SESSION TITLE: Bronchology/Interventional Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Congenital anomalies of lower respiratory tract are rare. Bronchopulmonary sequestration (BPS) is estimated to comprise 0.15 to 6.4% of congenital pulmonary malformations, making it an extremely rare disorder. In several reports, even tertiary care referral centers diagnose less than one case per year. We report a rare congenital malformation known as BPS in an uncommon age range, middle aged adult
CASE PRESENTATION: We present a 37-year-old caucasian male with no significant past medical history who was first evaluated for abdominal discomfort. CT abdomen during the initial work up was notable for a left lower lobe mass which was followed over two years with serial CT chest exams. While the patient never reported respiratory symptoms, there was interval growth in the mass from 2 cm to 3.5 cm requiring further evaluation. PET scan was notable for hyper metabolic activity within the left lower lobe mass. Suspicion arose for malignancy, AVM, granulomatous disease, and fungal infection. Subsequent bronchial biopsy and brushings were non diagnostic and a VATS was scheduled for pathologic diagnosis. A left thorascopic resection of the mass was performed (Figure 1) and notably, there was an artery originating from the descending aorta that was traveling through the inferior pulmonary ligament and entering the mass in the left lower lobe (Figure 2), defining an intralobar intrapulmonary sequestration. Pathology revealed extensive inflammation with no evidence of malignancy.
DISCUSSION: The term sequestration, first used in 1946 by Pryce, refers to an abnormality in which a segment of bronchopulmonary tissue has an anomalous systemic arterial blood supply . Two types have been described. Intralobar sequestration, which accounts for 75% of cases, is a segment of lung contained within the normal visceral pleura. In contrast, in extralobar sequestration the bronchopulmonary tissue is outside the visceral pleura and may be enveloped in its own pleura. The arterial supply derives from the aorta, predominantly the descending thoracic aorta (75%) and less frequently the abdominal aorta. Intralobar sequestration is typically diagnosed in pediatric or adolescent patients, at age 20 years or younger in approximately 50% to 60% of cases, and it is rarely found in patients older . Here, we present a confirmed case of intralobar BPS in a 37 year old adult patient.
CONCLUSIONS: Due to the small prevalence and often asymptomatic presentation of this disease entity, one must have a heightened awareness of this process even in older adults. While it was not the case in our patient, this entity may be the unrecognized cause of recurrent pneumonias, abscesses, hemoptysis, and heart failure from persistent left-to-right shunting.
Reference #1: Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of lung: report of seven cases. J Pathol Bacteriol.1946;58:457-467
Reference #2: Gustafson, et al. Intralobar sequestration: a missed diagnosis. Ann Thorac Surg. 1989;47:841-84
DISCLOSURE: The following authors have nothing to disclose: Anita Rajagopal, Edward Mintz, Anthony Ascioti
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