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Background: Esophageal atresia (EA) is most often accompanied by some degree of tracheomalacia (TM), which negatively influences the airway by ineffective clearance of secretions. This can lead to lower airway bacterial colonization (LABC), which may cause recurrent respiratory tract infections (RTIs). This study aims to evaluate the prevalence and specific pathogens of LABC in EA patients., Methods: A 5-year retrospective single-site cohort study was conducted including all EA patients that had undergone an intraoperative bronchoalveolar lavage (BAL) during various routine surgical interventions. Concentrations of greater than 10 cfu were considered evidence of LABC., Results: We recruited 68 EA patients, of which 12 were excluded based on the exclusion criteria. In the remaining 56 patients, a total of 90 BAL samples were obtained. In 57% of the patients, at least 1 BAL sample was positive for LABC. Respiratory symptoms were reported in 21 patients at the time of the BAL, of which 10 (48%) had LABC. Haemophilus influenzae (14%) and Staphylococcus aureus (16%) were most frequently found in the BAL samples. The number of respiratory tract infections and the existence of a recurrent fistula were significantly associated with LABC ( P = 0.008 and P = 0.04, respectively)., Conclusions: This is the first study showing that patients with EA have a high prevalence of bacterial colonization of the lower airways which may be a leading mechanism of severe and recurrent respiratory complications., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Esophageal atresia (EA) is a rare birth defect in which respiratory tract disorders are a major cause of morbidity. It remains unclear whether respiratory tract disorders are in part caused by alterations in airway epithelial cell functions such as the activity of motile cilia. This can be studied using airway epithelial cell culture models of patients with EA. Therefore, the aim of this study was to evaluate the feasibility to culture and functionally characterize motile cilia function in the differentiated air-liquid interface cultured airway epithelial cells and 3D organoids derived from nasal brushings and bronchoalveolar lavage (BAL) fluid from children with EA. We demonstrate the feasibility of culturing differentiated airway epithelia and organoids of nasal brushings and BAL fluid of children with EA, which display normal motile cilia function. EA patient-derived airway epithelial cultures can be further used to examine whether alterations in epithelial functions contribute to respiratory disorders in EA.

Background: Esophageal atresia (EA) is a rare congenital malformation of the esophagus. Surgical treatment is required to restore the continuity of the esophagus. This can be performed through thoracotomy. However, an increasing number of hospitals is performing minimal invasive surgery (MIS). In this article, we describe the technique of thoracoscopic repair of EA in neonates in more detail and show the outcome of a patient cohort operated by young pediatric surgeons in training. Methods: Between 2014 and 2019 correction was performed in 64 EA type C patients at the UMC Utrecht, Wilhelmina Children's Hospital, mainly by young pediatric surgeons in training. Results: All patients were corrected through MIS, 3 days after birth. The median operation duration was 181 (127-334) minutes. Nasogastric tube feeding was started on the first postoperative day, and oral feeding 6 days postop. Postoperative complications included leakage (14.1%), stenosis (51.1%), and recurrent tracheoesophageal fistula (7.8%). Conclusion: Thoracoscopic repair of EA can be performed safely, with good outcome and all the benefits of MIS. However, it remains a challenging procedure and should be performed only in pediatric centers with a vast experience in MIS, especially when training young pediatric surgeons. These centers must have access to a multidisciplinary team of neonatologists, pediatric anesthesiologists, surgeons, and ENT specialists to ensure the best possible care in hemodynamic, respiratory, and cerebral monitoring and gastrointestinal and developmental outcome.

Background: Thoracoscopic external traction technique (TTT) is a relatively new surgical intervention for patients with long-gap esophageal atresia (LGEA) that preserves the native esophagus. The major accomplishment with TTT is that esophageal repair can be achieved within days after birth. This study evaluates the childhood outcome in LGEA patients treated with TTT, including gastrointestinal outcome, nutritional status and Health-Related Quality of Life (HRQoL)., Methods: A cohort study including all LGEA patients that underwent TTT between 2006-2017 was conducted. Patients and/or their parents were invited to fill out questionnaires regarding reflux symptoms and HRQoL., Results: TTT was successful in 11/13 patients (85%). Esophageal anastomosis was accomplished at a median age of 12 days (range 7-138), first oral feeding was started at a median of 16 days postoperatively (range 5-37). All patients required multiple dilatations and 10 patients required anti-reflux surgery. At median follow-up of seven years, five patients reported mild and one moderate reflux complaints. All patients but one reached age-appropriate oral diet. Most patients (80%) were within normal growth range. Overall HRQoL was comparable to healthy controls., Conclusion: TTT provides acceptable results in childhood. Oral feeding can be started as soon as two weeks postoperatively. Almost all patients are able to eat an age-appropriate oral diet. Overall HRQoL was comparable to healthy controls., (Copyright © 2021. Published by Elsevier Inc.)

