Intralobar Bronchopulmonary Sequestration With Unusual Presentation of Recurrent Abdominal Pain

Abstract

SESSION TITLE: Genetic and Developmental Disorders SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Bronchopulmonary sequestration (BPS) is a rare congenital malformation characterized by non-functioning lung tissue that does not communicate with the bronchial tree or pulmonary arteries, but typically has its own arterial supply arising from the aorta. It can be divided into extralobar sequestration (ELS), which is outside of the lung, has its own visceral pleura, and manifests earlier in life; and intralobar sequestration (ILS), which is within the pulmonary lobe, shares a visceral pleura, and often manifests later in life. Usually BPS in patients is unidentified unless infected or diagnosed incidentally on imaging. When symptomatic, patients can present with fever, cough, chest pain or hemoptysis. Here, we report a rare case of a young male who presented with recurrent left upper quadrant (LUQ) pain, diagnosed with ILS. CASE PRESENTATION: A 29 year old male with no significant history presented to ED with recurrent LUQ pain. He had 2 prior ED visits spanning 6 months where no specific diagnosis was made. He denied chest pain, cough, and fever. Vital signs, physical exam, and labs including CBC, LFTs and lipase were unremarkable. Chest x-ray and EKG were within normal limits. On the third ED visit, CT abdomen was performed. This showed no abnormalities in the abdomen but a mass in the left lower lobe, concerning for infection, atelectasis, congenital malformation, or malignancy. To clarify the mass, a CT chest with IV contrast was performed that showed a 6x4x7cm mass in the left lower lobe supplied by an aberrant artery off the descending thoracic aorta, highly suspicious for BPS. Patient was referred to thoracic surgery, had a pre-operative angiogram and embolization of the feeding artery, and underwent left thoracotomy with complete resection of BPS, which resolved his abdominal pain. Pathology confirmed the diagnosis of intralobar sequestration with inflammation. DISCUSSION: BPS is very rare, comprising only 0.15-6.4% of all pulmonary malformations. ELS is often identified on prenatal ultrasound and becomes symptomatic early in life. ILS often presents later during the second decade of life, and is found due to recurrent infections, mimicking pneumonia symptoms. It is typically within the lower lobe, on the left side, involving the posterior basal segment1. However, clinical presentation can vary depending on its type, size, and location, and sometimes presentation can be unusual, making it difficult to diagnose, as seen in our case where inflamed BPS caused diaphragmatic irritation resulting in referred abdominal pain. CONCLUSIONS: We report a rare case of ILS presenting with recurrent abdominal pain later in life. Screening for thoracic etiologies should be considered when no obvious abdominal cause is found to explain for the presenting abdominal pain. Reference #1: Wei Y, Li F. Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur J Cardiothorac Surg. 2011;40(1):39-42. DOI: 10:1016/j.ejcts.2011.01.080. Epub 2011. DISCLOSURE: The following authors have nothing to disclose: David Attalla, Ji Hyun Rhee No Product/Research Disclosure Information