European Journal of Pediatric Surgery最新文献

Although VACTERL association is a recognized entity in patients with esophageal atresia (EA), its impact on surgical outcomes remains unclear. This study aimed to evaluate the influence of VACTERL association and chromosomal anomalies (VACTERL-CA) on the surgical outcomes of EA patients, offering novel insights into risk stratification.All patients enrolled in the European Pediatric Surgeons' Association (EUPSA) Esophageal Atresia Registry (EAR) between July 2014 and December 2017 were included. Patients were classified into two groups: those with VACTERL-CA and those without these anomalies (non-VACTERL). Groups were compared for demographics, associated malformations, surgical approach, complications, and outcomes.Among 372 patients, 22% (n = 82) were classified as VACTERL-CA. This group had significantly lower gestational age (35.9 weeks vs. 37.1 weeks, p = 0.004), birth weight (2,312 g vs. 2,663 g, p < 0.001), and APGAR scores at 5 and 10 minutes (p = 0.005). Surgical strategies, including rates of primary anastomosis (88% in both groups), did not differ. Anastomotic leak and stricture rates were similar; however, recurrent fistula was more common in VACTERL-CA (4.9% vs. 1.0%, p = 0.023). Overall mortality was higher in VACTERL-CA (14.6% vs. 5.2%, p = 0.003), largely due to associated anomalies such as cardiac or neurologic conditions, whereas EA-related mortality was more frequent in non-VACTERL (1% vs. 0%). Sepsis was also more frequent in VACTERL-CA (10.9% vs. 4.5%, p = 0.033). In multivariate analysis, low birth weight (adjusted odds ratios [aOR]: 0.95 per 100 g, p = 0.010) and cardiac malformations (aOR: 2.33, p = 0.002) were independently associated with VACTERL-CA.EA patients with VACTERL-CA represent a high-risk subgroup characterized by prematurity, major cardiac defects, and increased sepsis risk. These findings highlight the need for early cardiac screening, standardized infection-prevention bundles, and tailored multidisciplinary care to improve survival and reduce preventable complications.

Open repair of congenital diaphragmatic hernia (CDH) in neonates often requires surgical reconstruction of the abdominal wall. Lateral release (LR) of the abdominal wall fascia, a novel technique avoiding prosthetic patches, offers potential advantages. However, data comparing its outcomes to traditional patch repair are limited.A preliminary prospective cohort study was conducted at the University Medical Center Mannheim from 2021 to 2024, including neonates undergoing CDH surgery with abdominal wall reconstruction via direct closure, LR, or patch repair based on intraoperative surgeon decision. Perioperative, postoperative, and short-term outcome data were analyzed during our standardized follow-up protocols and compared between groups.Among 77 eligible neonates, 11 underwent patch repair and 10 received LR. Baseline characteristics between groups were comparable. The median follow-up was 391 days in the patch group and 215 days in the LR group (p = 0.1971). The LR group had significantly shorter median intubation duration, neonatal intensive care unit stay, and overall length of stay compared with the patch group (32.0 days vs. 43.0 days, p = 0.0445; 33.5 days vs. 66.0 days, p = 0.0309; 68.0 days vs. 97.0 days, p = 0.0435). There were no significant differences in recurrence rates, short-term complications, or motor developmental outcomes.LR appears to be associated with shorter hospital stays and faster recovery, without an increase in perioperative or long-term complications. While these findings suggest potential benefits of LR, they must be interpreted with caution due to the limited sample size. Further randomized, multicenter studies with larger cohorts, including long-term assessment of complications, are needed to confirm its efficacy and refine clinical guidelines.

Nuss procedure is the standard technique for pectus excavatum repair. Despite its minimally invasive nature, this procedure is associated with significant postoperative pain and high opioid consumption. Intercostal nerve cryoablation (INC) has emerged as an adjunct to multimodal analgesia (MMA) to improve pain control, reduce opioid use, and shorten length of stay (LOS). This systematic review aims to assess INC outcomes following the Nuss procedure in pediatric patients.A systematic search was conducted in PubMed, Web of Science, Scopus, and Cochrane Library databases through December 2024. Studies comparing INC with standard MMA, with or without thoracic epidural, in pediatric patients undergoing the Nuss procedure were included. The primary outcome was LOS, and the secondary outcomes were opioid consumption, postoperative pain, complications, operative time, and hospitalization costs. Risk of bias was determined using the National Institutes of Health assessment tool. Meta-analysis was performed using R software.Eleven studies met the inclusion criteria, comprising 922 patients (476 INC and 446 control). INC significantly reduced LOS (-2.2 days; 95% CI: -2.8 to -1.8) at the expense of increased operating room time (+23 minutes; 95% CI: 10-39). Qualitative analysis showed reduced opioid use and comparable pain scores and complication rates with INC, while its impact on costs was conflicting.INC reduces LOS and opioid use in pediatric patients undergoing the Nuss procedure without increasing complications. Further studies are needed to assess long-term safety and cost-effectiveness.

