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Procedure-related registries in general surgical practice offer a platform for prospective trials, the pooling of data, and detailed outcome analysis. Recommendations by the Idea, Development, Exploration, Assessment, and Long-term follow-up (IDEAL) collaboration and Outcome4Medicine have further improved the uniform reporting of complications and adverse events. In the pediatric surgical network, disease-specific registries for rare and inherited congenital anomalies are gaining importance, fostering international collaborations on studies of low-incidence diseases. However, to date, reporting of complications in the pediatric surgical registries has been inconsistent. Therefore, the European Reference Network for Rare Inherited and Congenital Anomalies (ERNICA) recently endorsed the validation of the first severity grading system for children. The planned reform of the European Paediatric Surgical Audit (EPSA) registry, which includes the implementation of the Clavien–Madadi classification, represents a further effort to establish uniform outcome reporting. This article provides an overview of experiences with surgical registries and complication reporting, along with the potential application of this knowledge to future pediatric surgical practice. [ABSTRACT FROM AUTHOR]

Introduction: Previous studies demonstrated a release of toxic metals, e.g. nickel and chromium, from stainless steel bars used for minimally invasive repair of pectus excavatum (MIRPE). In the present study, we investigated the impact of titanium nitride coating on the metal release and exposure of MIRPE patients. Material and methods: We analyzed the courses of nickel and chromium levels in blood, urine and local tissue in patients undergoing MIRPE with a titanium nitride coated pectus bar between 03/2017 and 10/2018. Sample collection was scheduled prior to MIRPE, at defined postoperative time points and at bar removal. Additionally, we evaluated irritative symptoms. Results were compared to a control group who received uncoated stainless steel bars in a previous time period (03/2015–02/2017). Results: 12 patients received coated pectus bars (mean age 15.7 years). The control group included 28 patients. After implantation of a titanium nitride coated bar, significant increase in systemic nickel and chromium levels after one, two and three years was noted. In an interim analysis one year after MIRPE, we observed patients with coated bars to have significantly elevated trace metal values compared to the control group. This elevation persisted throughout the observation period. Tissue metal values were also significantly increased. Irritative symptoms occurred significantly more often in study patients compared to controls (50.0% vs. 14.3%). Conclusions: Coating of pectus bars with titanium nitride failed to reduce metal contamination after MIRPE. Instead, it resulted in a significant increase of trace metal levels after MIRPE, compared to patients with stainless steel bars, which may be explained by wear of the coating and inter-component mobilization processes. [ABSTRACT FROM AUTHOR]

Background: Inconsistent definitions of complications and unexpected events have limited accurate analysis of surgical outcomes. Perioperative outcome classifications currently used for adult patients have limitations when used for children. Methods: A multidisciplinary group of experts modified the Clavien--Dindo classification to increase its utility and accuracy in paediatric surgery cohorts. Organizational and management errors were considered in the novel Clavien--Madadi classification, which focuses on procedural invasiveness rather than anaesthetic management. Unexpected events were prospectively documented in a paediatric surgery cohort. Results of the Clavien--Dindo and Clavien--Madadi classifications were compared and correlated with procedure complexity. Results: Unexpected events were prospectively documented in a cohort of 17 502 children undergoing surgery between 2017 and 2021. The results of both classifications were highly correlated (ρ = 0.95), although the novel Clavien--Madadi classification identified 449 additional events (organizational and management errors) over the Clavien--Dindo classification, increasing the total number of events by 38 per cent (1605 versus 1158 events). The results of the novel system correlated significantly with the complexity of procedures in children (ρ = 0.756). Furthermore, grading of events > grade III according to the Clavien--Madadi classification showed a higher correlation with procedure complexity (ρ = 0.658) than the Clavien--Dindo classification (ρ = 0.198). Conclusion: The Clavien--Madadi classification is a tool for the detection of surgical and non-medical errors in paediatric surgery populations. Further validation in paediatric surgery populations is required before widespread use. [ABSTRACT FROM AUTHOR]

