Purpose: Pulmonary hypoplasia remains a major cause of high mortality in congenital diaphragmatic hernia (CDH). Notch1 signaling pathway plays a critical role in alveolar epithelial cells(AECs) differentiation which dominated in the pulmonary hypoplasia of CDH. However, the effect of Notch1 pathway in CDH remains unclear. We design this study to investigate the hypothesis that the aberrant Notch1 pathway leads to the pulmonary dysplasia in nitrofen-induced CDH rat model. Methods: Pregnant rats were designed as follows: Control, CDH, CDH+CUR. Morphological, Immunostaining and qRT-PCR study were performed to determine the expression levels of T1α and TTF1 in AECs and the expression levels of gene including NICD1 and Hes1 associated with Notch1 signaling pathway. Results: Immunostaining of AEC-I marker T1α and AEC-II marker TTF1 showed that the positive area ratio of T1α in CDH group was less in CUR and Control group, TTF1 was larger in CUR and Control than CDH group (p< 0.05). NICD1 and Hes1 in CDH group were lager than CUR and Control group (p< 0.05). qRT-PCR showed that the expression levels of T1α in CDH group was lower than that in CUR and Control group (p< 0.05), and TTF1, NICD1 and Hes1 in CDH group were higher than CUR and Control group (P < 0.05). Conclusion: Aberrant Notch1 signaling pathway and AECs differentiation disorders are involved in the pathogenesis of pulmonary hypoplasia in nitrofen-induced CDH rat model. Our data suggest that overexpressed Notch1 signaling pathway may cause pulmonary hypoplasia in CDH by inhibiting the differentiation of AECs.

Abstract Purpose: To evaluate our learning curve of pediatric totally thoracoscopic lobectomy (TTL), we review the safety and efficiency of our initial experiences with TTL on pediatric patients with congenital lung malformation. Materials and Methods: This was a retrospective study of all the pediatric patients undergoing TTL between March 2011 and January 2017. Cumulative summation (CUSUM) analysis of operative time (OT) was used. Results: One hundred and three patients were retrospectively analyzed and were divided chronologically into two phases, ascending phase (A) and descending phase (B), through CUSUM of OT. Phases A and B comprised 52 and 51 cases, respectively. OT decreased significantly from phases A to B (P < 0.05). No significant differences were observed in the demographic factors (except for age and body weight) and the conversion or the complication rates between the two phases. Six cases were converted to open surgery (5.8%). Four conversions occurred within the first third of the series and two in the last third. There were no mortalities. Conclusions: Repeated standardized training play a role in overcoming the learning curve for totally thoracoscopic lobectomy in children, and CUSUMOT indicates that the learning curve of around 52 cases is required in our institute.

Objectives: Intralobar pulmonary sequestration (ILS) is rare and its optimal clinical management remains ambiguous. This study aimed to introduce our ten-year experience in clinical management of ILS. And the application of our novel surgical method, thoracoscopic anatomical lesion resection (TALR) on ILS was introduced. Materials and Methods: Patients with ILS who received treatment between December 2010 and December 2020 were included in this study, retrospectively. A binary logistic regression model was used to assess risk factors for preoperative symptoms. Intraoperative and postoperative outcomes were compared between the thoracoscopic lobectomy and lung-sparing surgery groups. Results: A total of 112 patients were included in this study. Age and maximum cyst diameter were risk factors for preoperative symptoms. Lung-sparing surgery proved to be safe and feasible with no residual lesions. Conclusions: The overall prognosis of early thoracoscopic surgery for ILS was good. Lung-sparing surgery, especially TALR could be used as a first-line surgery for ILS. It may resolve the long-standing controversy over whether surgery for asymptomatic patients with ILS.

Introduction: It is rarely seen that neonates with congenital lung mass presenting with respiratory distress require emergency intervention. No consensus has been achieved concerning the best policy facing such condition. This study aims to evaluate the efficacy and safety of our strategies in managing neonates with congenital lung mass presenting with respiratory distress. Methods: We retrospectively reviewed the data of five congenital lung mass (CLM) neonates presenting with respiratory distress from April 2020 to April 2022 for whom different strategies were adopted and favorable outcomes were obtained. The relevant data was reviewed and analyzed. Results: All the patients were prenatally diagnosed with CLM, and among them, four neonates with cystic lung masses accepted percutaneous thoracic catheter drainage prior to surgery. The first neonate with macrocystic lung mass experienced prompt open lobectomy due to failure of drainage. The other three patients obtained good drainage of the large air-filled cyst, thus gaining the opportunity for elective thoracoscopic surgery in median 48 d. The fifth patient directly underwent emergency surgery due to the solid nature of the lung lesion. Conclusions: For neonates with cystic lung masses presenting with respiratory distress due to mediastinal compression, percutaneous thoracic catheter drainage and subsequent elective thoracoscopic surgery were superior to emergency thoracotomy in terms of safety and parenchyma-sparing surgery based on the study and literature. For those with solid lung lesions whose CVR value is no less than 1.6, either EXIT surgery or emergency thoracotomy is a reliable strategy depending on the medical resources available.