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Purpose: The purpose of this study was to understand the provision and distribution of esophageal atresia (EA) follow-up (FU) and transition services across European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) member and affiliate centers., Methods: A REDCap questionnaire was sent to clinical leads of 18 ERNICA members and 14 affiliate centers., Results: 29 of 32 centers responded (91%), the majority of which were highly specialized. Two-thirds had a dedicated EA clinic with a specialist multi-disciplinary team (MDT), offered to selected/complex patients only in 40% of centers. ERNICA centers were more likely to offer an MDT FU clinic than affiliates, with lack of resources most cited as a barrier to uptake (67%). Delivery of routine investigations was heterogeneous, particularly provision of three endoscopies over the course of FU (24%). Only 55% had a dedicated transition pathway, more prevalent in ERNICA centers (81% vs. 30%; p < 0.01). Self-reported awareness of ERNICA and European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) guidance for FU and transition was poor (28%)., Conclusion: Despite the existence of European follow-up and transition guidelines, their delivery is not uniform and may be limited by lack of awareness of the guidelines and a lack of resources., (© 2024. The Author(s).)

Purpose: The STAT trial is a multicenter randomized controlled trial in 12 centers worldwide aiming to determine the most effective operation for neonates with necrotizing enterocolitis (NEC) requiring intestinal resection: stoma formation (ST) or primary anastomosis (PA)., Methods: Infants having a primary laparotomy for NEC were randomized intraoperatively to PA or ST if the operating surgeon thought that both were viable treatment options for that patient. The primary outcome (duration of parenteral nutrition [PN]) was evaluated by Cox regression., Results: Eighty patients were recruited from 2010 to 2019. Infants undergoing anastomosis finished PN significantly earlier than patients undergoing stoma (hazard ratio PA vs. ST 2.38, 95% CI 1.36-4.12 p = 0.004). There was no difference in mortality between the two groups (PA 4/35 vs. ST 8/38 p = 0.35) or in the rate of complications requiring further unplanned operations (p = n.s.). Multiple intestinal complications were more frequent in the stoma group compared to the anastomosis group (ST 12/26 vs. PA 5/31, p = 0.02, Fisher's Exact test)., Conclusion: At laparotomy for NEC, when there is no disease distal to resected intestine, primary anastomosis should be performed as it enhances the recovery from NEC, reduces the risk of multiple intestinal complications and does not increase adverse outcomes., (© 2024. The Author(s).)

Objective: We aimed to determine if the incidence of lower respiratory tract infections (LRTI) among children with asymptomatic, observationally managed congenital lung abnormalities (CLA) differed from that of symptomatic patients who underwent surgery. Second, we sought to compare the pre- and post-resection incidence of LRTI in patients who underwent surgery., Methods: This retrospective cohort study included patients born between 1999 and 2021 with CLA confirmed by CT scan who were enrolled in a prospective longitudinal follow-up program. The LRTI incidence rates at 1, 2, 5, 8, and 12 years were compared between surgically and observationally managed patients using incidence rate ratios (IRR). Differences in pre- and post-resection LRTI incidence rates among patients who underwent CLA-related surgery were assessed through IRR., Results: Among 217 included patients, 81 (37%) had undergone surgery and 136 (63%) had been observationally managed. The LRTI incidence rates did not significantly differ at any follow-up moment between the surgical and observational groups. Among the children who underwent CLA-related surgery, the pre-resection LRTI incidence rates were significantly higher than the post-resection LRTI incidence rates (IRR of 3.57, 95% confidence interval: [2.00; 6.33], p < .001)., Conclusion: We could not demonstrate differences in LRTI incidence throughout childhood between patients with surgically and observationally managed CLA. We recommend discussing cases of LRTI in patients with CLA in a multidisciplinary setting, using additional diagnostics such as chest X-ray to screen for CLA involvement, enabling a well-considered decision on surgical resection of the lesion., (© 2024 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Objective:  Survival of neonates with esophageal atresia (EA) is relatively high and stable, resulting in increased attention to optimizing care and longer-term morbidity. This study aimed to reach consensus on a quality indicator set for benchmarking EA care between hospitals, regions, or countries in a European clinical audit., Methods:  Using an online Delphi method, a panel of EA health care professionals and patient representatives rated potential outcome, structure, and process indicators for EA care identified through systematic literature and guideline review on a nine-point Likert scale in three questionnaires. Items were included based on predefined criteria. In rounds 2 and 3, participants were asked to select the five to ten most essential of the included indicators., Results:  An international panel of 14 patient representatives and 71 multidisciplinary health care professionals representing 41 European hospitals completed all questionnaires (response rate: 81%), eventually including 22 baseline characteristics and 32 indicators. After ranking, 10 indicators were prioritized by both stakeholder groups. In addition, each stakeholder group highly prioritized one additional indicator. Following an additional online vote by the other group, these were both added to the final set., Conclusion:  This study established a core indicator set of twenty-two baseline characteristics, eight outcome indicators, one structure indicator, and three process indicators for evaluating (quality of) EA care in Europe. These indicators, covering various aspects of EA care, will be implemented in the European Pediatric Surgical Audit to enable recognition of practice variation and focus EA care improvement initiatives., Competing Interests: None declared., (Thieme. All rights reserved.)

