Endobronchial valves for treatment of persistent air leak after secondary spontaneous pneumothorax in patients with cystic fibrosis

SESSION TITLE: Great Procedural Cases: Fire, Ice, Struts, Valves, and GlueSESSION TYPE: Case ReportsPRESENTED ON: 10/19/2022 11:15 am - 12:15 pmINTRODUCTION: Secondary spontaneous pneumothoraxes commonly occur in patients with cystic fibrosis (CF) and can be complicated by persistent air leak (PAL) due to bronchopleural or alveolopleural fistula. More recently, bronchoscopic placement of one-way endobronchial valves (EBV) for PAL have been explored. We present the first case series of patients with CF who developed secondary spontaneous pneumothoraxes and were successfully treated with EBV.CASE PRESENTATION: A 30-year-old female with CF (F508del/dupex6B-10) and history of pneumothorax, presented with acute hypoxic respiratory failure. She was found to have a right-sided pneumothorax. A chest tube was placed, with a continuous air leak for 4 days. She was a suboptimal surgical candidate given extensive apical lung disease, making localization of the air leak difficult. In addition, the increased tissue density would have made wedge resection challenging. After multidisciplinary discussion, the patient underwent placement of 5 Zephyr EBV (Pulmonx Inc., Redwood City, CA) for PAL. The lung had re-expanded, but there was still an intermittent air leak. She underwent pleurodesis with betadine. Her chest tube was removed 2 days later, and she was discharged. She was seen in the pulmonary clinic 5 days after being discharged and was noted to have recurrent right pneumothorax. She underwent chest tube placement with flutter valve. The chest tube was removed after 10 days. The patient was scheduled for removal of EBV 6 weeks after placement, but the procedure was delayed to symptomatic COVID-19 infection. EBV were eventually removed 12 weeks after placement. Pneumothorax has not recurred 6 weeks post EBV removal.A 53-year-old female with CF (394delTT/3272-26A-G) and a small right apical secondary spontaneous pneumothorax 3 months prior to hospitalization presented with progressive dyspnea. Imaging showed that the pneumothorax had enlarged. A chest tube was placed with continuous air leak. After a multidisciplinary discussion, 5 Zephyr EBV were placed 2 days later. There was immediate improvement of the pneumothorax, with almost no air leak. Her chest tube was removed 48 hours after placement of EBV, and patient was discharged on day 4. The EBV were removed 8 weeks later with no recurrence of pneumothorax 1 month after valve removal.DISCUSSION: To our knowledge this is the first case series describing the use of Zephyr EBV in CF patients with secondary spontaneous pneumothorax complicated by PAL. Although previous guidelines still recommend surgery and/or pleurodesis for PAL, this may not be the best option for patients with CF who may require lung transplantation. EBV are currently FDA approved for lung volume reduction to treat emphysema, but it is likely a viable non-surgical alternative for PAL.CONCLUSIONS: EBV is a well-tolerated treatment option for PAL due to secondary spontaneous pneumothoraxes.Reference #1: Bongers KS, De Cardenas J. Endobronchial valve treatment of persistent alveolopleural fistulae in a patient with cystic fibrosis and empyema. J Cyst Fibros. 2020 Sep;19(5):e36-e38. doi: 10.1016/j.jcf.2020.03.014. Epub 2020 Apr 18. PMID: 32312675.Reference #2: Travaline JM, McKenna RJ Jr, De Giacomo T, Venuta F, Hazelrigg SR, Boomer M, Criner GJ; Endobronchial Valve for Persistent Air Leak Group. Treatment of persistent pulmonary air leaks using endobronchial valves. Chest. 2009 Aug;136(2):355-360. doi: 10.1378/chest.08-2389. Epub 2009 Apr 6. Erratum in: Chest. 2009 Sep;136(3):950. PMID: 19349382.Reference #3: Dugan KC, Laxmanan B, Murgu S, Hogarth DK. Management of Persistent Air Leaks. Chest. 2017 Aug;152(2):417-423. doi: 10.1016/j.chest.2017.02.020. Epub 2017 Mar 4. PMID: 28267436; PMCID: PMC6026238.DISCLOSURES: No relevant relationships by Bryan Bennno disclosure on file for Julie Biller;No relevant relationships by Rose FrancoSpeaker/Speaker's Bureau relationship with Biodesix Please note: 2018 - present by Jonathan Kurman, value=HonorariaConsultant relationship with Level Ex Please note: 2018 - present by Jonathan Kurman, value=Consulting feeConsultant relationship with Medtronic Please note: 2020 - present by Jonathan Kurman, value=Consulting feeConsultant relationship with Pinnacle Biologics Please note: 2020 - present Added 04/01/2022 by Jonathan Kurman, value=Consulting feeConsultant relationship with Boston Scientific Please note: 2021 - present by Jonathan Kurman, value=Consulting feeConsultant relationship with Cook Medical Please note: 2021 - present by Jonathan Kurman, value=Consulting feeSpeaker/Speaker's Bureau relationship with Erbe Please note: 2021 - present by Jonathan Kurman, value=Honorariaresearch panel relationship with Intuitive Please note: 2020 - present by Jonathan Kurman, value=HonorariaRemoved 04/01/2022 by Jonathan KurmanConsultant relationship with Pulmonx Please note: 2020 - present by Jonathan Kurman, value=Consulting feeTravel relationship with Ambu Please note: 2021-present Added 04/01/2022 by Jonathan Kurman, value=TravelRemoved 04/01/2022 by Jonathan KurmanConsultant relationship with Ambu Please note: 2022-present Added 04/01/2022 by Jonathan Kurman, value=Consulting feeSpeaker/Speaker's Bureau relationship with Veracyte Please note: 2021-present Added 04/01/2022 by Jonathan