congenital lung malformations
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Children ◽  
2022 ◽  
Vol 9 (1) ◽  
pp. 50
Author(s):  
Gloria Pelizzo ◽  
Sara Costanzo ◽  
Margherita Roveri ◽  
Giulia Lanfranchi ◽  
Maurizio Vertemati ◽  
...  

Video assisted thoracoscopic surgery (VATS) has been adopted in pediatric age for the treatment of congenital lung malformations (CLM). The success of VATS in pediatrics largely depends on the surgeon’s skill ability to understand the airways, vascular system and lung parenchyma anatomy in CLM. In the last years, virtual reality (VR) and 3-dimensional (3D) printing of organ models and VR head mounted display (HMD) technologies have been introduced for completion of preoperative planning in adult patients. To date no reports about the use of VR HMD technologies in a pediatric setting are available. The aim of this report is to introduce a VR HMD model in VATS procedure to improve the quality of care in children with CLM. VR HMD set-up for planning thoracoscopic surgery was performed in a series of pediatric patients with diagnosis of CLM. The preoperative VR HMD evaluation allowed a navigation into the malformation with the aim to explore, interact, and make the surgeon more confident and skilled to answer to the traps. A development of surgical simulations models and teaching program dedicated to education and training in pediatric VATS is suitable among the pediatric surgery community. Further studies should demonstrate all the benefits of such technology in pediatric patients submitted to VATS procedure.


Author(s):  
Steven C. Mehl ◽  
Walker D. Short ◽  
Austin Kinley ◽  
Tim C. Lee ◽  
Raphael C. Sun ◽  
...  

2022 ◽  
Vol 60 (1) ◽  
pp. 41-54
Author(s):  
Patrick Tivnan ◽  
Abbey J. Winant ◽  
Monica Epelman ◽  
Edward Y. Lee

BMC Genomics ◽  
2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Weili Yang ◽  
Pu Zhao ◽  
Yun Liu ◽  
Ping Cao ◽  
Xiang Ji ◽  
...  

Abstract Objectives To explore the long non-coding RNA (lncRNA) expression pattern of congenital lung malformations on a genome-wide scale and investigate their potential biological function in four subtypes of congenital lung malformations. Methods We obtained both lesions and normal lung control tissues from the patients diagnosed with CPAM-I, CPAM-II, ILS, and ILS-CPAM, and underwent lobectomy (i.e., surgical removal of the whole lobe which contains the localized lesion as well as normal lung tissue). Then, we performed lncRNA transcriptome profiling in these tissues by RNA sequencing (RNA-seq). A comprehensive bioinformatics analysis was conducted to characterize the expression profiles and relevant biological functions and for multiple comparisons of lncRNA expression in the different subtypes of congenital lung malformation tissues. Furthermore, the lncRNA-mRNA co-expression network was constructed, and dysregulated mRNAs were functionally analyzed. Finally, gene set enrichment analysis (GSEA) was used to predict the potential molecular mechanism of the identified lncRNAs. Results A total of 5921 lncRNA transcripts were identified between congenital lung malformations tissues and normal lung control tissues. Compared with normal lung control, 481of these expressed lncRNAs were upregulated and 142 were downregulated in CPAM-I, 91 were upregulated and 14 were downregulated in CPAM-II, 39 were upregulated and 38 were downregulated in ILS, and 201 were upregulated and 38 were downregulated in ILS-CPAM. Unsupervised clustering and principal component analysis of the expressed lncRNAs visualized the differences between normal lung control and different subtypes of congenital lung malformations samples. We also confirmed significant differences in the composition of differentially expressed genes (DEGs) and the differentially expressed lncRNAs (DE lncRNAs) between CPAM-I and other subtypes of congenital lung malformations, as well as in normal lung control tissues, and observed enrichment of DEGs in the regulation of the immune system, cell projection organization, and inflammatory pathways. Finally, we identified the lncRNA FLJ26850 might be related to congenital lung malformations via ZNF473. Conclusions Significant differences in lncRNAs expression patterns were observed between different subtypes of congenital lung malformations and normal control. The lncRNA FLJ26850 might be related to congenital lung malformations via ZNF473.


2021 ◽  
Vol 9_2021 ◽  
pp. 72-80
Author(s):  
Mashinets N.V. Mashinets ◽  
Demidov V.N. Demidov ◽  
Dorofeeva E.I. Dorofeeva ◽  
Podurovskaya Yu.L. Podurovskaya ◽  
Burov A.A. Burov ◽  
...  

