Journal
PEDIATRIC SURGERY INTERNATIONAL
Volume 38, Issue 4, Pages 559-568Publisher
SPRINGER
DOI: 10.1007/s00383-022-05086-2
Keywords
Congenital lobar emphysema; Flexible bronchoscopy; Lobectomy; Imaging; Congenital pulmonary malformations
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This study retrospectively analyzed the clinical, radiological, and bronchoscopy findings of 20 patients with congenital lobar emphysema. The results showed that patients who underwent surgery were more likely to experience respiratory distress and mediastinal shift, and they were diagnosed at an earlier age compared to those who were conservatively followed up.
Introduction Congenital lobar emphysema (CLE), a rare developmental lung malformation, involves the hyperaeration of one or more lung lobes caused by partial obstruction and occurs at a rate of 1/20,000-30,000 live births. Here, we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated by surgical or non-surgical (conservative) approaches at our center and compared our results with those in the literature. Methods We examined the clinical, radiological, and bronchoscopy findings of 20 patients with CLE aged 0-18 years at our center between 2013 and 2020. In addition, we examined the symptoms and findings recorded during the patients' follow-up in this retrospective descriptive study. Results The median age of 20 patients with CLE at diagnosis was 3.2 years (range 1 day-17 years). Respiratory distress and mediastinal shift were more prominent in patients who underwent surgery, and they were diagnosed at an earlier age compared with patients who were followed up conservatively (p = 0.001, 0.049, 0.001, respectively). Neither the pulmonary lobe involvement nor the bronchoscopy findings were found to be indicative of surgery. Discussion We observed that respiratory distress and mediastinal shift were more prominent in patients with a diagnosis of CLE who underwent surgery compared with patients who were conservatively followed up. Moreover, we observed that those who underwent surgery were diagnosed with CLE at an earlier age. In line with the literature, the pulmonary symptoms and CLE-related imaging findings in our study were reduced during conservative follow-up.
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