Severity grading systems for complications in surgical patients have been used since 1992. An increasing assessment of these instruments in pediatric surgery is also noticed, without their validation in children. To analyze the current practice, we performed a literature review with focus on the assessment and grading of complications. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Studies reporting on postoperative complications as a primary or secondary endpoint using a severity grading system were included. Definition for simple adverse events, classification systems used, and the time horizon of postoperative documentation were analyzed. A total of 566 articles were screened, of which 36 met the inclusion criteria. About 86.1% of the papers were retrospective and 13.9% prospective analyses. None of the studies were prospective-randomized trials. Twenty (55.6%) studies did not include a definition of adverse events, whereas the remaining 16 (44.4%) showed variations in their definitions. All studies applied the Clavien-Dindo classification, whereas five (13.9%) additionally used the Comprehensive Complication Index. One study compared alternative grading instruments with the Clavien-Dindo classification, without demonstrating the superiority of any classification in pediatric surgery. Twenty-two studies (61.1%) did not report the time horizon of perioperative complication documentation, while 8 studies (22.2%) used 30 days and 6 studies (16.7%) used 3 months of postoperative documentation. Definition and classification of postoperative complications are inconsistent in the pediatric surgical literature. Establishment of a standardized protocol is mandatory to accurately compare outcome data. [ABSTRACT FROM AUTHOR]

Background and Objectives: Appendiceal carcinoids are rare neuroendocrine tumors and mainly found incidentally during histopathological examination following appendectomy. This observational cohort study was performed to determine the prevalence, treatment modalities and outcomes in children diagnosed with an appendiceal carcinoid tumor. Materials and Methods: Data from the largest German statutory health insurance "Techniker Krankenkasse" were analyzed within an 8-year period: January 2010 to December 2012 and January 2016 to December 2020. Patient characteristics, surgical technique, type of surgical department, diagnostic management, and postoperative morbidity were analyzed. Results: Out of 40.499 patients following appendectomy, appendiceal carcinoids were found in 44 children, resulting in a prevalence of 0.11%. Mean age at appendectomy was 14.7 (±2.6) years. Laparoscopic approach was performed in 40 (91%) cases. Right-sided hemicolectomy was performed in 8 (18%) patients. Additional diagnostic work-up (CT and MRI) was recorded in 5 (11%) children. Conclusions: This large nationwide pediatric study shows that 1 in 1000 patients was found to have a neuroendocrine tumor of the appendix (prevalence 0.11%), emphasizing its low prevalence in the pediatric age group. The majority of patients were treated with appendectomy only. However, treatment modalities are still variable. Longer follow-up analyses are needed to evaluate published guidelines and recommendations to aim for a limited surgical approach. [ABSTRACT FROM AUTHOR]

Nowadays laparoscopic interventions enable the collection of resident macrophage populations out of the human cavities. We employed this technique to isolate pleural monocytes/macrophages from healthy young adults who underwent a correction of pectus excavatum. High quality CD14+ monocytes/macrophages (plMo/Mφ) were used for RNA‐sequencing (RNA‐seq) in comparison with human monocyte‐derived macrophages (MDM) natural (MDM‐0) or IL‐4‐polarized (MDM‐IL4). Transcriptome analysis revealed 7166 and 7076 differentially expressed genes (DEGs) in plMo/Mφ relative to natural MDM‐0 and polarized MDM‐IL4, respectively. The gene set enrichment analysis, which was used to compare RNA‐seq data from plMo/Mφ with single‐cell (scRNA‐seq) data online from human bronchial lavage macrophages, showed that plMo/Mφs are characterized by a high expression of genes belonging to the metallothionein (MT) family, and that the expression of these genes is significantly higher in plMo/Mφ than in MDM‐0 or MDM‐IL4. Our results provide additional insights on high MTs‐expressing macrophage subsets, which seem to be present not only in bronchial lavage of healthy adults or in pleural exudates of lung cancer patients but also in pleural fluid of healthy young adults. Macrophage subsets expressing high MTs may have specific roles in lung defense, repair, and homeostasis, and require further investigations. [ABSTRACT FROM AUTHOR]

