European Journal of Pediatric Surgery最新文献

The genetic study of vascular anomalies provides a better understanding of their etiopathogenesis and allows the use of targeted therapies. Activating KRAS pathogenic variants promote cell proliferation by activating MAPK and PI3K signalling pathways, which have been associated with the pathogenesis of vascular anomalies, especially high-flow ones such as arteriovenous malformations (AVMs). AVMs' genomic landscape is extensive, and a genotype-phenotype correlation has not been shown. In this study, we aimed to prove an association between KRAS gene mutations and the presence of osteolysis in patients with AVMs. Herein, we present a clinical-molecular retrospective study of patients with AVMs, bone involvement, and KRAS pathogenic variants.A retrospective review of patients with AVMs and KRAS somatic variants followed by the Vascular Anomalies Unit at our institution was performed. All patients present bone involvement. We analyzed demographics, clinical features (AVMs location, phenotype), treatment received, and response to treatment. Previous imaging studies were used to assess bone involvement. Genetic studies were performed by high-throughput sequencing using a custom-designed panel.Of the 77 patients with AVMs currently followed in our clinic, 60 (77.9%) had genetic testing, and 19 (31.6%) presented a KRAS somatic activating variant and were therefore included in the study. There were 12 women and 7 men aged 10 to 79 years. When studying radiographies or CT scans, we found that all 19 patients associated osteolysis adjacent to the AVMs. Regarding the KRAS variants, the most frequent one was p.Gly12Asp, followed by p.Gln61His and p.Gly13Arg. Additionally, we reviewed imaging studies from the other 41 patients with AVMs and different pathogenic variants such as MAP2K1, RASA1, and BRAF, and did not find osteolysis.We have described for the first time the relationship between somatic, activating KRAS pathogenic variants and osteolysis in patients with AVMs. Early detection of these KRAS alterations in this type of patient should lead us to rule out bone involvement. Moreover, identifying these mutations may help guide targeted therapies, potentially preventing the development of osteolysis and improving patient outcomes.

Objective:  This study aims to assess publishing trends, motivations, preferences, and challenges among pediatric surgeons globally.

Methods:  A cross-sectional survey was conducted among pediatric surgeons from multiple countries, distributed through the Trainees of European Pediatric Surgery (TEPS) network and social media. The anonymous questionnaire contained 26 items focusing on journal preferences, motivations for publishing, obstacles faced, peer-review experiences, open access publishing, and methods of research dissemination.

Results:  A total of 172 responses were collected from pediatric surgeons in 33 countries. Most respondents worked in tertiary hospitals (88%) and were consultants or senior attendings (49%). Over half (65%) had published at least one scientific paper in the last 3 years. PubMed was the primary search engine (82%), and pediatric surgical journals were the preferred outlets for publication (87%). Key motivations for choosing a journal were impact factor (22%) and scope (19%), while publication costs (38%) and slow review processes (22%) were the primary deterrents. Open access publication options were used by more than half of respondents, with a third spending less than €2,500 on fees. Social media, particularly Instagram, emerged as a popular platform for research dissemination.

Conclusion:  Pediatric surgeons prefer publishing in specialized journals, with impact factor and scope being key drivers of journal choice. Publication costs and the peer-review process are the most significant obstacles. Efforts to address these challenges, such as reducing fees and enhancing the review process, are crucial for facilitating research dissemination in pediatric surgery.

This study compares various surgical approaches for treating congenital diaphragmatic eventration (CDE) in children to identify the most effective and safest method.We conducted a retrospective analysis of a multicentric cohort of pediatric patients operated on for CDE between 2010 and 2021. The different surgical approaches, including robot-assisted thoracoscopic surgery (RATS), and their outcomes were compared (Clinical Trials NCT04862494).One hundred and twelve patients, aged 12 (5-21) months, underwent diaphragmatic plication. Thoracoscopy or RATS was performed in 69 (62%) cases, posterolateral thoracotomy (PLT) in 15 (13%), and an abdominal approach in 28 (25%), based on surgeons' choice. Symptom relief was achieved in 88% of patients, and 90% showed radiographic improvement. There were 31 peri- or early postoperative complications (28%), mainly including pleural effusions, infections, and lobar atelectasis, and 8 recurrences of eventration (7%), with no significant correlation between these complications and the surgical approach. Compared to other approaches, thoracotomy multiplied the duration of intravenous analgesia by three (96 h vs. 36 h, p < 0.0001) and hospital stay length by two (8 vs. 4 days, p = 0.002). RATS, although comparable to thoracoscopy in short-term outcomes, had a higher incidence of perioperative hepatic injuries and long-term complications, including persistent symptoms in all five patients and chest wall deformities in two.Diaphragmatic plication via a minimally invasive thoracic approach may be the best treatment option for cases of symptomatic CDE. Further research is required to establish potential added risks of RATS as compared to thoracoscopy in this indication.

