadia Benzaquen, M.D. University of Cincinnati College of Medicine Cincinnati, Ohio
References 1. Johnson S. Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax 1999;54: 254–264. 2. McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest 2008;133:507–516. 3. Almoosa KF, Ryu JH, Mendez J, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest 2006;129:1274–1281. 4. Johnson SR, Tattersfield AE. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000;55:1052–1057. 5. Yim AP, Hwong TM, Lee TW, Li WW, Lam S, Yeung TK, Hui DS, Ko FW, Sihoe AD, Thung KH, et al. Early results of endoscopic lung volume reduction for emphysema. J Thorac Cardiovasc Surg 2004;127: 1564–1573. 6. Fischer W, Feller-Kopman D, Shah A, et al. Endobronchial valve therapy for pneumothorax as a bridge to lung transplantation. The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 2012;31: 334–336. 7. Feller-Kopman D, Bechara R, Garland R, Ernst A, Ashiku S. Use of a removable endobronchial valve for the treatment of bronchopleural fistula. Chest 2006;130:273–275. 8. Schiavon M, Marulli G, Zuin A, Nicotra S, Di Chiara F, De Filippis F, Fantoni U, Rea F. Endobronchial valve for secondary pneumothorax in a severe emphysema patient. Thorac Cardiovasc Surg 2011;59: 509–510.
Copyright © 2014 by the American Thoracic Society
491
Copyright © 2014 by the American Thoracic Society
Successful Management of a Chronic, Refractory Bronchopleural Fistula with Endobronchial Valves followed by Talc Pleurodesis To the Editor: Lymphangioleiomyomatosis (LAM) is a rare, neoplastic lung disease that affects women more often than men, and is associated with cystic destruction of the lung (1, 2). Over 65% of patients with LAM develop pneumothorax during the course of their illness, followed by two or more recurrences, on average (3, 4). An attempt at pleural symphysis with the first pneumothorax is therefore recommended, despite pleurodesis failure rates that exceed those of
most other chronic lung diseases (3). The following case describes the use of one-way endobronchial valves to treat a persistent bronchopleural fistula that had failed to resolve after multiple prior medical and surgical management approaches. The patient was a 39-year-old nonsmoking female physician who developed a spontaneous right pneumothorax. Computed tomography (CT) scanning of the chest revealed a large collection of air in the right anterior hemithorax, and numerous large round cysts varying in size from 3 mm to 4.6 cm (Figure 1A). Because of the unusual cyst dimensions, and an atypical basilar and peripheral distribution, the patient was initially thought to have Birt-HoggDub´e syndrome, but genetic testing for folliculin mutations was negative. Her pulmonary function tests were normal, and the pneumothorax was treated with simple chest tube drainage. She developed a recurrent right-sided pneumothorax 6 months later, treated initially for 2 days with small-bore chest tube drainage as an inpatient and continuing for 5 days after discharge with Heimlich valve–regulated drainage. She developed shortness of breath and decreased exercise tolerance 3 days after the chest tube was removed. She was found to have a right-sided pneumothorax and
Figure 1. (A) Large right-sided pneumothorax (arrow) and multiple cystic lesions (arrow) were later confirmed to be due to lymphangioleiomyomatosis. Recurrent right-sided pneumothorax presented with dyspnea on exertion and reduction in forced vital capacity. Posteroanterior (B) and lateral (C) chest radiograph showed a right pleural effusion (arrow), but pneuomothorax was not readily apparent. Coronal (D) and sagittal (E) images from a computed tomography (CT) scan of the chest revealed a large anterior pleural air collection (D, arrow), and leftward mediastinal shift consistent with tension pneumothorax (E, arrow). (F) CT scan showing complete resolution of right-sided pneuomothorax 3 months after second endobronchial valve replacement and talc pleurodesis. A collection of talc within the fissure is visible (arrow).