Background:  Numerous studies from Asian countries, including large collectives, have reported excellent results after laparoscopic resection of choledochal malformation (CM). However, the role of laparoscopic CM resection is still controversial outside Asia. We aimed to analyze the outcome of laparoscopic CM resection in our institution and to compare our outcome with the data reported in the literature.Methods:  All patients who underwent laparoscopic CM resection in our pediatric surgical department from 2002 to 2019 were retrospectively analyzed for surgical details and postoperative complications, which were graded according to the Clavien-Dindo classification. A systematic literature search identified all reports on over 10 cases of laparoscopic pediatric CM resection and surgical details, follow-up, and complication rates were extracted.Results:  Fifty-seven patients (72% female) with a mean age of 3.6 + 4.1 years underwent laparoscopic CM resection in our department. Conversion rate was 30%. Total complication rate was 28%. The rate of major complications (Clavien-Dindo grade III or more) was 16% and included stricture of the biliodigestive or enteric anastomosis (n = 4), adhesive ileus (n = 3), portal vein thrombosis (n = 1), and recurrent cholangitis with consecutive liver transplantation (n = 1). With increasing experience, complication rates decreased. The majority of publications on laparoscopic CM resections originated from Asia (n = 36) and reported on low complication rates. In contrast, publications originating from non-Asian countries (n = 5) reported on higher complications following laparoscopic CM resection.Conclusion:  Our data indicate that laparoscopic CM resection can be safely performed. The learning curve in combination with the low incidence calls for a centralization of patients who undergo laparoscopic CM resection. There seems to be a discrepancy on complications rates reported from Asian and non-Asian countries following laparoscopic CM resection. [ABSTRACT FROM AUTHOR]

Background: Minimally-invasive repair of pectus excavatum (MIRPE) has been shown to be associated with high release of trace metals into patient's body. The aim of our study was to analyze the kinetics of metal contamination after MIRPE and after bar removal. Methods: We prospectively assessed nickel and chromium changes in blood, urine, and local tissue in patients undergoing MIRPE with stainless-steel bar(s). Baseline samples were taken prior to surgery, further samples were taken at six defined time points until 30 months after bar removal. Clinical symptoms were evaluated at the time of every sample collection. Results: 28 patients were included (mean age 16.4 years). At four weeks after MIRPE and persisting up to bar removal, we found significantly elevated trace metal levels in blood and urine. Tissue nickel and chromium levels were significantly elevated at the time of bar removal. After bar removal, the concentration of trace metal in urine and the concentration of chromium in plasma decreased gradually. In contrast, nickel levels in blood further increased. Five patients showed irritative symptoms after MIRPE, all symptomatic patients had elevated metal levels. Conclusions: Following MIRPE, we found a rapid systemic increase of nickel and chromium. Our data indicate that trace metal release could cause irritative symptoms. The prolonged elevated systemic nickel levels beyond bar removal necessitate further investigations of the long-term side effects of MIRPE. [ABSTRACT FROM AUTHOR]

Introduction:  In Germany, pediatric surgery is organized in a decentralized manner. A nationwide clinical registry does not exist. The aim of this research is to analyze the current status of pulmonary surgery in the country in respect of children and adolescents.Materials and Methods:  Claims data have been extracted from two health insurance databases representing 34% of the German population. Operative diagnoses and procedures, as well as patients' characteristics, were recorded from 2016 to 2019.Results:  The incidence of the four index diagnoses investigated matched the current literature in three of four entities. The main indications <8 years were congenital lung malformations (61%) and spontaneous pneumothorax at age 8 to 17 years (77%). Furthermore, 1,668 pediatric pulmonary procedures (0-17 years) were identified (668 pulmonary resections). Two age peaks were identified: 13% of patients were operated on before 1 year old and 50% of patients were between 15 and 17 years old. Video-assisted-thoracoscopic-surgery (VATS) for pulmonary resections was applied in 62% of cases. 96% of pulmonary resections in patients <1 year and 70% of pulmonary resections in children between 1 and 14 years were performed by pediatric surgeons. Of patients between 15 and 17 years old, only 14% were operated on in pediatric surgery. The estimated average pulmonary resection caseload was 3.2 cases per year and per institution for pediatric surgery and 1.5 for adult surgery.Conclusion:  Indications for pulmonary surgery in children arise from rare diagnoses. In total, 66% of lung resections are performed in patients <1 year and >14 years. The majority of lung resections are performed as VATS. Patients ≤14 years are predominantly operated on by pediatric surgeons. The accessibility of pediatric pulmonary surgery is acceptable, but there is a low caseload per center. [ABSTRACT FROM AUTHOR]

