CRITICAL PRESENTATION OF A BRONCHOPULMONARY SEQUESTRATION IN ADULTHOOD

Abstract

SESSION TITLE: Medical Student/Resident Genetic and Developmental Disorders Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Congenital pulmonary anomalies are typically found at birth due to neonatal symptoms or imaging. Bronchopulmonary sequestrations (BPS) are rare, congenital, nonfunctioning masses of pulmonary tissue that lacks airway communication to the main bronchial tree and are supplied by systemic circulation [1]. Although most of these patients are identified at birth, some can live into adulthood without knowledge. In this case, we will discuss the rare appearance and complications of BPS in adult medicine and the steps for curative treatment. CASE PRESENTATION: A 28-year-old healthy female presented with sudden onset hematemesis and hemoptysis. On arrival, she was in hypovolemic shock with systolic blood pressure in the 50s. Patient received aggressive fluid resuscitation. Hemoglobin was stable despite blood loss. CT chest showed a 16 x 9cm bronchopulmonary sequestration in the right base. Patient was emergently intubated and underwent bronchoscopy showing normal mucosa without evidence of active bleed. A second bronchoscopy was performed to place a bronchial blocker to prevent further suspected bleeding into the right lower lung. Cardiothoracic surgery recommended transfer to University Hospital. After transfer, patient underwent repeat bronchoscopy to reposition the bronchial balloon. Interventional radiology embolized the right bronchial artery and two segmental branch arteries from the right inferior phrenic artery, all supplying the BPS. Thoracic surgery delayed resection due to severe aspiration pneumonitis. Patient was discharged and completed a course of antibiotics. She later underwent right muscle sparing thoracotomy with total resection of the BPS and therapeutic right lower lobe wedge resection (Figure 1). She gradually recovered inpatient and was discharged. She followed up with thoracic surgery without any symptoms. DISCUSSION: Although a rare disorder (1 in 10-35,000 live births), BPS typically presents as an incidental finding in a neonate. In adulthood, diagnosis is made through CT angiography of the chest showing systemic circulation supplying an aberrant lung tissue. Definitive diagnosis is made after resection of the tissue [1]. Treatment involves elective surgery in symptomatic patients to remove the BPS with or without partial lobectomy or wedge resection, such as in this case. In asymptomatic patients, risk assessment is evaluated. Patients with large lesions > 20% of the thorax or with characteristics of pleuropulmonary blastoma should have resection [1]. Low risk asymptomatic patients can be monitored via chest x-ray and CT chest. Patients may, however, still choose to proceed with elective surgery to prevent future complications that may cause the surgery to become urgent. CONCLUSIONS: If detected early, removal of BPS can prevent critical events. Rare, undetected BPS can suddenly appear in adulthood requiring stabilization prior to removal as curative treatment. Reference #1: Oermann, Christopher M, et al. “Bronchopulmonary Sequestration.” UpToDate, 2020, www.uptodate.com/contents/bronchopulmonary-sequestration?search=pulmonary%2Bcongenital%2Banomalies&topicRef=6346&source=related_link DISCLOSURES: No relevant relationships by Kalpan Desai, source=Web Response No relevant relationships by Vishal Jivan, source=Web Response No relevant relationships by Jeremy Renz, source=Web Response