Background: Esophageal atresia (EA) is often accompanied by tracheomalacia (TM). TM can lead to severe respiratory complaints requiring invasive treatment. This study aims to evaluate if thoracoscopic primary posterior tracheopexy (PPT) can prevent the potential sequelae of TM in patients with EA. Methods: A cohort study including all consecutive EA patients treated between 2014 and July 2019 at the Wilhelmina Children's Hospital was conducted. Two groups were distinguished: (group 1) all EA patients born between January 2014 and December 2016 and (group 2) all EA patients born between January 2017 and July 2019, after introduction of PPT. In the latter group, PPT was performed in EA patients with moderate (33-66%) or severe (67-100%) tracheomalacia, seen during preoperative bronchoscopy. Group differences were assessed using the Fisher's exact test for bivariate variables and the Mann-Whitney U -test for continuous variables. Results: A total of 64 patients were included in this study (28 patients in group 1; 36 patients in group 2). In group 2, PPT was performed in 14 patients. Respiratory tract infections (RTIs) requiring antibiotics within the first year of life occurred significantly less in group 2 (61 vs. 25%, p = 0.004). Brief resolved unexplained events (BRUEs) seemed to diminish in group 2 compared to group 1 (39 vs. 19%, p = 0.09). Conclusion: Thoracoscopic primary posterior tracheopexy decreases the number of respiratory tract infections in EA patients. The clinical impact of reducing RTIs combined with the minimal additional operating time and safety of PPT outweighs the risk of overtreatment., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 van Tuyll van Serooskerken, Tytgat, Verweij, Bittermann, Coenraad, Arets, van der Zee and Lindeboom.)

Hereditary spherocytosis (HS) is the most common form of hereditary chronic hemolytic anemia. It is caused by mutations in red blood cell (RBC) membrane and cytoskeletal proteins, which compromise membrane integrity, leading to vesiculation. Eventually, this leads to entrapment of poorly deformable spherocytes in the spleen. Splenectomy is a procedure often performed in HS. The clinical benefit results from removing the primary site of destruction, thereby improving RBC survival. But whether changes in RBC properties contribute to the clinical benefit of splenectomy is unknown. In this study we used ektacytometry to investigate the longitudinal effects of splenectomy on RBC properties in five well-characterized HS patients at four different time points and in a case-control cohort of 26 HS patients. Osmotic gradient ektacytometry showed that splenectomy resulted in improved intracellular viscosity (hydration state) whereas total surface area and surface-to-volume ratio remained essentially unchanged. The cell membrane stability test (CMST), which assesses the in vitro response to shear stress, showed that after splenectomy, HS RBCs had partly regained the ability to shed membrane, a property of healthy RBCs, which was confirmed in the case-control cohort. In particular the CMST holds promise as a novel biomarker in HS that reflects RBC membrane health and may be used to asses treatment response in HS., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Berrevoets, Bos, Huisjes, Merkx, van Oirschot, van Solinge, Verweij, Lindeboom, van Beers, Bartels, van Wijk and Rab.)

Objective: The objective of this study was to assess the interobserver agreement between on-call radiology residents and radiology specialists in the evaluation of pulmonary embolism (PE) using computed tomographic pulmonary angiography (CTPA)., Materials and Methods: Single center, prospective cohort study of 119 consecutive inpatients and outpatients with clinically suspected PE in whom CTPA was performed during out-of-office hours and weekends. The on-call radiology resident interpreted the CTPA for the presence of PE. An experienced radiology specialist, without knowledge of earlier reading, reviewed the CTPA the following morning. In discordant cases, a consensus reading was conducted. The radiology specialists' assessment and the consensus reading in case of discordance were considered as the gold standard., Results: Five CTPA studies were excluded from the analysis. In the remaining cases, radiology residents and radiology specialists agreed on the interpretations of CTPAs (no PE vs PE present) in 106 (93%) of the 114 cases, with a kappa value of 0.84 (95% confidence interval, 0.74-0.95). The consensus reading equally disagreed with both radiology resident and radiology specialist in 8 (7%) of the 114 discordant cases. Seven out of 8 discordant cases had suboptimal scan quality., Conclusions: The assessment of CTPA in patients with suspected PE by radiology residents seems reliable during out-of-office hours.