Background:  Potential underlying genetic variations of pectus excavatum (PE) are quite rare. Only one-fifth of PE cases are identified in the first decade of life and thus are of congenital origin. The objective of this study is to test if early-onset PE is more likely to be part of genetic variations than PE that becomes apparent during puberty or adolescence., Materials and Methods:  Children younger than 11 years who presented with PE to the outpatient clinic of the Department of Pediatric Surgery at our center between 2014 and 2020 were screened by two clinical geneticists separately. Molecular analysis was performed based on the differential diagnosis. Data of all young PE patients who already had been referred for genetic counseling were analyzed retrospectively., Results:  Pathogenic genetic variations were found in 8 of the 18 participants (44%): 3 syndromic disorders (Catel-Manzke syndrome and two Noonan syndromes), 3 chromosomal disorders (16p13.11 microduplication syndrome, 22q11.21 microduplication syndrome, and genetic gain at 1q44), 1 connective tissue disease (Loeys-Dietz syndrome), and 1 neuromuscular disorder (pathogenic variation in BICD2 gene)., Conclusion:  Early-onset PE is more likely to be part of genetic variations than PE that becomes apparent during puberty or adolescence. Referral for genetic counseling should therefore be considered., Trial Registration:  NCT05443113., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Introduction:  The aims of this survey were to assess the experiences of the transition from in-person to virtual meetings among European pediatric surgeons since the beginning of the coronavirus disease 2019 (COVID-19) pandemic and to identify preferences for future formats., Materials and Methods:  An online questionnaire was circulated among members of the European Reference Network for rare Inherited and Congenital Anomalies Network (ERNICA) in 2022. Two time periods were compared (3 years before the COVID-19 pandemic vs. the year 2021)., Results:  A total of 87 pediatric surgeons from 16 countries completed the survey. In addition, 27% of respondents were trainees/residents and 73% consultants/lead surgeons. Consultants participated in significantly more in-person congresses prior to the COVID-19 pandemic compared with trainees (5.2 vs. 1.9; p  < 0.001). A significant increase in attendance of virtual meeting was reported during 2021 compared with pre-COVID-19 (1.4 vs. 6.7; p  < 0.001). Consultants had experienced significantly less absenteeism thanks to virtual meetings compared with trainees (42/61 vs. 8/23; p  < 0.05). Most surgeons considered virtual meetings more economic (82%), practical (78%), and family-friendly (66%). However, a majority reported missing social events (78%). The communication among attendees or between attendees and speakers or scientific faculty was deemed worse. A minority (14%) experienced equalized representation of trainees and consultants at virtual meetings. Most respondents (58%) agreed that future meeting strategies should focus on offering virtual formats. Regarding future congresses, respondents prefer hybrid (62%) over in-person (33%) or virtual (6%) formats., Conclusion:  According to European pediatric surgeons, virtual learning formats hold multiple advantages and should be continued. Improved technology must meet the challenges, particularly to enhance communication, equal representation, and networking between attendees., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD., Methods: A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs. Recruitment of centres was done by the ERNs and EUPSA, using mailing lists and social media accounts. Only descriptive statistics were reported., Results: In total, 82 centres from 21 different countries entered the survey. Approximately half of them were ERN network members. Seventy-two centres (87.8%) had a self-reported area of expertise for both ARM and HD. Specific TOC programs were installed in 44% of the centres and AC programs in 31% of these centres. When comparing centres, wide variation was observed in the content of the programs., Conclusion: Despite the awareness of the importance of TOC and AC programs, these programs were installed in less than 50% of the participating centres. Various transition and AC programs were applied, with considerable heterogeneity in implementation, content and responsible caregivers involved. Sharing best practice examples and taking into account local and National Health Care Programs might lead to a better continuation of care in the future., Level of Evidence: III., Competing Interests: Conflict of interest The authors declare that they have no competing interests., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon., (© 2023. Springer Nature Limited.)

