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Pediatrics: Fellow Case Report Poster- Pediatrics |

Approach to Congenital Lobar Emphysema Presenting With Chronic Respiratory Failure Surgical vs Conservative Treatment

Wail Tfankji, MD; Wendy Estrellado, MD
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Department of Pediatrics, University of Kansas Medical Center, Kansas City, KS


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(4_S):953A. doi:10.1016/j.chest.2016.08.1056
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SESSION TITLE: Fellow Case Report Poster- Pediatrics

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: The rarity of reported cases of congenital lobar emphysema (CLE) makes management of a patient with failed conservative treatment a unique challenge

CASE PRESENTATION: A two-week old previously healthy male initially presented with respiratory distress and central cyanosis. On admission, he developed supraventricular tachycardia. He reverted back to sinus rhythm after 2 doses of Adenosine. Respiratory viral panel and pertussis panel were negative. He remained mildly tachypneic and continued to require ½ LPM of oxygen. Initial CXR (Fig1) showed right upper lobe (RUL) opacity. His serum bicarbonate level continued to rise. Arterial blood gas revealed uncompensated respiratory acidosis. Flexible bronchoscopy showed left main stem bronchus and left upper lobe (LUL) malacia. Computed tomography (CT) of the chest (Fig2) revealed hyper-inflated LUL with compression atelectasis of the RUL. Other work ups include a normal brain MRI, normal alpha 1 anti-trypsin level and a negative sweat test. Surgery recommended conservative management at that time. The patient was re-admitted 45 days later due to acute respiratory failure secondary to human metapneumovirus bronchiolitis. Improvement in ventilation was noted while the patient was on mechanical ventilation; however, evidence of respiratory acidosis was seen after extubation. Upon follow in the Pulmonary and Surgery clinic, the patient continued to show evidence of respiratory acidosis, mild tachypnea, and sub-optimal weight gain. Due to failure of conservative management, a left upper lobectomy was performed. Microsopic findings from the surgical specimen showed uniform overdistension of the alveolar spaces. After the surgery, the patient clinically improved with resolution of tachypnea, normalization of respiratory acidosis and improved weight gain

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