B47-20 Endobronchial Valve Placement Followed by Lobectomy for Management of Secondary Spontaneous Pneumothorax With Persistent Air Leak in a Patient With Cystic Fibrosis

Abstract Introduction Secondary spontaneous pneumothorax is a known, potentially life-threatening complication of cystic fibrosis (CF). Endobronchial valve (EBV) placement has emerged as a therapy for pneumothorax with persistent air leak (PAL), but there is limited experience with EBVs in people with CF (pwCF). Lobectomy has separately been described for management of severe localized lung disease in pwCF. Here, we report successful EBV placement followed by lobectomy for the treatment of PAL in a patient with CF and severe localized lung disease. Case A 24-year-old female with CF (genotype 406-1GA / 3849 + 10 kb CT) on tezacaftor/ivacaftor, Mycobacterium avium complex pulmonary disease (MAC-PD), and chronic methicillin-resistant Staphylococcus aureus infection presented with sudden-onset dyspnea and chest pain. Chest radiograph demonstrated a large right-sided pneumothorax. A chest tube was placed (Fig. 1c), revealing a continuous air leak that failed to resolve. A single Zephyr 4.0 EBV (Pulmonx, Redwood City, CA) was placed in the right upper lobe (RUL) posterior segmental bronchus with immediate resolution of the air leak. The chest tube was removed two days later before discharge. She was briefly readmitted one week later due to a CF pulmonary exacerbation (PEx) responsive to antibiotics.Given her extensive RUL disease (Fig. 1a), risk of recurrent pneumothorax, and ongoing infection, definitive surgery was planned. To prevent surgical site infections, she continued therapy for MAC-PD and received four weeks of preoperative tedizolid. Approximately 8 weeks after initial presentation, she underwent uncomplicated EBV removal and right upper lobectomy via video-assisted thoracoscopic surgery. Subsequent imaging demonstrated re-expansion of the right lung (Fig 1d). Despite a small post-operative decrement in lung function, she felt well with preserved exercise tolerance. Discussion To our knowledge, this is the first report of EBV placement followed by planned lobectomy for the management of PAL in a patient with CF. This case supports the utility of EBVs for treatment of PAL in pwCF, including as a bridge to lobectomy, and highlights the importance of multidisciplinary care of pwCF. EBVs should be used cautiously in the setting of pulmonary infection as they can impair regional drainage and potentially provide a nidus for infection. This patient had one PEx after EBV placement, which resolved with antibiotics. Early EBV removal and surgical resection may help mitigate both the risk for subsequent infection as well as pneumothorax recurrence. Further experience with EBVs in pwCF is required to understand if EBV placement may increase the risk of PExs. This abstract is funded by: None