Introduction: Clarifying the effect of music on pain endurance in an experimental design could aid in how music should be applied during both surgical and non-surgical interventions. This study aims to investigate the effect of music on pain endurance and the involvement of the sympathetic adrenomedullary axis (SAM) and the hypothalamic-pituitary-adrenocortical axis (HPA)., Materials and Methods: In this randomized controlled trial all participants received increasing electric stimuli through their non-dominant index finger. Participants were randomly assigned to the music group (M) receiving a 20-minute music intervention or control group (C) receiving a 20-minute resting period. The primary outcome was pain endurance, defined as amount milliampere tolerated. Secondary outcomes included anxiety level, SAM-axis based on heart rate variability (HRV) and salivary alpha-amylase, and HPA-axis activity based on salivary cortisol., Results: In the intention-to-treat analysis, the effect of music on pain tolerance did not statistically differ between the M and C group. A significant positive effect of music on pain endurance was noted after excluding participants with a high skin impedance (p = 0.013, CI 0.35; 2.85). Increased HRV was observed in the M-group compared to the C-group for SDNN (B/95%CI:13.80/2.22;25.39, p = 0.022), RMSSD (B/95%CI:15.97/1.64;30.31, p = 0.032), VLF (B/95%CI:212.08/60.49;363.67, p = 0.008) and HF (B/95%CI:821.15/150.78;1491.52, p = 0.0190). No statistical significance was observed in other secondary outcomes., Conclusions: The effect of the music intervention on pain endurance was not statistically significant in the intention-to-treat analysis. The subgroup analyses revealed an increase in pain endurance in the music group after correcting for skin impedance, which could be attributed to increased parasympathetic activation., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction:  Sufficient training is needed to acquire and retain the procedural skills needed for the surgical correction of congenital anatomical malformations. This study aims to assess the opinions of trainees and pediatric surgeons on the use of simulation-based continued at-home training, which can help to acquire these skills., Methods:  This study consisted of two parts. First, an international survey among trainees and pediatric surgeons assessed their opinions on simulation-based at-home training for pediatric surgical procedures (5-point Likert scale). Second, participants of pediatric colorectal courses were instructed to practice the posterior sagittal anorectoplasty procedure at-home on a simulation model and, subsequently, complete a questionnaire regarding their opinions on continuous at-home training (5-point Likert scale)., Results:  A total of 163 participants (83% pediatric surgeons) completed the international survey (response rate 43%). Overall, participants considered the training useful for both laparoscopic (mean 4.7) and open procedures (mean 4.2) and agreed that it may be used at home after a hands-on course (mean 4.3).Twenty participants completed the questionnaire on continued training (response rate 36%). All agreed that at-home training was of added value (mean 4.5) and that the skills were transferable to the clinical setting (mean 4.3). At-home training was regarded a suitable exercise after a hands-on workshop (mean 4.3), but less so without a workshop (mean 3.7, p =0.017)., Conclusion:  Participating trainees and pediatric surgeons were of the opinion that simulation models and at-home training have added value. This implies that simulation-based training may be used more often in pediatric surgical training., Competing Interests: None declared., (Thieme. All rights reserved.)