490
American Journal of Respiratory and Critical Care Medicine Volume 189 Number 4 | February 15 2014
CORRESPONDENCE underwent right video-assisted thoracotomy with biopsy, bleb resection, mechanical pleurodesis, and subtotal pleurectomy. Pathology was consistent with LAM, including typical smooth muscle cell infiltration and positive HMB-45 staining. She was discharged with good control of the pneumothorax, but was admitted to the hospital on three subsequent occasions over a 3-month period with recurrent right-sided pneumothorax, managed on each occasion with chest tube drainage. One month after the last chest tube was removed, she complained of reduced exercise tolerance, and her pulmonary function tests revealed a 50% reduction in FVC compared with a study completed 7 months before. Although a chest radiograph suggested that her lungs were fully expanded, a CT image of the chest showed a large anterior pleural air collection on the right with subtle leftward mediastinal displacement, consistent with a mild degree of tension (Figures 1B–1E). A chest tube was placed by interventional radiology, and she was found to have a large air leak. Repeat CT of the chest showed a persistent right-sided pneumothorax despite drainage under suction. The patient was taken to the operating room and intubated. A balloon occlusion catheter was deployed through bronchoscope into each subsegmental bronchus while monitoring the intensity of the air leak through the water chamber in the pleural drainage device to localize the airways subtending the leak. The leak was markedly attenuated by obstruction of superior segment of the right lower lobe and the medial basilar segment of the right lower lobe, the sites of her prior lung biopsies. One-way endobronchial valves were deployed into a subsegment of the superior segment of the right lower lobe. After the procedure, only rare bubbles appeared in the water seal chamber. A chest radiograph suggested good pleural apposition, and talc was delivered through the chest tube to provoke pleural symphysis. Repeat chest CT, however, revealed a residual hydropneumothorax. She was taken again to the operating room, due to continued air leak and failed talc pleurodesis. The endobronchal valve was removed and new valves were placed in the right lower lobe, including the posterior and anterior subsegments of the superior segment, and in the most proximal portion of the medial basilar segment. Postoperatively, the leak resolved, and repeat CT showed resolution of the right-sided pneumothorax. Chemical pleurodesis with talc was again delivered through the chest tube, and the patient was discharged home on Day 4 after the second pleurodesis attempt. At a follow-up visit 3 months later, the patient was asymptomatic, pulmonary function tests were normal, and the CT scan of the chest showed complete resolution of the pneumothorax (Figure 1F). The endobronchial valves were removed without complications. This case illustrates the use of one-way endobronchial valves to treat a chronic pneumothorax that had recurred and persisted despite multiple prior chest tube drainage procedures, and thoracic surgical interventions including mechanical pleurodesis, bleb resection, and pleurectomy. It was clear that the pneumothorax was under tension, and had not resolved because of a persistent bronchopleural fistula. The use of positive pressure and balloon occlusion to localize the airways subtending the fistula, followed by deployment of endobronchial valves, was successful in reducing the rate of air leak to levels that allowed for pleural apposition and fusion upon pleurodesis with talc. Although the use of endobronchial valves has been described as an alternative to lung Correspondence
volume reduction surgery (5), as a bridge to transplant in patients with cystic fibrosis with bronchopleural fistula (6), for management of pneumothorax due to lung volume reduction surgery (7), and for spontaneous pneumothorax in patients with emphysema (8), this is the first report of use of the technology to facilitate successful pleurodesis in a patient with chronic pneumothorax. Early use of removable endobronchial valves may be a viable strategy to reduce the morbidity associated with persistent bronchopleural fistulas in patients with LAM and other conditions. n Author disclosures are available with the text of this letter at www.atsjournals.org. William B. Cundiff, D.O. Francis X. McCormack, M.D. University of Cincinnati College of Medicine Cincinnati, Ohio Kathryn Wikenheiser-Brokamp, M.D. Cincinnati Children’s Hospital Medical Center and University of Cincinnati Cincinnati, Ohio Sandra Starnes, M.D. University of Cincinnati Cincinnati, Ohio Robert Kotloff, M.D. University of Pennsylvania Philadelphia, Pennsylvania Sadia Benzaquen, M.D. University of Cincinnati College of Medicine Cincinnati, Ohio
References 1. Johnson S. Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax 1999;54: 254–264. 2. McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest 2008;133:507–516. 3. Almoosa KF, Ryu JH, Mendez J, et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest 2006;129:1274–1281. 4. Johnson SR, Tattersfield AE. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000;55:1052–1057. 5. Yim AP, Hwong TM, Lee TW, Li WW, Lam S, Yeung TK, Hui DS, Ko FW, Sihoe AD, Thung KH, et al. Early results of endoscopic lung volume reduction for emphysema. J Thorac Cardiovasc Surg 2004;127: 1564–1573. 6. Fischer W, Feller-Kopman D, Shah A, et al. Endobronchial valve therapy for pneumothorax as a bridge to lung transplantation. The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 2012;31: 334–336. 7. Feller-Kopman D, Bechara R, Garland R, Ernst A, Ashiku S. Use of a removable endobronchial valve for the treatment of bronchopleural fistula. Chest 2006;130:273–275. 8. Schiavon M, Marulli G, Zuin A, Nicotra S, Di Chiara F, De Filippis F, Fantoni U, Rea F. Endobronchial valve for secondary pneumothorax in a severe emphysema patient. Thorac Cardiovasc Surg 2011;59: 509–510.
Copyright © 2014 by the American Thoracic Society
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