Background Postoperative adverse events may be associated with substantial morbidity and mortality. Numerous severity grading systems for these events have been introduced and validated but have not yet been systematically applied in paediatric surgery. This study aimed to analyse the advantages and disadvantages of these classifications in a paediatric cohort. Methods Unexpected events associated with interventional or organizational problems in the department of paediatric surgery during 2017–2020 were prospectively documented daily for all children. Events were classified according to the Clavien–Dindo grading system during monthly morbidity and mortality conferences. All events were also classified according to five additional grading systems: T92, contracted Accordion, expanded Accordion, Memorial Sloan Kettering Cancer Center, and Comprehensive Complication Index (CCI)®. Results Of 6296 patients, 673 (10.7 per cent) developed adverse events and 240 (35.7 per cent) had multiple events. Overall, 1253 adverse events were identified; of these, 574 (45.2 per cent) were associated with surgical or medical interventions and 679 (54.8 per cent) included organizational problems. The grading systems demonstrated high overall correlation (r pears = 0.9), with minor differences in sentinel events. The Clavien–Dindo classification offered the most detailed assessment. However, these details had only limited additional value. The CCI® scores were correlated with other grading systems (r pears = 0.9) and were useful in analysing multiple events within individual patients. Conclusion Grading systems demonstrated similar scoring patterns for minor and sentinel events, with none being superior for unexpected events in children. However, the CCI® can be a major improvement in assessing morbidity in patients with multiple events. Its use is therefore recommended in prospective studies on paediatric surgery. [ABSTRACT FROM AUTHOR]

Introduction:  According to the Declaration of Helsinki, medical research and new therapeutic interventions involving human subjects require prior informed consent and ethical approval. In 2010, 46% of pediatric surgical publications lacked documentation of ethical approval and 84% lacked documentation of informed parental consent with lowest rates of ethical adherence found in articles concerning novel methods. The aim of this study was to investigate whether adherence to ethical standards has improved in pediatric surgical publications.Materials and Methods:  All 3,093 consecutive articles published in Journal of Pediatric Surgery, European Journal of Pediatric Surgery, and Pediatric Surgery International over the last 5 years were systematically reviewed for publications describing novel surgical methods. Novel methods were defined as surgical methods not published before or not considered common practice. The publications were reviewed as to whether ethical approval and informed consent to participate was documented.Results:  In total, 105 articles describing novel surgical methods were identified (61 Journal of Pediatric Surgery, 16 European Journal of Pediatric Surgery, and 28 Pediatric Surgery International). Authors reported on new operative techniques (62%), modified techniques (31%), or use of new materials (7%). Ethical approval was documented in 52% of the articles with almost half reporting approval for retrospective data analysis only but not the application of the novel method. Informed consent was documented in 21% of publications. Complications were reported in 48% of the studies, including recurrences and reinterventions for the unsuccessful novel methods. Two authors reported mortalities due to underlying disease, one of which failed to report prior ethical approval or informed consent.Conclusion:  Adherence to ethical publication principles in pediatric surgery has improved over the last years but is still lacking in many publications. When implementing new methods, prior ethical approval and informed consent and their documentation are mandatory, specifically in the light of potential hazard to patients. [ABSTRACT FROM AUTHOR]