This study aimed at evaluating how transition of care is currently being organized in the European Reference Networks (ERNs) health care providers (HCPs) in pediatric areas and in the Anorectal Malformation Network (ARM-Net) Consortium hospitals. An online questionnaire was sent to a total of 80 surgeons, members of or affiliated members of three networks: ARM-Net Consortium, ERN eUROGEN, and ERN ERNICA. Complete information were obtained for 45 HCPs, most of which deal with transition and still see a few adult patients (ca. 10%). Gynecological, gastroenterological, urological, colorectal, and continence issues were the major problems described by adult patients to their physicians, and in line with these prevalent complaints, they are referred to the appropriate adult specialists. Forty percent of patients complain about sexual and fertility problems, but the percentage of andrologists and sexologists involved in the caring of adult patients with ARM/Hirschsprung's disease is low, just above 10.9%. Most hospitals deal with transition, but three basic criteria (i.e., presence of: [1] an official written transitional program, [2] a transitional coordinator, and [3] written information on transition to be handled to patients) are jointly met only by six HCPs. According to the responders, the most important issue requiring improvement is the lack of interest and of specific preparation by adult specialists. The overall results of this exploratory survey confirm the need for the development of comprehensive programs for transition in these rare and complex diseases, and identify the hospitals that, in collaboration with the networks, could share best practices in organizing structured transitional pathways and well follow-ups., Competing Interests: None declared., (Thieme. All rights reserved.)

The importance of multidisciplinary long-term follow-up for adults born with esophageal atresia (EA) is increasingly recognized. Hence, a valid, condition-specific instrument to measure health-related quality of life (HRQoL) becomes imperative. This study aimed to develop and validate such an instrument for adults with EA. The Specific Quality of life in Esophageal atresia Adults (SQEA) questionnaire was developed through focus group-based item generation, pilot testing, item reduction and a multicenter, nationwide field test to evaluate the feasibility, reliability (internal and retest) and validity (structural, construct, criterion and convergent), in compliance with the consensus-based standards for the selection of health measurement instruments guidelines. After pilot testing (n = 42), items were reduced from 144 to 36 questions. After field testing (n = 447), three items were discarded based on item-response theory results. The final SQEA questionnaire (33 items) forms a unidimensional scale generating an unweighted total score. Feasibility, internal reliability (Cronbach's alpha 0.94) and test-retest agreement (intra-class coefficient 0.92) were good. Construct validity was discriminative for esophageal replacement (P < 0.001), dysphagia (P < 0.001) and airway obstruction (P = 0.029). Criterion validity showed a good correlation with dysphagia (area under the receiver operating characteristic 0.736). SQEA scores correlated well with other validated disease-specific HRQoL scales such as the GIQLI and SGRQ, but poorly with the more generic RAND-36. Overall, this first condition-specific instrument for EA adults showed satisfactory feasibility, reliability and validity. Additionally, it shows discriminative ability to detect disease burden. Therefore, the SQEA questionnaire is both a valid instrument to assess the HRQoL in EA adults and an interesting signaling tool, enabling clinicians to recognize more severely affected patients., (© The Author(s) 2022. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction:  As survival rates of infants born with esophageal atresia (EA) have improved considerably, research interests are shifting from viability to morbidity and longer-term outcomes. This review aims to identify all parameters studied in recent EA research and determine variability in their reporting, utilization, and definition., Materials and Methods:  Following PRISMA guidelines, we performed a systematic review of literature regarding the main EA care process, published between 2015 and 2021, combining the search term "esophageal atresia" with "morbidity," "mortality," "survival," "outcome," or "complication." Described outcomes were extracted from included publications, along with study and baseline characteristics., Results:  From 209 publications that met the inclusion criteria, 731 studied parameters were extracted and categorized into patient characteristics ( n  = 128), treatment and care process characteristics ( n  = 338), and outcomes ( n  = 265). Ninety-two of these were reported in more than 5% of included publications. Most frequently reported characteristics were sex (85%), EA type (74%), and repair type (60%). Most frequently reported outcomes were anastomotic stricture (72%), anastomotic leakage (68%), and mortality (66%)., Conclusion:  This study demonstrates considerable heterogeneity of studied parameters in EA research, emphasizing the need for standardized reporting to compare results of EA research. Additionally, the identified items may help develop an informed, evidence-based consensus on outcome measurement in esophageal atresia research and standardized data collection in registries or clinical audits, thereby enabling benchmarking and comparing care between centers, regions, and countries., Competing Interests: The EPSA|ERNICA Registry was funded by the European Commission in the 3rd Health Program [HP-PJ-2019]., (Thieme. All rights reserved.)