Appendicitis is the most common indication for emergent abdominal surgery in childhood. Intravenous antibiotic therapy reduces infections-related complications. Epidemiological data concerning bacterial pathogens are important in tailoring antibiotic stewardship recommendations.This study aims to assess trends over the years in bacterial distribution, and sensitivities (specifically multidrug-resistant [MDR] Enterobacteriaceae) in surgical appendicitis among two different ethnic communities in southern Israel.This was an observational, single-center, retrospective study. Included were children less than 18 years of age with surgical appendicitis treated in Soroka University Medical Center between 2010 and 2020 that had a positive intraoperative intraperitoneal bacterial swab culture. Data were assessed using univariable and multivariable analyses including multiple linear regression and negative binomial regressions with time series analysis to compare between periods during the study while accounting for confounders.Overall, 1,858 specimens were available for analysis from 2,264 children with confirmed surgical appendicitis. Pathogenic bacteria were recovered in 684 (36.8%), with nearly half polymicrobial. MDR Enterobacteriaceae pathogens were more common in the Arab-Bedouin community as compared with the Jewish community (32.6% vs. 18.6%, p < 0.001). Time series analysis detected an 8.7% significant increase in MDR pathogens per year (p = 0.003) with male children (10% per year [p = 0.016]), children younger than 12 years (10% per year [p = 0.014]), and children of the Arab-Bedouin community (8.7% per year [p = 0.025]) accounting for this increase in MDR isolates.MDR pathogens incidence has significantly increased between 2010 and 2020 and this should be considered in the choice of antibiotic therapies and antibiotic stewardship programs in the hospital and the community.

Since 2019, the European Pediatric Surgical Audit (EPSA) has been the official registry of the European Reference Network for Inherited and Congenital Anomalies (ERNICA). The primary aim of this prospective patient registry is benchmarking (quality of) care for patients with rare congenital malformations throughout Europe. Data collected comprise baseline, treatment, and outcome variables, permitting calculation of disease-specific, hospital-level quality indicator results reflecting between-hospital variation. This practice and outcome variation is fed back as actionable information to clinicians on a web-based, real-time dashboard to help focus local and central improvement initiatives. Secondly, realizing joint research initiatives with quality improvement purposes through secondary data use will increase our knowledge of these rare conditions and optimize care. Currently, 27 hospitals in 15 European countries have connected to this unique, European-wide audit. Henceforward, the focus will be on the further expansion of hospitals and diseases, as EPSA aspires to become all-encompassing, including all European patients with congenital malformations.

Pathophysiological effects of abdominal CO₂ insufflation on cerebral and splanchnic oxygenation in neonates and infants undergoing laparoscopy remain poorly investigated. We investigated laparoscopic pyloromyotomy as a paradigm to determine such changes in this specific population.Single-center, prospective cohort of 28 infants undergoing laparoscopic pyloromyotomy at the mean age of 30.9 ± 10.6 days. The pneumoperitoneum was set at 6 to 8 mmHg. Regional cerebral oxygen saturation (cSO₂) and splanchnic oxygen saturation (sSO₂) were measured by near-infrared spectroscopy. End-tidal carbon dioxide (EtCO₂) levels, heart rate, body temperature, systemic blood pressure, and urine output were also recorded. Data (mean ± SD) were collected intraoperatively at 0, 15, and 30 minutes and compared to baseline values for each patient using the t-test.A significant decrease in cSO₂ was recorded only at the beginning of surgery, while sSO₂ significantly decreased from 15 intraoperative minutes (-7.1% ± 7.2; p = 0.0009) until the end of insufflation, followed by an increasing trend, although still below the baseline values (-6.5% ± 11.2; p = 0.01). EtCO₂ increased significantly from the initial 15 intraoperative minutes, reaching a maximum of 42.6 ± 8.9 mmHg at 30-minute intervals. Urine output significantly decreased within the first 4 postoperative hours.Laparoscopic pyloromyotomy using low-pressure CO₂ insufflation (6-8 mmHg) maintains stable cerebral oxygenation in neonates and infants, while splanchnic oxygenation and urine output experience temporary, reversible reductions. These findings suggest that low-pressure pneumoperitoneum is a safe and effective approach in neonatal laparoscopy, with minimal oxygenation and metabolic risks.

