Abstract
BACKGROUND: Congenital lobar emphysema (CLE) is a rare developmental malformation of the lung but can be associated with high morbidity and mortality. The objective of this study is to review our experience with 45 patients with CLE highlighting clinical features, aspects of diagnosis, and management. METHODS: The medical records of all patients diagnosed with CLE in our center were reviewed. Patients age at the time of diagnosis, sex, clinical presentation, associated anomalies, the lobes affected, treatment modality, and outcome were described. A comparison was made between those who had lobectomy via open thoracotomy and those treated thoracoscopically. RESULTS: From January 2000 to December 2018, a total of 45 infants with CLE were presented to our institution. There were 30 male and 15 female, and the mean age at presentation was 3.35 months. Twenty-five patients presented with respiratory distress. Nine patients presented immediately after birth, and two of them had surgery within the 1(st) week of life. Twenty patients were presented with repeated chest infections. Left upper lobe was affected in 27 patients, right middle lobe in 13, and right upper lobe in 5 patients. Forty-four patients had a lobectomy, and one was managed conservatively. One patient had a postoperative bronchopleural fistula. Nine patients had a thoracoscopic lobectomy, and two of them were converted to open thoracotomy because of persistent air leak. The operative time and hospital stay were nonsignificantly longer in thoracoscopic lobectomy (P = 0.5 and 0.4, respectively). There was no operative mortality in both groups. CONCLUSIONS: CLE is a rare malformation with variable presentation. Infants presenting with respiratory distress or recurrent chest infection should be evaluated for the possibility of CLE. Lobectomy is the treatment of choice, and rarely, the patients may be managed conservatively. Thoracoscopic lobectomy is a safe procedure with the possibility of air leak and conversion to open lobectomy.