Background: The effect of peri-operative management on the neonatal brain is largely unknown. Triggers for perioperative brain injury might be revealed by studying changes in neonatal physiology peri-operatively., Objective: To study neonatal pathophysiology and cerebral blood flow regulation peri-operatively using the neuro-cardiovascular graph., Design: Observational, prospective cohort study on perioperative neuromonitoring. Neonates were included between July 2018 and April 2020., Setting: Multicentre study in two high-volume tertiary university hospitals., Patients: Neonates with congenital diaphragmatic hernia were eligible if they received surgical treatment within the first 28 days of life. Exclusion criteria were major cardiac or chromosomal anomalies, or syndromes associated with altered cerebral perfusion or major neurodevelopmental impairment. The neonates were stratified into different groups by type of peri-operative management., Intervention: Each patient was monitored using near-infrared spectroscopy and EEG in addition to the routine peri-operative monitoring. Neurocardiovascular graphs were computed off-line., Main Outcome Measures: The primary endpoint was the difference in neurocardiovascular graph connectivity in the groups over time., Results: Thirty-six patients were included. The intraoperative graph connectivity decreased in all patients operated upon in the operation room (OR) with sevoflurane-based anaesthesia ( P  < 0.001) but remained stable in all patients operated upon in the neonatal intensive care unit (NICU) with midazolam-based anaesthesia. Thoracoscopic surgery in the OR was associated with the largest median connectivity reduction (0.33 to 0.12, P  < 0.001) and a loss of baroreflex and neurovascular coupling. During open surgery in the OR, all regulation mechanisms remained intact. Open surgery in the NICU was associated with the highest neurovascular coupling values., Conclusion: Neurocardiovascular graphs provided more insight into the effect of the peri-operative management on the pathophysiology of neonates undergoing surgery. The neonate's clinical condition as well as the surgical and the anaesthesiological approach affected the neonatal physiology and CBF regulation mechanisms at different levels., Trial Registration: NL6972, URL: https://www.trialre-gister.nl/trial/6972 ., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society of Anaesthesiology.)

