Follow-up and transition practices in esophageal atresia: a review of European Reference Network on rare Inherited and Congenital Anomalies (ERNICA) centres and affiliates.

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.

The Role of GERD Diagnosis and Treatment in Preventing Lung Function Decline After Pediatric Lung Transplantation.

Background/purpose:  Gastroesophageal reflux disease (GERD) after lung transplantation (LuTx) can lead to chronic lung allograft dysfunction. Our aim was to assess the prevalence of GERD in pediatric LuTx recipients and to investigate the impact of medical and surgical GERD treatment on lung function.

Methods:  Ethical approval was obtained.

Medical Costs of Patients Undergoing Esophageal Atresia Repair are Mainly Influenced by Associated Malformations.

Background:  Esophageal atresia (EA) is a rare disease requiring surgical repair, usually within the first days of life. Patients with EA require intensive postoperative care and often have comorbidities. There is a lack of data on the costs incurred by patients with EA during the first year of life.

Lost in transition? Loss of follow-up and quality of life in adults after resection of choledochal malformation in childhood.

Objectives: Choledochal malformation (CM) is a rare disease that can lead to malignancy and potential long-term sequelae despite surgical resection. There is no long-term follow-up data on patients after CM resection in Germany. We aimed to determine the long-term outcome of our patients with a duration of follow-up >10 years and focused on long-term sequelae and health-related quality of life (HRQOL).

Validation of the Clavien-Madadi Classification for Unexpected Events in Pediatric Surgery: A Collaborative ERNICA Project.

Background: The Clavien-Madadi classification is a novel instrument for the assessment and grading of unexpected events in pediatric surgery, based on the Clavien-Dindo classification. The system has been adjusted to better fit the pediatric population in a prospective single-center study. There is a need now to validate the Clavien-Madadi classification within an international expert network.

Selection of Quality Indicators to Evaluate Quality of Care for Patients with Esophageal Atresia Using a Delphi Method.

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.

Benchmarks for Pediatric Surgical Registries: Recommendations for the Assessment and Grading of Complications.

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.

Titanium nitride coating of pectus bar increases metal contamination after minimally-invasive repair of pectus excavatum.

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).

Impact of the COVID-19 Pandemic on Pediatric Surgical Scientific Formats: An ERNICA Survey on Challenges and Future Directions.

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.

MUC-FIRE: Study protocol for a randomized multicenter open-label controlled trial to show that MUCous FIstula REfeeding reduces the time from enterostomy closure to full enteral feeds.

Background: After enterostomy creation, the distal bowel to the ostomy is excluded from the physiologic passage of stool, nutrient uptake, and growth of this intestinal section. Those infants frequently require long-term parenteral nutrition, continued after enterostomy reversal due to the notable diameter discrepancy of the proximal and distal bowel. Previous studies have shown that mucous fistula refeeding (MFR) results in faster weight gain in infants.

Implementation and validation of a novel instrument for the grading of unexpected events in paediatric surgery: Clavien-Madadi classification.

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.

Surgical management of large tracheoesophageal fistula in infants after button battery ingestion.

Objectives: In recent years, an increase in severe and even fatal outcomes related to oesophageal or airway button battery (BB) ingestion by infants and small children has been reported. Extensive tissue necrosis caused by lodged BB can lead to major complications, including tracheoesophageal fistula (TEF). In these instances, best treatment remains controversial.

Variability in the Reporting of Baseline Characteristics, Treatment, and Outcomes in Esophageal Atresia Publications: A Systematic Review.

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.

Definition, Documentation, and Classification of Complications in Pediatric Surgical Literature-A Plea for Standardization.

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.

Appendiceal Carcinoids in Children-Prevalence, Treatment and Outcome in a Large Nationwide Pediatric Cohort.

: 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. : 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.

A genome-wide association study with tissue transcriptomics identifies genetic drivers for classic bladder exstrophy.

Classic bladder exstrophy represents the most severe end of all human congenital anomalies of the kidney and urinary tract and is associated with bladder cancer susceptibility. Previous genetic studies identified one locus to be involved in classic bladder exstrophy, but were limited to a restrict number of cohort. Here we show the largest classic bladder exstrophy genome-wide association analysis to date where we identify eight genome-wide significant loci, seven of which are novel.

Pleural CD14 monocytes/macrophages of healthy adolescents show a high expression of metallothionein family genes.

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).

Laparoscopic versus Open Inguinal Hernia Repair Is Feasible in Infants with Caudal Anesthesia and Spontaneous Respiration.

Introduction:  Minimally invasive surgery (i.e., laparoscopy) and minimally invasive anesthesia (i.

Nickel contamination after minimally-invasive repair of pectus excavatum persists after bar removal.

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).

Heterozygous variants in the DVL2 interaction region of DACT1 cause CAKUT and features of Townes-Brocks syndrome 2.

Most patients with congenital anomalies of the kidney and urinary tract (CAKUT) remain genetically unexplained. In search of novel genes associated with CAKUT in humans, we applied whole-exome sequencing in a patient with kidney, anorectal, spinal, and brain anomalies, and identified a rare heterozygous missense variant in the DACT1 (dishevelled binding antagonist of beta catenin 1) gene encoding a cytoplasmic WNT signaling mediator. Our patient's features overlapped Townes-Brocks syndrome 2 (TBS2) previously described in a family carrying a DACT1 nonsense variant as well as those of Dact1-deficient mice.

Need for transition medicine in pediatric surgery - health related quality of life in adolescents and young adults with congenital malformations.

Survival rates of patients with visceral congenital malformations have increased considerably. However, long-term morbidity in these patients is high. In the last decades, these circumstances have led to a shift in goals of caretakers and researchers with a new focus on patients' perspectives and long-term morbidity.