Introduction:  Human peritoneal macrophages are resident in the abdominal cavity where they support the specific microenvironmental regulation. We have previously observed a phenotypic switch of murine macrophages during infancy that was associated with a functional development. To investigate the age related changes in human peritoneal macrophages, we analyzed peritoneal macrophages of children undergoing laparoscopic procedures.Materials and Methods:  Immunologically healthy children who received minimally invasive surgery in our department were included in this study. In all cases, the written consent was obtained. At the beginning of laparoscopy, physiologic NaCl-solution was instilled and manually removed through the umbilical trocar to gain macrophages. Lavage cells were processed for flow cytometry analysis. CD14+ myeloid cells were monitored for specific lineage marker expression.Results:  A total of 21 donors (age: 7 days-18 years) were included and divided into three groups. In all age groups, 97% of myeloid cells expressed CD11b. 70% of these expressed CD14. Three subsets of CD14 cells were detected on the basis of CD14/CD16 expression (CD14 + CD16dim, CD14 + CD16inter, and CD14 + CD16high). In neonates, >80% belonged to the CD14 + CD16high subset, reducing to 30% in adolescents. In none of the cases, the M2 markers CD23 and CD25 were expressed.Conclusion:  This is the first study showing that lineage marker expression of peritoneal macrophages in neonates differs from that in adults. The knowledge about neonatal tissue resident macrophages might help to understand their complex interaction and to use specific macrophage properties for therapeutic approaches. [ABSTRACT FROM AUTHOR]

Introduction:  The pros and cons of video-assisted thoracoscopic versus conventional thoracic surgery in infants and children are still under debate. We assessed reported advantages and disadvantages of video-assisted thoracoscopy in pediatric surgical procedures, as well as the evidence level of the available data.Materials and Methods:  A systematic literature search was performed to identify manuscripts comparing video-assisted thoracoscopic and the respective conventional thoracic approach in classic operative indications of pediatric surgery. Outcome parameters were analyzed and graded for level of evidence (according to the Oxford Centre of Evidence-Based Medicine).Results:  A total of 48 comparative studies reporting on 12,709 patients, 11 meta-analyses, and one pilot randomized controlled trial including 20 patients were identified. More than 15 different types of advantages for video-assisted thoracoscopic surgery were described, mostly with a level of evidence 3b or 3a. Most frequently video-assisted thoracoscopic surgery was associated with shorter hospital stay, shorter postoperative ventilation, and shorter time to chest drain removal. Mortality rate and severe complications did not differ between thoracoscopic and conventional thoracic pediatric surgery, except for congenital diaphragmatic hernia repair with a lower mortality and higher recurrence rate after thoracoscopic repair. The most frequently reported disadvantage for video-assisted thoracoscopic surgery was longer operative time.Conclusion:  The available data point toward improved recovery in pediatric video-assisted thoracoscopic surgery despite longer operative times. Further randomized controlled trials are needed to justify the widespread use of video assisted thoracoscopy in pediatric surgery. [ABSTRACT FROM AUTHOR]

Introduction:  Evidence supports long-term oral antibiotic prophylaxis to prevent cholangitis after Kasai procedure. Data regarding perioperative intravenous prophylaxis are lacking. Ascending pathogens from the intestine are made responsible for recurrent cholangitis. Therefore, we analyzed the flora in the upper jejunum during the Kasai procedure and their potential impact on postoperative cholangitis.Materials and Methods:  In 26 patients, swabs were taken at the bowel prepared for the Roux-en-Y-loop. Our postoperative protocol includes intravenous third-generation cephalosporins for 2 weeks and rectal steroids starting at day 4. Cholangitis was defined as the postoperative reappearance of acholic stools or increase of serum bilirubin in combination with fevers or increase of inflammatory parameters. In this scenario, Tazocin was administered for another 2 weeks.Results:  Swabs remained sterile in nine patients (34.6%). In 17 patients (65.4%), gram-positive and gram-negative pathogens were identified; all belonging to physiological intestinal flora. A total of 96.2% pathogens were covered by the antibiotic prophylaxis. The cholangitis incidence was 55.6% in the sterile cohort, and 23.5% in the gram-positive and gram-negative cohort (p = 0.06). In the cholangitis cohort, no significant differences were detected for the age at Kasai and the pre- and postoperative total bilirubin.Conclusion:  We found that our antibiotic regiment covered bacteria in the upper gastrointestinal (GI) tract in the majority of our patients at the time of Kasai. Nonetheless, a significant proportion of patients developed signs of cholangitis. There was no higher rate of cholangitis in patients with resistant bacteria. Thus, our data do not support the hypothesis of extended postoperative intravenous antibiotics to prevent ascending cholangitis. [ABSTRACT FROM AUTHOR]