Background and aim: Neonatal brain monitoring is increasingly used due to reports of brain injury perioperatively. Little is known about the effect of sedatives (midazolam) and anesthetics (sevoflurane) on cerebral oxygenation (rScO 2 ) and cerebral activity. This study aims to determine these effects in the perioperative period. Methods: This is an observational, prospective study in two tertiary pediatric surgical centers. All neonates with a congenital diaphragmatic hernia received perioperative cerebral oxygenation and activity measurements. Patients were stratified based on intraoperatively administrated medication: the sevoflurane group (continuous sevoflurane, bolus fentanyl, bolus rocuronium) and the midazolam group (continuous midazolam, continuous fentanyl, and continuous vecuronium). Results: Intraoperatively, rScO 2 was higher in the sevoflurane compared to the midazolam group (84%, IQR 77-95 vs. 65%, IQR 59-76, p = < 0.001), fractional tissue oxygen extraction was lower (14%, IQR 5-21 vs. 31%, IQR 29-40, p = < 0.001), the duration of hypoxia was shorter (2%, IQR 0.4-9.6 vs. 38.6%, IQR 4.9-70, p = 0.023), and cerebral activity decreased more: slow delta: 2.16 vs. 4.35 μ V 2 ( p = 0.0049), fast delta: 0.73 vs. 1.37 μ V 2 ( p = < 0.001). In the first 30 min of the surgical procedure, a 3-fold increase in fast delta (10.48-31.22 μ V 2 ) and a 5-fold increase in gamma (1.42-7.58 μ V 2 ) were observed in the midazolam group. Conclusion: Sevoflurane-based anesthesia resulted in increased cerebral oxygenation and decreased cerebral activity, suggesting adequate anesthesia. Midazolam-based anesthesia in neonates with a more severe CDH led to alarmingly low rScO 2 values, below hypoxia threshold, and increased values of EEG power during the first 30 min of surgery. This might indicate conscious experience of pain. Integrating population-pharmacokinetic models and multimodal neuromonitoring are needed for personalized pharmacotherapy in these vulnerable patients. Trial Registration: https://www.trialregister.nl/trial/6972, identifier: NL6972., 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 Costerus, Hendrikx, IJsselmuiden, Zahn, Perez-Ortiz, Van Huffel, Flint, Caicedo, Wijnen, Wessel, de Graaff, Tibboel and Naulaers.)

The role of CDK1 in PDAC onset and development is two-fold. Firstly, since CDK1 activity regulates the G2/M cell cycle checkpoint, overexpression of CDK1 can lead to progression into mitosis even in cells with DNA damage, a potentially tumorigenic process. Secondly, CDK1 overexpression leads to the stimulation of a range of proteins that induce stem cell properties, which can contribute to the development of cancer stem cells (CSCs). CSCs promote tumor-initiation and metastasis and play a crucial role in the development of PDAC. Targeting CDK1 showed promising results for PDAC treatment in different preclinical models, where CDK1 inhibition induced cell cycle arrest in the G2/M phase and led to induction of apoptosis. Next to this, PDAC CSCs are uniquely sensitive to CDK1 inhibition. In addition, targeting of CDK1 has shown potential for combination therapy with both ionizing radiation treatment and conventional chemotherapy, through sensitizing tumor cells and reducing resistance to these treatments. To conclude, CDK1 inhibition induces G2/M cell cycle arrest, stimulates apoptosis, and specifically targets CSCs, which makes it a promising treatment for PDAC. Screening of patients for CDK1 overexpression and further research into combination treatments is essential for optimizing this novel targeted therapy.

Introduction:  Evidence supporting best practice for long-gap esophageal atresia is limited. The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) organized a consensus conference on the management of patients with long-gap esophageal atresia based on expert opinion referring to the latest literature aiming to provide clear and uniform statements in this respect., Materials and Methods:  Twenty-four ERNICA representatives from nine European countries participated. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing items on perioperative, surgical, and long-term management, and literature review. The 2-day conference was held in Berlin in November 2019. Anonymous voting was conducted via an internet-based system using a 1 to 9 scale. Consensus was defined as ≥75% of those voting scoring 6 to 9., Results:  Ninety-seven items were generated. Complete consensus (100%) was achieved on 56 items (58%), e.g., avoidance of a cervical esophagostomy, promotion of sham feeding, details of delayed anastomosis, thoracoscopic pouch mobilization and placement of traction sutures as novel technique, replacement techniques, and follow-up. Consensus ≥75% was achieved on 90 items (93%), e.g., definition of long gap, routine pyloroplasty in gastric transposition, and avoidance of preoperative bougienage to enable delayed anastomosis. Nineteen items (20%), e.g., methods of gap measurement were discussed controversially (range 1-9)., Conclusion:  This is the first consensus conference on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia. Substantial statements regarding esophageal reconstruction or replacement and follow-up were formulated which may contribute to improve patient care., Competing Interests: None declared., (Thieme. All rights reserved.)

