Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium

Abstract

The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium. Methods: After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015). Results: Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p<0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p<0.0001). The most common locations for CLE were the left upper (n=24, 45.3%), right middle (n=16, 30.2%), and right upper (n=10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0). Conclusions: Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion.

Congenital lung abnormality quantification by computed tomography: The CLAQ method

“…For the total disease percentage we found a cut-off at 18% for cystic abnormalities (sensitivity 31%, specificity 98%, and accuracy 86%) and 17% for CLO (sensitivity 66%, specificity 89%, and accuracy 86%). Our study cohort was comparable to that in previous studies in terms of distribution of CLA diagnoses, [23][24][25] disease extent, 26,27 development of symptoms, and reason for surgical resection. In our cohort, nearly two-thirds of patients remained asymptomatic, which proportion is comparable to that in previous studies ranging from 55% to 74%.…”

“…35 On the contrary, lesions may also appear larger on CT due to hyperinflation. 24 Identification of specific CT-features may be of value in predicting outcome for prospective studies. To this end, we assessed individual components of the CLAQ method and found that hyperdensity within lesions were associated with an increased probability of becoming symptomatic and having a lower exercise tolerance.…”

Iatrogenic pneumothorax in a 4-week-old girl: new diagnosis of congenital lobar emphysema

“…The progressive air trapping became more clinically apparent by 4 weeks of age with diagnostic radiological findings that were missed at the time. This insidious onset of symptoms of respiratory distress symptoms is in keeping with CLE 8. Patients with CLE normally present with respiratory distress in infancy, hyperinflated chest, compression of healthy lung tissue, cyanosis and bronchial hypoplasia 10…”

“…The diagnosis is usually supported by a chest X-ray demonstrating hyperlucency, ipsilateral atelectasis, mediastinal shift, widened rib space and diaphragmatic flattening 11. It most often occurs in the upper and middle lobes with the left upper lobe having the highest incidence as in this case, with rates reported of 45% and 57% 8 12…”

“…The incidence of CLE is estimated to be one in 20 000–30 000 births 7. One retrospective cohort study including 53 confirmed patients with CLE undergoing surgical management found that 75.5% were not detected antenatally, highlighting the need to consider this diagnosis in patients in the first few days to weeks of life with respiratory distress, as in this case 8. The majority of patients are diagnosed in the first few weeks of life and almost all before 6 months of age 9.…”