Pediatrics: Fellow Case Report Poster- Pediatrics |

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

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

DISCUSSION: CLE is a rare congenital lung anomaly. Clinical presentation varies from acute respiratory distress to recurrent tachypnea. Chest radiograph, CT and bronchoscopy are usually sufficient for the diagnosis. Lobectomy is the universally accepted mode of treatment for CLE with severe symptoms. Some studies have reported successful conservative treatment in older patients presenting with milder symptoms, provided the patient is observed closely. During observation, if recurrent episodes of respiratory distress or recurrent infections occur, or in this case, chronic respiratory failure and poor weight, lobectomy should be performed

CONCLUSIONS: To our knowledge, this is one of the few cases of CLE that failed conservative management. Surgical intervention should be considered in symptomatic CLE patients and those who failed conservative management.

Reference #1: Chao M,et al.CLE:an Otolaryngologic Perspective.International Journal of Pediatric Otorhinolaryngology(2005)69,549-554

Reference #2: Ulku R,et al.CLE:Differential diagnsosis and therapeutic approach.Pediatrics International(2008)50,658-661

Reference #3: Mei-Zahav,et al Is CLE a surgical disease?Journal of Pediatric Surgery(2006)41,1058-1061

DISCLOSURE: The following authors have nothing to disclose: Wail Tfankji, Wendy Estrellado

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