Congenital lobar emphysema. Report of 17 cases.

The aim of this report is to determine frequency and clinical characteristics of Congenital lobar emphysema (CLE) at Children's Hospital of Tunis.

Methods: Cases of CLE managed between January the 1st 1994 until December the 31st 2004 were reviewed.

Results: Amongst 31 cases of cystic pulmonary malformations we report 17 CLE. They were 12 males and 5 females. The mean age at diagnosis was 41/2 months (20 days, 22 months). Symptoms were: progressive respiratory distress (n=11) recurrent attacks of dyspnea (n=5); pulmonary infection (n=1). Chest X ray and CT scans showed hyper aeration of the affected lobes. Three patients had two affected lobes. CLE was associated to bronchogenic cyst (n=2) and to congenital cardiac anomalies (n=3). All patients underwent lobectomy. Post operative course was uneventful in 16 children.

Conclusions: CLE is an uncommon cause of respiratory distress in neonates and infants. CLE is the most common cystic pulmonary malformation in our institution.