2021 ◽  
Vol 11 ◽  
Author(s):  
Sara Patrizi ◽  
Federica Pederiva ◽  
Adamo Pio d’Adamo

Background and ObjectivesThe treatment of asymptomatic patients with congenital pulmonary malformations (CPMs) remains controversial, partially because the relationship between congenital lung malformations and malignancy is still undefined. Change in methylation pattern is a crucial event in human cancer, including lung cancer. We therefore studied all differentially methylated regions (DMRs) in a series of CPMs in an attempt to find methylation anomalies in genes already described in association with malignancy.MethodsThe DNA extracted from resected congenital lung malformations and control lung tissue was screened using Illumina MethylationEPIC arrays. Comparisons between the group of malformed samples or the malformed samples of same histology or each malformed sample and the controls and between a pleuropulmonary blastoma (PPB) and controls were performed. Moreover, each malformed sample was pairwise compared with its respective control. All differentially methylated regions (DMRs) with an adjusted p-value <0,05 were studied.ResultsEvery comparison highlighted a number of DMRs closed to genes involved either in cell proliferation or in embryonic development or included in the Cancer Gene Census. Their abnormal methylation had been already described in lung tumors.ConclusionsMethylation anomalies already described in lung tumors and also shared by the PPB were found in congenital lung malformations, regardless the histology. The presence of methylation abnormalities is suggestive of a correlation between congenital lung malformations and some step of malignant transformation.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Kenan C. Ceylan ◽  
Güntuğ Batihan ◽  
Ahmet Üçvet ◽  
Soner Gürsoy

Abstract Background Congenital lung malformation is an umbrella term and consist of various kind of parenchymal and mediastinal pathologies. Surgical resection is often required for diagnosis and curative treatment. We aimed to review our experience in surgical treatment for congenital lung disease and present the role of minimally invasive surgery. Methods Surgical resections performed for benign lesions of the lung and mediastinum between January 2009 and May 2019 were retrospectively analyzed. Patients who were found to have congenital lung malformation as a result of pathological examination were included in our study. Distribution characteristics of the patients according to congenital lung malformation subtypes, differences in surgical approach and postoperative results were investigated. Results A total of 94 patients who underwent surgical resection and were diagnosed with the bronchogenic cyst, sequestration, bronchial atresia, congenital cystic adenomatoid malformation (CCAM), or enteric cyst as a result of pathological examination were included the study. There were no significant differences between pathological subtypes in the postoperative length of hospital stay and drainage duration however, perioperative complication rate was higher in the sequestration group. In addition, in the first three days postoperatively, the mean pain score was found to be lower in the VATS group compared to thoracotomy. Conclusions Congenital lung malformations consist of a heterogeneous group of diseases and the surgical treatment in these patients can range from a simple cyst excision to pneumonectomy. Video-assisted thoracoscopic surgery should be considered as the first choice in the surgical treatment of these patients in experienced centers.


Author(s):  
D. B. Giller ◽  
I. I. Enilenis ◽  
V. V. Koroev ◽  
O. Sh. Kesayev ◽  
L. P. Severova ◽  
...  

Congenital malformations of the bronchopulmonary system in combination with tuberculosis are extremely rare in practice, but they usually require surgical treatment. This combination often causes diagnostic difficulties.Characteristics of children and research methods. We have analyzed the experience of surgical treatment of a combination of various congenital lung defects and tuberculosis in 20 children. 9 (45%) children had lung sequestration, 4 (20%) children had lung hypoplasia, 5 (25%) children had congenital bronchiectasis, 1 (5%) child had arteriovenous malformation, and 1 (5%) child had a congenital lung cyst. There were 3 segmental resections, 15 lobeand bilobectomies, and 3 pneumonectomies. All surgeries were performed using minimally invasive approaches (VATS).Results. 11 (55%) patients had diagnostic difficulties of concomitant pathology. Only in 5 (25%) cases they found mycobacteria in sputum before surgery. Postoperative study of material from tuberculous lesions helped to establish multidrug resistance of mycobacterium tuberculosis and to change the chemotherapy regimen in 3 (15%) patients. There were no postoperative complications or deaths. All surgeries were effective. Long-term results were studied in 16 (80%) of 20 patients over a period from 1 to 10 years. No cases of reactivation of tuberculosis or disability.Conclusion. Resection surgery for combination of tuberculosis and congenital lung defects allowed to clarify the diagnosis and to achieve a direct effect in all patients by clarifying the drug resistance of mycobacterium tuberculosis and to optimize postoperative chemotherapy.


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