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., Competing Interests: None declared., (Thieme. All rights reserved.)

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., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Children with intestinal failure (IF) are at risk of growth failure, but little information about body composition is available. Our aim was to assess body composition using air displacement plethysmography (ADP) and relate it to clinical and growth parameters., Methods: In this prospective descriptive observational 2-center cohort study, children aged 2-18 years receiving home parenteral nutrition (PN) for ≥6 months underwent ADP measurement. Fat mass index (FMI) and fat-free mass index (FFMI) standard deviation scores (SDSs) were calculated to normalize for small body size., Results: Twenty-one out of 22 children, median age 7.4 years, underwent successful ADP measurement after a median PN duration of 5.5 years. They were significantly lighter (median weight for age SDS -0.71, P = 0.004) and shorter (median height for age SDS -1.55, P < 0.001) than the normal population mean; 52% were growing below target height range. They had low FFMI (median SDS -1.53, P < 0.001) and high FMI (median SDS 0.80, P = 0.002). Weight for height and body mass index (BMI) were significantly associated with FFMI and BMI with FMI, but children with the same weight and height showed different body composition. In 13 patients with 1-year follow-up, growth and body composition did not change significantly., Conclusion: Children with IF receiving long-term PN show lower FFM and higher FM than healthy children. Additionally, children with similar routine growth parameters showed different body composition. Further studies should evaluate the effect of a patient-tailored approach including physical activity and nutrition advice based on body composition., (© 2019 American Society for Parenteral and Enteral Nutrition.)

Objective: Improvements in imaging and surgical technological innovations have led to the increasing implementation of fetal surgical techniques. Open fetal surgery has demonstrated more favorable clinical outcomes in children born with open myelomeningocele (MMC) than those following postnatal repair. However, primarily because of maternal risks but also because of fetal risks, fetal surgery for MMC remains controversial. Here, the authors evaluated the contemporary management of MMC in the hope of identifying barriers and facilitators for neurosurgeons in providing fetal surgery for MMC., Methods: An online survey was emailed to members of the Congress of Neurological Surgeons (CNS) and the International Society for Pediatric Neurosurgery (ISPN) in March 2019. The survey focused on 1) characteristics of the respondents, 2) the practice of counseling on and managing prenatally diagnosed MMC, and 3) barriers, facilitators, and expectations of fetal surgery for MMC. Reminders were sent to improve the response rate., Results: A total of 446 respondents filled out the survey, most (59.2%) of whom specialized in pediatric neurosurgery. The respondents repaired an average of 9.6 MMC defects per year, regardless of technique. Regardless of the departments in which respondents were employed, 91.0% provided postnatal repair of MMC, 13.0% open fetal repair, and 4.9% fetoscopic repair. According to the surgeons, the most important objections to performing open fetal surgery were a lack of cases available to become proficient in the technique (33.8%), the risk of maternal complications (23.6%), and concern for fetal complications (15.2%). The most important facilitators according to advocates of prenatal closure are a decreased rate of shunt dependency (37.8%), a decreased rate of hindbrain herniation (27.0%), and an improved rate of motor function (18.9%). Of the respondents, only 16.9% agreed that open fetal surgery should be the standard of care., Conclusions: The survey results showed diversity in the management of patients with MMC. In addition, significant diversity remains regarding fetal surgery for MMC closure. Despite the apparent benefits of open fetal surgery in selected pregnancies, only a minority of centers and providers offer this technique. As a more technically demanding technique that requires multidisciplinary effort with less well-established long-term outcomes, fetoscopic surgery may face similar limited implementation, although the surgery may pose fewer maternal risks than open fetal surgery. Centralization of prenatal treatment to tertiary care referral centers, as well as the use of sophisticated training models, may help to augment the most commonly cited objection to the implementation of prenatal closure, which is the overall limited caseload.

