TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Bronchopulmonary sequestration (BPS), is defined as a non-functional mass of lung tissue with systemic arterial blood supply, but without normal tracheobronchial communication. BPS often presents in adulthood with recurrent pulmonary infections and even fatal hemoptysis. We present a case of a 53-year-old African American male with large volume hemoptysis while hiking in upstate New York, who was found to have right lower lobe intralobar pulmonary sequestration. CASE PRESENTATION: A 53-year-old African American male with a past medical history of hypertension, hyperlipidemia, and diabetes mellitus presented with a subacute cough for one month and one episode of large volume hemoptysis while hiking. Review of systems was negative for fever, chills, chest pain, pleurisy, history of tuberculosis, weight loss, and anticoagulation use. He admitted to recreational marajuana use, but denied smoking cigarettes. Initially he was tachypneic, saturating 91% on room air, but hemodynamically stable. Computed tomography angiography (CTA) revealed right lower lobe intralobar pulmonary sequestration with internal locules of air suggesting superimposed infection. Flexible bronchoscopy revealed fresh blood in the right mainstem and lower lobe. Thoracotomy was followed by a lower lobectomy, which confirmed the diagnosis as it demonstrated an aberrant arterial supply from the descending thoracic aorta. There was no envelopment of pleura around the lung fed by the large pulsatile artery. DISCUSSION: Pulmonary airway malformations are the most common type of congenital abnormalities of the lower respiratory tract, diagnosed 1 in 10,000 to 35,000 live births [1]. An intralobar sequestration (IS) is located in a normal lobe and an extralobar sequestration (ES) is outside, with both having their own visceral pleura. Hybrid lesions are most common and have features of both. ES presents early with associated congenital malformations, whereas IS presents later with hemoptysis and recurrent pulmonary infections [2]. BPS can appear as other pathologies on CT such as a mass lesion, cyst, cavity lesion and localized emphysema [3,4]. Lobectomy should be prompt to prevent complications, such as fatal hemoptysis [5]. Shorter recovery times have been noted with video-assisted thoracoscopic surgery (VATS) [6,7]. Postoperative complications include empyema, hemoptysis, prolonged air leak, and fistula formation. Berna et al described their surgical approach in 25 patients in 2011, and all patients were doing well at long-term follow-up [7]. CONCLUSIONS: BPS can be fatal despite its rarity and innocent presentation. Radiographic ambiguity and nonspecific symptoms may delay appropriate management. It is imperative to include BPS on the initial query when managing hemoptysis. In the era of COVID-19, where hemoptysis may herald a deadly disease, understanding the broad differential of hemoptysis expedites appropriate management. REFERENCE #1: [1] Durell J, Thakkar H, Gould S, Fowler D, Lakhoo K. Pathology of asymptomatic, prenatally diagnosed cystic lung malformations. J Pediatr Surg. 2016;51(2):231-235. doi:10.1016/j.jpedsurg.2015.10.061 REFERENCE #2: [2] Van Raemdonck D, De Boeck K, Devlieger H, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg. 2001;19(4):388-395. doi:10.1016/s1010-7940(01)00603-0 REFERENCE #3: [3] Wei Y, Li F. Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40(1):e39-e42. doi:10.1016/j.ejcts.2011.01.080[4] Qi W, Zhao J, Shi G, Yang F. Intralobar pulmonary sequestration displayed as localized emphysema on computed tomography image. J Cardiothorac Surg. 2017;12(1):83. Published 2017 Sep 8. doi:10.1186/s13019-017-0646-9[5] Rubin EM, Garcia H, Horowitz MD, Guerra JJ Jr. Fatal massive hemoptysis secondary to intralobar sequestration. Chest. 1994 Sep;106(3):954-5. doi: 10.1378/chest.106.3.954. PMID: 8082388.[6] Sun X, Xiao Y. Pulmonary sequestration in adult patients: a retrospective study. Eur J Cardiothorac Surg. 2015;48(2):279-282. doi:10.1093/ejcts/ezu397[7] Polaczek, M., Baranska, I., Szolkowska, M., Zych, J., Rudzinski, P., Szopinski, J., Orlowski, T., & Roszkowski-Sliz, K. (2017). Clinical presentation and characteristics of 25 adult cases of pulmonary sequestration. Journal of thoracic disease, 9(3), 762–767. https://doi.org/10.21037/jtd.2017.03.107 DISCLOSURES: No relevant relationships by Christian Castaneda, source=Web Response no disclosure on file for Rammohan Gumpeni; No relevant relationships by Sophia Ji, source=Web Response No relevant relationships by Parmjyot Singh, source=Web Response No relevant relationships by Anthony Smith, source=Web Response