Evaluation and Long-Term Follow-Up of Congenital Lobar Emphysema

Document Type : Original Article(s)

Authors

1 Assistant Professor, Department of Surgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.

2 Medical Student, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.

Abstract

Background: Congenital lobar emphysema (CLE) is a malformation of the lung characteristic over distension of the affected lobe. Although surgical removal of the affected lobe is the most commonly accepted form of treatment, detection of mild or even asymptomatic cases is usually followed by conservative method. Infants and children have an excellent response to lobectomy. Mortality is rare in a modern pediatric surgical environment. The risks of lung resection include morbidity related to empyema, infection. The diagnosis is best established by CXR.Methods: This study was performed in Alzahra haspital and we studied all 30 patients with CLE. Data was collected using reliable and valid questionnaire.Finding: According to results of this study, 77% boys and 66% girls had Abnormal bronchial cartilage support pathology. Diagnosis was obtained by CXR. The mean age at diagnosis in girls was 4.7± 1.2 months and in the boys was 7.2 ± 2.3 months. Lobectomy were done for 26(86.7%) patients, 4 patients were followed conservatively. Twenty four (92.3%) patients had only one affected lobe. In the long-term follow up, 5% of boys and 30% of girls had cyanosis. 20% in both girls and boys had pneumothorax after lobectomy but 5% and 20% in boys and girls had infection after lobectomy respectively. None of the patients had tracheobronchial fistula and 20% girls had early fatigue. Three of 4 deaths in this study belonged to the male sex.Conclusion: In this study CLE was with 2:1 male predominance. The age that symptoms developed was lower in girls than boys. The prognosis of CLE was better in boys than girls. Height growth in boys was more than girls. Early fatigue was more common in girls. In our study most of the deaths were in boys. 

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