Introduction:  In patients with choledochal cysts (CDC), a hyperplasia-dysplasia-carcinoma sequence can lead to biliary tract malignancy. The limited data available suggest that the risk decreases considerably after excision in childhood. We analyzed samples of resected CDC from pediatric patients histologically and performed mutational analysis of the proto-oncogenes KRAS and BRAF as early markers of malignant alteration in cholangiocytes.Materials and Methods:  After institutional review board approval, patients undergoing resection for CDC in our center from 2011 to 2019 were retrospectively identified. Histopathological reports were searched for inflammation and endothelial alteration. Cases with sufficient tissue specimen were tested for KRAS codon 12/13 and BRAF codon 600 mutations by pyrosequencing.Results:  In total, 42 patients underwent resection for choledochal cyst in the study period. Median age at surgery was 2.4 years (range = 18 days-18 years). Histopathological analysis showed no malignancy, but various degrees of inflammation or fibrosis in approximately 50% of the patients and in all age groups. Sufficient tissue for mutation analysis was available for 22 cases, all of which tested negative for KRAS or BRAF mutation.Conclusion:  In our series, chronic inflammatory changes were frequently present in CDC of infants and children. However, the lack of KRAS and BRAF mutations suggests that no malignant changes have been initiated in this group of European patients undergoing early resection. [ABSTRACT FROM AUTHOR]

Introduction:  Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF.Materials and Methods:  The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9.Results:  Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items.Conclusion:  Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care. [ABSTRACT FROM AUTHOR]

Introduction:  In the last three decades, minimally invasive surgery (MIS) has been widely used in pediatric surgery. Meta-analyses (MAs) showed that studies comparing minimally invasive with the corresponding open operations are available only for selected procedures. We evaluated all available MAs comparing MIS with the corresponding open procedure in pediatric surgery.Materials and Methods:  A literature search was performed on all MAs listed on PubMed. All analyses published in English, comparing pediatric minimally invasive with the corresponding open procedures, were included. End points were advantages and disadvantages of MIS. Results of 43 manuscripts were included. MAs evaluating the minimally invasive with the corresponding open procedures were available for 11 visceral, 4 urologic, and 3 thoracoscopic types of procedures. Studies included 34 randomized controlled trials. In 77% of MAs, at least one advantage of MIS was identified. The most common advantages of MIS were a shorter hospital stay in 20, a shorter time to feeding in 11, and a lower complication rate in 7 MAs. In 53% of MAs, at least one disadvantage of MIS was found. The most common disadvantages were longer operation duration in 16, a higher recurrence rate of diaphragmatic hernia in 4, and gastroesophageal reflux in 2 MAs. A lower native liver survival rate after laparoscopic Kasai-portoenterostomy was reported in one MA.Conclusion:  In the available MAs, the advantages of MIS seem to outnumber the disadvantages. However, for some types of procedures, MIS may have considerable disadvantages. More randomized controlled trials are required to confirm the advantage of MIS for most procedures. [ABSTRACT FROM AUTHOR]

Introduction:  Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature.Materials and Methods:  Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9.Results:  Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1-9). Eight of these (62%) did not reach consensus.Conclusion:  Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery. [ABSTRACT FROM AUTHOR]