Congenital diaphragmatic hernia (CDH) is a malformation that significantly impacts neonatal morbidity and mortality. Recurrence after surgical repair remains a potentially life-threatening long-term complication. Conventionally, recurrent CDH has been managed through open surgery. However, thoracoscopic repair (TR) represents a novel alternative for recurrent CDH as it has reduced the length of hospital stay and mortality rate in the primary CDH repair.A prospective, propensity score-matched analysis was conducted on pediatric patients who underwent recurrent CDH repair at the University Hospital Mannheim between 2013 and 2023, to compare the outcomes of laparotomy versus TR. Patients were categorized based on the surgical technique employed. Comparative analysis, including propensity scoring, encompassed outcome measures such as duration of ICU and hospital stays, rate of complications, and operative duration.In total, 703 patients were treated for CDH, of whom 69 children underwent laparotomy (56) or TR (16) for CDH recurrence. After propensity score matching, TR group demonstrated a significantly shorter duration of surgery (178 [93-311] versus 225 [113-450] min, p = 0.042), reduced ICU stay (0 [0-10] versus 1 (0-69) days, p = 0.011), and decreased overall hospital stay (6 [3-34] versus 12 [7-40] days, p = 0.001). Moreover, the postoperative complications were significantly lower in the TR group (21.43% versus 73.68%, p = 0.003).TR for recurrent CDH repair is associated with shorter operation times, reduced ICU and overall hospital stays, and fewer complications compared with laparotomy. These findings suggest that TR may be preferable for the management of recurrent CDH, warranting larger randomized controlled studies to confirm the long-term safety and efficacy of this approach.

The optimal approach for repairing severe hypospadias remains debated. In our institution, the single-stage double-face preputial island flap (DFPIF) has been employed to reduce the number of procedures and costs. Given the resource-limited context, an evidence-based comparison of repair techniques was warranted to inform cost-effective surgical decision-making.A prospective randomized study was conducted on 36 patients with proximal hypospadias and chordee >30 degrees, treated between 2022 and 2025. Patients were randomly allocated to Group A (DFPIF) or Group B (Byars' flap) using a computer-generated block randomization with allocation concealment. All surgeries were performed by the same team. Patients were followed for 12 months. Groups were compared regarding complications, functional, and cosmetic outcomes. Functional outcomes were assessed using parent-reported urinary stream and erection, with objective chordee assessment under anesthesia. Cosmetic results were evaluated using the Hypospadias Objective Scoring Evaluation (HOSE) score and a 10-point parental satisfaction scale. Outcomes were assessed by a blinded team member who was not involved in the operative procedures. Data were analyzed by a blinded analyst.Thirty-six patients were included, with 18 patients in each group. There was no statistically significant difference in complications, except for partial wound dehiscence, which was higher in group A (p = 0.041). Functional and cosmetic outcomes, as well as parental satisfaction, did not differ significantly.While both techniques achieved acceptable early outcomes, the double-face flap group showed higher rates of partial wound dehiscence. Further studies with larger samples and longer follow-up are required to determine long-term efficacy and safety.

Artificial intelligence (AI) is increasingly integrated into pediatric healthcare, offering opportunities to improve diagnostic accuracy and clinical decision-making. However, the complexity and opacity of many AI models raise concerns about trust, transparency, and safety, especially in vulnerable pediatric populations. Explainable AI (XAI) aims to make AI-driven decisions more interpretable and accountable. This review outlines the role of XAI in pediatric surgery, emphasizing challenges related to bias, the importance of ethical frameworks, and the need for standardized benchmarks. Addressing these aspects is essential to developing fair, safe, and effective AI applications for children. Finally, we provide recommendations for future research and implementation to guide the development of robust and ethically sound XAI solutions.

Artificial intelligence (AI) and machine learning (ML) models rapidly transform health care with applications ranging from diagnostic image interpretation, predictive modeling, personalized treatment planning, real-time intraoperative guidance, and outcome prediction. However, their implementation in general pediatric surgery remains limited due to the rarity and complexity of pediatric surgical conditions, small and heterogeneous datasets, and a lack of formal AI training and competencies among pediatric surgeons.This narrative review explores the current landscape of AI and ML applications in general pediatric surgery, focusing on five key conditions: appendicitis, necrotizing enterocolitis, Hirschsprung's disease, congenital diaphragmatic hernia, and biliary atresia. For each, we summarize recent developments, including the use of AI in image analysis, diagnostic support, prediction of disease severity and outcome, postoperative monitoring, and histopathological evaluation. We also highlight novel tools such as explainable AI models, natural language processing, and wearable technologies.Recent findings demonstrate promising diagnostic and prognostic capabilities across multiple conditions. However, most AI/ML models still require external validation and standardization. The review underscores the importance of collaborative, multicenter research based on joint datasets as well as targeted AI education for pediatric surgeons to fully explore the benefits of these technologies in clinical practice.AI and ML offer significant potential to improve pediatric surgical care, but broader implementation will require multicenter collaboration, a robust dataset, and targeted AI education for pediatric surgeons.