Title: A RARE CASE OF INTRALOBAR PULMONARY SEQUESTRATION
Abstract: SESSION TITLE: Fellows Genetic and Developmental Disorders Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Bronchopulmonary sequestration (BPS) is a congenital abnormality wherein a residual mass of non-functioning lung tissue exists within the thorax, with blood supply from the systemic circulation. The two main types are extralobar (EPS) and intralobar (IPS) pulmonary sequestration. IPS is often detected later in life, constitutes 75% of BPS, lacks its own visceral pleura, and is located within a normal lung lobe. One theory of pathogenesis describes formation of excess lung tissue prior to separation of pulmonary and systemic circulations. 50% of adults with IPS are asymptomatic; others may present with recurrent pulmonary infections, exertional dyspnea, hemoptysis, cough, or back pain. Diagnosis is made with CT angiogram, with the primary goal of establishing blood supply originating from systemic vasculature. CASE PRESENTATION: A 28-year-old man presented with two days of hemoptysis. Review of systems was positive for dry cough associated with marijuana use. Upon presentation, vital signs, blood work, and a chest x-ray were normal. A CT scan of the chest revealed a mixed-density lesion abutting the descending aorta in the left lung, with surrounding ground-glass opacities (GGOs). Bronchoscopy was completed by pulmonology which was unremarkable. A CT angiogram (CTA) of the chest was ordered due to suspicion for an arteriovenous malformation versus pulmonary sequestration due to imaging appearance. The CTA showed resolution of the previously noted lesion and GGOs, but the vessel supplying the relevant lung segment appeared to be arising from the systemic circulation. A chest CTA of the aorta was then completed, confirming the diagnosis of IPS. Patient was referred to cardiothoracic surgery for evaluation for surgical resection. DISCUSSION: A recent retrospective review revealed a high rate of missed diagnosis; 62.5% of cases of IPS were diagnosed after surgery. Biopsy is controversial given high risk of bleeding from systemic vasculature. Pulmonary function tests reveal obstruction in about 8% of cases, and obstruction is usually mild to moderate. Complications include hemoptysis (due to high pressure derived from systemic blood supply), hemorrhagic pleural effusion (due to fibrinoid necrosis within sequestered tissue), recurrent infections, and malignancy within sequestered tissue. CONCLUSIONS: Lobectomy is the treatment of choice, currently also recommended in asymptomatic patients to prevent recurrent infections and progressive inflammation. Embolization via coiling has been utilized recently; however, recurrence rate is high. In the future, it may be helpful to establish guidelines for perioperative coiling, close observation of asymptomatic patients, and post-operative surveillance. Reference #1: Alsumrain M, Ryu JH. Pulmonary sequestration in adults: a retrospective review of resected and unresected cases. BMC Pulm Med. 2018 Jun 05;18(1):97. Reference #2: Chakraborty RK, Sharma S. Pulmonary Sequestration. [Updated 2020 Mar 28]. In: StatPearls. Treasure Island (FL): StatPearls Publishing. 2020. Reference #3: Sun X, Xiao Y. Pulmonary sequestration in adult patients: a retrospective study. Eur J Cardiothorac Surg. 2015;48(2):279-282. DISCLOSURES: No relevant relationships by Mohamed Elnaggar, source=Web Response No relevant relationships by Priyanka Teckchandani, source=Web Response