Introduction:  Multiple reports have questioned the feasibility of neonatal thoracoscopic repair of congenital diaphragmatic hernia (CDH) and esophageal atresia (EA). The aim of this study is to examine the effects of CO 2 pneumothorax on cerebral and renal rSO 2 and to assess the potential predictive value of these data on neurodevelopmental outcome after neonatal thoracoscopic surgery for CDH or EA., Materials and Methods:  A prospective observational pilot study. Cerebral and renal regional tissue oxyhemoglobine saturation (rSO 2 ) rSO 2 were assessed using near-infrared spectroscopy (NIRS) during thoracoscopic surgery in neonates with CDH and with EA, in addition to routine anesthesia monitoring. Cerebral and renal rSO 2 , linked to repeated arterial blood gas analyses, heart rate, blood pressure, and to structured longitudinal neurodevelopmental follow-up., Results:  Baseline estimated marginal means of cerebral rSO 2 values (CDH: 82%, EA: 91%) did not change significantly during pneumothorax (CDH: 81%, EA 79% [n.s. versus baseline]) despite severe acidosis (lowest pH, CDH: 6.99, EA: 7.1). Neurodevelopmental outcomes at 24 months were normal in all 7 patients who were available for evaluation., Conclusion:  Neonatal thoracoscopic repair of CDH and EA using CO 2 -pneumothorax leads to severe acidosis. Cerebral rSO 2 remained within clinical acceptable limits during intraoperative periods of acidosis. Neurodevelopmental outcome was favorable within the first 24 months. The potential of NIRS to further improve perioperative care and long-term outcome in this specific patient group deserves further investigation., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background:  Epidural analgesia (EDA) is used as postoperative pain treatment in children, but little is known about its efficacy after laparotomy in children. This retrospective study investigated the efficacy of postoperative EDA after laparotomy in children and the frequencies of adverse effects and complications., Materials and Methods:  Data of all children aged 0 to 18 years undergoing laparotomy and treated with EDA in our hospital from January 2014 to December 2015 were collected. EDA was classified as successful either if the catheter was removed when there was no further need, and the patient was comfortable with oral/rectal analgesics or when EDA was continued after intervention., Results:  Ninety children with a median age of 3.7 years were included in the study. In 65% (59/90), EDA was classified as successful. In 34% (20/59) of all successful cases, an additional intervention was needed. In 35% (31/90), the catheter was removed earlier than planned, in half of these cases after an intervention. The primary reason for earlier removal was inadequate analgesia and technical failure. Adverse effects occurred in 18% and complications in 1% of cases. The successful group was significantly younger than the unsuccessful ( p  = 0.003)., Conclusion:  EDA after laparotomy positively contributed to postoperative pain treatment in two-thirds of children. Numbers of adverse effects and complications were low. It is important to optimize pain management after laparotomy in children; thus, further prospective studies should focus on optimizing EDA and comparing EDA with other techniques of analgesia., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Intestinal failure (IF) is the reduction of gut function or mass below a minimum needed to absorb nutrients and fluids, such that patients are dependent on parenteral nutrition (PN). Patients with IF have an altered gut microbiome. Our aim was to review and evaluate the current evidence on gut microbiome and its metabolic activity, as well as its association with disease characteristics in adults and children with IF. We performed a PubMed literature search for articles published after 2000 using the following terms: intestinal, microbiome, microbiota, short-chain fatty acids, short bowel syndrome, and PN. Literature search was restricted to human studies only. The gut microbiome diversity is remarkably reduced, and community structure is altered with a noticeable overabundance of Proteobacteria, especially the Enterobacteriaceae family. A substantial increase in Lactobacillus level is often reported in patients with IF. Gut microbiome characteristics have been associated with poor growth, liver disease, D-lactic acidosis, and duration of intestinal adaptation. Differences in microbiome characteristics have been found between patients receiving PN and those whose guts have adapted and have been weaned off PN. Future research with prospective sample collection should explore the value of the gut microbiome as a biomarker to guide clinical practice and as a modifiable therapeutic target to optimize outcomes of patients with IF., (© 2018 American Society for Parenteral and Enteral Nutrition.)