Congenital lobar emphysema: differential diagnosis and therapeutic approach

Pediatr Int. 2008 Oct;50(5):658-61. doi: 10.1111/j.1442-200X.2008.02630.x.


Background: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respirator distress and pulmonary lobar hyperinflation. It is commonly confused with pneumothorax. The aim of the present paper was to review the authors' experience in order to emphasize the importance of differential diagnosis with pneumothorax.

Methods: Children with CLE treatment at Department of Thoracic Surgery, Dicle University School of Medicine, Turkey, between January 1993 and June 2004, were reviewed.

Results: Ten children consisting of six boys and four girls (age range, 6 h-12 months) had CLE. Major presenting symptoms were tachypnea(n = 100%) and respiratory distress in (n = 80%). On chest radiograph, emphysema was seen in all patients, and shift-herniation to the opposite lung, atelectasis were observed. Computed tomography was performed in all patients, which indicated emphysema in the affected lobes in all cases. Pulmonary perfusion scan was performed in two patients, showing loss of perfusion in the affected lobe. The most common affected lobe was the left upper lobe (50%). In the present series, three patients were mistakenly diagnosed as pneumothorax and intercostal drains were inserted in the emergency department. Eight patients underwent lobectomy, and postoperative course was uneventful. Two patients were followed conservatively. Emphysema was detected in all pathological specimens. One patient was lost to follow up. Mean follow-up duration of all patients was 26.8 +/- 29.24 months (range, 1-89 months).

Conclusions: CLE is established on combined clinical, radiological and scintigraphic imaging. Surgical excision of the affected lobe is the appropriate treatment. Particularly, differential diagnosis should be made between CLE and pneumothorax.

MeSH terms

  • Diagnosis, Differential
  • Emphysema / congenital*
  • Emphysema / diagnosis*
  • Emphysema / surgery
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Lung / diagnostic imaging
  • Male
  • Perfusion Imaging
  • Pneumonectomy
  • Pneumothorax / congenital*
  • Pneumothorax / diagnosis*
  • Respiratory Distress Syndrome, Newborn / diagnosis
  • Respiratory Distress Syndrome, Newborn / etiology
  • Respiratory Distress Syndrome, Newborn / surgery
  • Retrospective Studies
  • Tomography, X-Ray Computed
  • Treatment Outcome