Congenital lobar emphysema: a 10-year case series review
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...
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Publicaciones Permanyer
2025-01-01
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| Series: | Portuguese Journal of Pediatrics |
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| author | André Assunção Pedro Miragaia Filipa Flôr-de-Lima Susana Guimarães Gustavo Rocha Catarina Ferraz Inês Azevedo |
| author_facet | André Assunção Pedro Miragaia Filipa Flôr-de-Lima Susana Guimarães Gustavo Rocha Catarina Ferraz Inês Azevedo |
| author_sort | André Assunção |
| collection | DOAJ |
| description | 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.
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| format | Article |
| id | doaj-art-04d61a3e6aec4044b22fc12d6bb02f0c |
| institution | DOAJ |
| issn | 2184-4453 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Publicaciones Permanyer |
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| series | Portuguese Journal of Pediatrics |
| spelling | doaj-art-04d61a3e6aec4044b22fc12d6bb02f0c2025-08-20T02:58:21ZengPublicaciones PermanyerPortuguese Journal of Pediatrics2184-44532025-01-0156110.24875/PJP.23000028Congenital lobar emphysema: a 10-year case series reviewAndré Assunção0Pedro Miragaia1Filipa Flôr-de-Lima2Susana Guimarães3Gustavo Rocha4Catarina Ferraz5Inês Azevedo6Department of Pediatrics, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; PortugalDepartment of Pediatrics, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; PortugalDepartment of Neonatology, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; PortugalDepartment 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. PortugalNeonatal Intensive Care Unit, Neonatology Department, Centro Hospitalar Universitário de São João, Porto, PortugalDepartment of Pediatrics, Unidade Local de Saúde São João, Porto; Department of Gynecology-Obstetrics and Pediatrics, Faculty of Medicine, University of Porto, Porto; PortugalDepartment 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. PortugalIntroduction 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. https://pjp.spp.pt/frame_eng.php?id=120Congenital lobar emphysema. Congenital lung disease. Lung overinflation. Pulmonary emphysema. |
| spellingShingle | André Assunção Pedro Miragaia Filipa Flôr-de-Lima Susana Guimarães Gustavo Rocha Catarina Ferraz Inês Azevedo Congenital lobar emphysema: a 10-year case series review Portuguese Journal of Pediatrics Congenital lobar emphysema. Congenital lung disease. Lung overinflation. Pulmonary emphysema. |
| title | Congenital lobar emphysema: a 10-year case series review |
| title_full | Congenital lobar emphysema: a 10-year case series review |
| title_fullStr | Congenital lobar emphysema: a 10-year case series review |
| title_full_unstemmed | Congenital lobar emphysema: a 10-year case series review |
| title_short | Congenital lobar emphysema: a 10-year case series review |
| title_sort | congenital lobar emphysema a 10 year case series review |
| topic | Congenital lobar emphysema. Congenital lung disease. Lung overinflation. Pulmonary emphysema. |
| url | https://pjp.spp.pt/frame_eng.php?id=120 |
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