Congenital lobar emphysema: A diagnostic dilemma with coexistent congenital heart defects

Key clinical message: Clinicians should think beyond pneumonia and left-to-right shunts when young children have persistent respiratory distress. Congenital lung anomalies, including congenital lobar emphysema, should be considered differential diagnoses. Chest X-ray and CT imaging should be conducted to gain insight regarding and establish diagnosis, respectively.

Abstract: Congenital lobar emphysema (CLE) is a rare and life-threatening congenital lung anomaly that often poses a diagnostic dilemma. The issue can be further confused with coexistent congenital heart defects (CHDs) with left-to-right shunts. The clinical presentation of CLE during infancy is similar to that of CHD, with CHD being more common entity, gets detected early. The presence of underlying CLE may only be suspected after CHD repair. In our case, a 2-month-old infant presented with respiratory distress. On evaluation, a large ventricular septal defect and patent ductus arteriosus were detected. After successful cardiac defect repair, the infant continued to experience cough, respiratory distress, and failure to thrive. Furthermore, on radiological reanalysis, CLE was suspected on X-ray and confirmed via computed tomography. CLE can be detected on chest X-rays; however, at an early stage, it is often confusing and misleading.