A Four-Week-Old Infant With Respiratory Distress: An Emergency Department Case Presentation of Congenital Lobar Emphysema

Abstract

Congenital lobar emphysema (CLE) and congenital pulmonary lymphangiectasis (CPL) are rare conditions that are most often identified with prenatal ultrasonography. Occasionally, this disease process is first identified in the emergency department (ED), where the physician should avoid common pitfalls in order to prevent acute decompensation. To the best of our knowledge, there are no prior reports in the emergency medicine literature of CLE or CPL presenting to the ED as undifferentiated respiratory distress in an infant. Here, we describe one such case and then discuss the importance of differentiating these congenital anomalies from more commonly encountered emergency diagnoses, such as pneumothorax and pneumonia. Management differs radically, and the use of chest tubes and positive pressure ventilation in CLE may precipitate acute cardiovascular decompensation.

Congenital Lobar Emphysema Presenting as Respiratory Distress in a Newborn

“…It usually manifests within the first six months of birth with non-specific clinical features of dyspnea, tachypnea, wheezing, retractions, or pulmonary infections. Rarely, asymptomatic adults can be diagnosed incidentally [ 3 , 4 ]. A case of CLE in an adult presenting with pneumothorax has also been reported [ 5 ].…”

“…Imaging studies can confirm the diagnosis of CLE before surgery. Overinflation of the affected lobe(s) with or without pulmonary vessel anomalies, atelectasis of the adjacent lobes, and mediastinal shift to the contralateral side are typical findings [ 3 , 8 , 9 ]. A ventilation-perfusion scan is helpful to detect ventilation-perfusion mismatches.…”