Kurman, value=HonorariaNo relevant relationships by Shreya Podder SESSION TITLE: Great Procedural Cases: Fire, Ice, Struts, Valves, and Glue SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm INTRODUCTION: Secondary spontaneous pneumothoraxes commonly occur in patients with cystic fibrosis (CF) and can be complicated by persistent air leak (PAL) due to bronchopleural or alveolopleural fistula. More recently, bronchoscopic placement of one-way endobronchial valves (EBV) for PAL have been explored. We present the first case series of patients with CF who developed secondary spontaneous pneumothoraxes and were successfully treated with EBV. CASE PRESENTATION: A 30-year-old female with CF (F508del/dupex6B-10) and history of pneumothorax, presented with acute hypoxic respiratory failure. She was found to have a right-sided pneumothorax. A chest tube was placed, with a continuous air leak for 4 days. She was a suboptimal surgical candidate given extensive apical lung disease, making localization of the air leak difficult. In addition, the increased tissue density would have made wedge resection challenging. After multidisciplinary discussion, the patient underwent placement of 5 Zephyr EBV (Pulmonx Inc., Redwood City, CA) for PAL. The lung had re-expanded, but there was still an intermittent air leak. She underwent pleurodesis with betadine. Her chest tube was removed 2 days later, and she was discharged. She was seen in the pulmonary clinic 5 days after being discharged and was noted to have recurrent right pneumothorax. She underwent chest tube placement with flutter valve. The chest tube was removed after 10 days. The patient was scheduled for removal of EBV 6 weeks after placement, but the procedure was delayed to symptomatic COVID-19 infection. EBV were eventually removed 12 weeks after placement. Pneumothorax has not recurred 6 weeks post EBV removal. A 53-year-old female with CF (394delTT/3272-26A-G) and a small right apical secondary spontaneous pneumothorax 3 months prior to hospitalization presented with progressive dyspnea. Imaging showed that the pneumothorax had enlarged. A chest tube was placed with continuous air leak. After a multidisciplinary discussion, 5 Zephyr EBV were placed 2 days later. There was immediate improvement of the pneumothorax, with almost no air leak. Her chest tube was removed 48 hours after placement of EBV, and patient was discharged on day 4. The EBV were removed 8 weeks later with no recurrence of pneumothorax 1 month after valve removal. DISCUSSION: To our knowledge this is the first case series describing the use of Zephyr EBV in CF patients with secondary spontaneous pneumothorax complicated by PAL. Although previous guidelines still recommend surgery and/or pleurodesis for PAL, this may not be the best option for patients with CF who may require lung transplantation. EBV are currently FDA approved for lung volume reduction to treat emphysema, but it is likely a viable non-surgical alternative for PAL. CONCLUSIONS: EBV is a well-tolerated treatment option for PAL due to secondary spontaneous pneumothoraxes. Reference #1: Bongers KS, De Cardenas J. Endobronchial valve treatment of persistent alveolopleural fistulae in a patient with cystic fibrosis and empyema. J Cyst Fibros. 2020 Sep;19(5):e36-e38. doi: 10.1016/j.jcf.2020.03.014. Epub 2020 Apr 18. PMID: 32312675. Reference #2: Travaline JM, McKenna RJ Jr, De Giacomo T, Venuta F, Hazelrigg SR, Boomer M, Criner GJ; Endobronchial Valve for Persistent Air Leak Group. Treatment of persistent pulmonary air leaks using endobronchial valves. Chest. 2009 Aug;136(2):355-360. doi: 10.1378/chest.08-2389. Epub 2009 Apr 6. Erratum in: Chest. 2009 Sep;136(3):950. PMID: 19349382. Reference #3: Dugan KC, Laxmanan B, Murgu S, Hogarth DK. Management of Persistent Air Leaks. Chest. 2017 Aug;152(2):417-423. doi: 10.1016/j.chest.2017.02.020. Epub 2017 Mar 4. PMID: 28267436; PMCID: PMC6026238. DISCLOSURES: No relevant relationships by Bryan Benn no disclosure on file for Julie Biller; No relevant relationships by Rose Franco Speaker/Speaker's Bureau relationship with Biodesix Please note: 2018 - present by Jonathan Kurman, value=Honoraria Consultant relationship with Level Ex Please note: 2018 - present by Jonathan Kurman, value=Consulting fee Consultant relationship with Medtronic Please note: 2020 - present by Jonathan Kurman, value=Consulting fee Consultant relationship with Pinnacle Biologics Please note: 2020 - present Added 04/01/2022 by Jonathan Kurman, value=Consulting fee Consultant relationship with Boston Scientific Please note: 2021 - present by Jonathan Kurman, value=Consulting fee Consultant relationship with Cook Medical Please note: 2021 - present by Jonathan Kurman, value=Consulting fee Speaker/Speaker's Bureau relationship with Erbe Please note: 2021 - present by Jonathan Kurman, value=Honoraria research panel relationship with Intuitive Please note: 2020 - present by Jonathan Kurman, value=Honoraria Removed 04/01/2022 by Jonathan Kurman Consultant relationship with Pulmonx Please note: 2020 - present by Jonathan Kurman, value=Consulting fee Travel relationship with Ambu Please note: 2021-present Added 04/01/2022 by Jonathan Kurman, value=Travel Removed 04/01/2022 by Jonathan Kurman Consultant relationship with Ambu Please note: 2022-present Added 04/01/2022 by Jonathan Kurman, value=Consulting fee Speaker/Speaker's Bureau relationship with Veracyte Please note: 2021-present Added 04/01/2022 by Jonathan Kurman, value=Honoraria No relevant relationships by Shreya Podder