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Surgical management of congenital lobar emphysema
R Doğan1, M Demircin, A Sarigül
1Department of Thoracic and Cardiovascular Surgery, Hacettepe University Faculty of Medicine, Ankara.
Insights
Congenital lobar emphysema (CLE) is a rare lung condition in infants. Surgical intervention, specifically lobectomy, is the recommended treatment for severe cases of CLE, offering the best outcomes.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Neonatal Medicine
Background:
- Congenital lobar emphysema (CLE) is a rare congenital lung malformation.
- It can lead to severe respiratory distress in neonates and infants.
- Diagnosis and timely intervention are crucial for patient outcomes.
Purpose of the Study:
- To report surgical outcomes in nine patients with congenital lobar emphysema.
- To review the etiologic factors and clinical presentation of CLE.
- To emphasize the role of surgery as the primary treatment modality.
Main Methods:
- Retrospective review of nine patients diagnosed with CLE over 10 years.
- Analysis of patient demographics, clinical symptoms, diagnostic methods, and surgical procedures.
- Histopathological examination to identify underlying causes.
Main Results:
- Nine patients underwent lobectomy for CLE.
- Symptoms ranged from 26 days to 11 months at diagnosis, with neonatal onset indicating more severe disease.
- Bronchial cartilage dysplasia and bronchial atresia were identified as etiologic factors.
- Two patients required initial tube thoracostomy due to misdiagnosis.
Conclusions:
- Surgery, particularly lobectomy, remains the treatment of choice for symptomatic congenital lobar emphysema.
- Early surgical intervention is vital for managing severe respiratory distress.
- Understanding the underlying pathology is key to effective treatment planning.
Abstract:
Here in nine patients with congenital lobar emphysema who had been treated surgically in the previous 10 years are reported. The ages of the patients at diagnosis ranged from 26 days to 11 months. The six patients whose symptoms started in the neonatal period had more severe dyspnea, cyanosis and respiratory distress. Tube thoracostomy was performed in two of three patients who had been misdiagnosed initially. The affected side was the left upper lobe in five patients, the right upper lobe in three, and the right middle and upper lobes in one patient. Lobectomy was performed in all cases. Dysplasia of the bronchial cartilage was found in six patients and bronchial atresia of the left upper lobe was found in another infant as the etiologic cause of the condition. Although the possibility of conservative management in congenital lobar emphysema has been reported recently, we believe that surgery is the treatment of choice in patients who have persistent or progressive, severe respiratory distress in spite of medical treatment.