Congenital lobar emphysema: a 10-year case series review

André Assunção, Department of Pediatrics, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; Portugal
Pedro Miragaia, Department of Pediatrics, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; Portugal
Filipa Flôr-de-Lima, Department of Neonatology, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; Portugal
Susana Guimarães, Department of Pathological Anatomy, Unidade Local de Saúde São João, Porto; Faculty of Medicine, University of Porto, Porto; National Institute of Legal Medicine and Forensic Sciences. Portugal
Gustavo Rocha, Neonatal Intensive Care Unit, Unidade Local de Saúde de São João, Porto, Portugal
Catarina Ferraz, Department of Pediatrics, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; Portugal
Inês Azevedo, Department of Pediatrics, Unidade Local de Saúde São João, Porto;; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; EPI Unit – Public Health Institute, University of Porto, Porto. Portugal

Introduction and objectives: Congenital lobar emphysema (CLE) is a rare congenital lung malformation that causes one or more pulmonary lobes to overinflate as a result of an air trapping-like mechanism and it may cause progressive respiratory distress. It is diagnosed in one in every 20,000 – 30,000 neonates. Diagnosis is usually made postnatally, with most cases identified in the first six months of life, but advances in ultrasound technology have allowed for some prenatal diagnosis. We report on the presentation, diagnostic methods, and management of a series of cases of CLE. Methods: Review of electronic files of patients diagnosed with CLE, over the past 10 years. Results: We identified four patients diagnosed with CLE: three female and one male. Two of these patients were diagnosed with CLE prenatally and remained asymptomatic for the entire recorded period, and the other two developed symptoms soon after birth. Chest radiographic imaging was performed in all four patients and a CT scan was carried out in three cases. The symptomatic cases required lobectomy and follow-up showed a slight decrease in residual volumes, total lung capacity, and forced expiratory volume, with no long-term pulmonary dysfunction. Discussion: Prenatal diagnosis of CLE is becoming more feasible with advances in imaging technology. In our population, we observed an inverted male-to-female ratio. Patients may remain asymptomatic and conservative management is advocated for such cases. When patients start to develop symptoms, there are progressive signs of increasing respiratory distress and, for these cases, surgery is recommended, with open thoracotomy being the preferred approach. Post-surgical complications are minimal and long-term pulmonary function remains stable.

Keywords: Congenital lobar emphysema. Congenital lung disease. Lung overinflation. Pulmonary emphysema.