Fatal congenital lobar emphysema in a puerpera: a case report and literature review

Abstract

Background Congenital lobal emphysema (CLE) is a developmental lung abnormality usually diagnosed in the neonatal period and is rarely observed in adults. Adults with CLE are usually asymptomatic and only a small fraction may present with coughing, recurrent pneumonia and respiratory distress. In imaging studies, the most frequently affected lobe of CLE is the left upper lobe, followed by the right middle lobe. However, multilobar involvement with severe mediastinal shift is extremely rare. Case presentation We report a case of fatal CLE in a 28-year-old puerpera with postpartum respiratory failure. Chest computed tomography (CT) revealed emphysema of the right upper, middle and lower lobes resulting in adjacent atelectasis. Hyperinflation of the right upper lobe crossed the midline, leading to a deviation of the mediastinal structure to the left hemithorax and severe compression of the left lung. Conclusions Early and timely diagnosis of CLE with routine follow-up is necessary for patients. CLE, especially with multilobar involvement or mediastinal shift, could be life-threatening and should be promptly and aggressively treated to prevent severe complications.

Congenital Lobar Emphysema Presenting as Respiratory Distress in a Newborn

“…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.…”