Creighton University, Omaha, NE
Copyright 2016, American College of Chest Physicians. All Rights Reserved.
SESSION TITLE: Student/Resident Case Report Poster - Genetic and Developmental Disorders
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
INTRODUCTION: Bronchopulmonary sequestration (BPS), a congenital anomaly, is isolated lung tissue receiving arterial blood from systemic vasculature which does not communicate with the tracheobronchial tree (1). Intralobar sequestration (ILS) lacks its own visceral pleura while extralobar sequestration (ELS) retains its own visceral pleura and venous drainage. We describe a case of pneumonia with an infected ILS.
CASE PRESENTATION: A 58 year old female with history of recurrent pneumonias in adolescence presented with recent fevers, sharp right lower back pain and hematuria. Except for tachycardia (103 beats/minute), physical examination and vitals were unremarkable. Laboratory testing revealed a WBC count of 22.4 k/ml (normal 4 - 12 k/ml), procalcitonin of 2.6 ng/mL (normal < = 0.05 ng/mL) and hemoglobin 12.8 mg/dL (normal 12 - 16 mg/dL). A non-contrast abdominal Computed Tomography (CT) scan demonstrated blood clots in the bladder and a cystic, air and fluid filled area in the right lower lobe (RLL), suspicious for an intrapulmonary abscess. Subsequent contrast enhanced chest CT scan revealed the cystic mass was supplied by an anomalous artery from the descending aorta. Also noted was a small ipsilateral pleural effusion and RLL consolidation consistent with pneumonia. Thus an intrapulmonary abscess due to BPS with active infection was diagnosed. The patient was discharged on oral amoxicillin and clavulanate for 2 weeks and referred to cardiothoracic surgery for BPS excision.
DISCUSSION: BPS is extremely rare, comprising 0.15 - 6.4 % of congenital pulmonary anomalies. It is left side predominant. ELS is more common in infancy, presenting as respiratory distress and can rarely be extra-thoracic. ILS is more frequent, presents later in life and often with recurrent infections. These can become cystic, as with our patient. BPS is often missed and goes undiagnosed for decades. It is rarely complicated by hemorrhage or lung cancer. Multidetector CT best delineates airways and parenchyma and is the test of choice to follow BPS; contrast CT or pulmonary angiography best detects it given the hallmark systemic arterial supply. Management of asymptomatic BPS in infants and children is controversial. Some authors advocate excision to prevent complications in adulthood while others recommend close follow up with serial imaging. Surgical excision is the treatment of choice for those with symptoms (1, 2).
CONCLUSIONS: A high index of suspicion is required to evaluate and detect BPS. Initial manifestation of this congenital anomaly in adulthood is not uncommon
Reference #1: Pulmonary sequestration: a comparison between pediatric and adult patients. Van Raemdonck D, De Boeck K, Devlieger H, Demedts M, Moerman P, Coosemans W, Deneffe G, Lerut T. European Journal of Cardio-thoracic Surgery 19 (2001) 388-395.
Reference #2: Conservative management of antenatally diagnosed cystic lung malformations. Ng C, Stanwell J, Burge DM, Stanton MP. Archives of Diseases in Childhood. 2014; 99(5): 432-7.
DISCLOSURE: The following authors have nothing to disclose: Karishma Bhatia, Vishisht Mehta, Dustin McCann, Zachary DePew
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