Giant omphalocele management had not reached a consensus from the pediatric surgical perspective regarding conservative treatments, surgical approaches, and clinical outcomes. This topic was therefore selected for the 2023 Consensus Session of the European Paediatric Surgeons' Association (EUPSA).Literature review was conducted by seven EUPSA members, guided by a set of predefined areas relating to the management of giant omphalocele: (1) conservative management, (2) surgical management, and (3) outcomes. Members were assigned to specific topics, with discrepancies resolved through structured group discussion and further literature review. Consensus was reached through unanimous agreement among the contributing members. Each topic was presented with available evidence to congress participants. Comments from participants were accounted to formulate the final consensus statement.Giant omphalocele is appropriately defined as viscero-abdominal disproportion preventing primary closure. Regarding (1) conservative management, the "paint and wait" approach is recommended when anatomical constraints or high surgical risk preclude primary closure. Common painting agents include povidone-iodine and silver sulfadiazine, with Manuka honey gaining interest, though consensus on dosing, duration, and complications remains unclear. With regards to (2) surgical management, early closure favors biological meshes, while delayed closure appears most effective using native tissues. High mortality in delayed patch closure likely reflects a population with the most severe defects. Finally, (3) outcomes highlights key prognostic factors including chromosomal abnormalities, cardiac defects, and low birth weight, which may guide counseling, screening, and treatment. Limited data on ruptured omphalocele indicates towards increased mortality, necessitating prompt intervention. Complications following management are relatively rare and typically intervention-related, underscoring the need for long-term, multidisciplinary follow-up.A consensus statement on the management of giant omphalocele was developed based on current evidence and peer practice, though imitations relating to a scarcity of high-level evidence and significant heterogeneity across studies should be acknowledged. Despite these constraints, this consensus statement provides evidence-based guidance to support pediatric surgeons in informed decision-making for this pathology.

Anorectal malformation (ARM) and Hirschsprung disease (HSCR) are common congenital gastrointestinal defects, but their co-occurrence is rare. This retrospective study analyzed the clinical characteristics of patients with ARM associated with HSCR for early diagnosis and treatment guidance to reduce the occurrence of severe complications.A single-center retrospective cohort analysis from 2010 to 2024 identified 2,341 patients with ARM and 1,721 with HSCR. The histopathologic assessment included hematoxylin and eosin (H&E) staining and immunohistochemical staining.Seven patients (0.3%) out of 2,341 cases of ARM were diagnosed with concurrent HSCR, three males (42.9%) and four females (57.1%). Seven cases are rectoperineal fistula. All cases developed constipation with abdominal distension within 1 month to 1 year after anoplasty, even following aggressive bowel management. Barium enema showed obvious transition zones, and anorectal manometry revealed absent rectoanal inhibitory reflex in seven cases. All patients underwent the Swenson procedure. The mean duration of postoperative follow-up was 7.5 ± 2.8 years. Seven cases had no constipation, no soiling, voluntary bowel movements by Krickenbeck classification, and excellent continence by the Rintala scoring system in recent follow-up.The association between ARM and HSCR may be rarer than previously reported. Low-type ARM and short or rectosigmoid aganglionosis appeared more common in these cases. Persistent postoperative constipation and abdominal distension unresponsive to conservative treatment should raise suspicion for HSCR, prompting timely diagnostic evaluations. Postoperative bowel function needs to be interpreted carefully, and prospective studies are needed to confirm these findings and guide standardized care.

The emergence of large language models (LLMs) has led to notable advancements across multiple sectors, including medicine. Yet, their effect in pediatric surgery remains largely unexplored. This study aims to assess the ability of the artificial intelligence (AI) models ChatGPT-4 and Microsoft Copilot to propose diagnostic procedures, primary and differential diagnoses, as well as answer clinical questions using complex clinical case vignettes of classic pediatric surgical diseases.We conducted the study in April 2024. We evaluated the performance of LLMs using 13 complex clinical case vignettes of pediatric surgical diseases and compared responses to a human cohort of experienced pediatric surgeons. Additionally, pediatric surgeons rated the diagnostic recommendations of LLMs for completeness and accuracy. To determine differences in performance, we performed statistical analyses.ChatGPT-4 achieved a higher test score (52.1%) compared to Copilot (47.9%) but less than pediatric surgeons (68.8%). Overall differences in performance between ChatGPT-4, Copilot, and pediatric surgeons were found to be statistically significant (p < 0.01). ChatGPT-4 demonstrated superior performance in generating differential diagnoses compared to Copilot (p < 0.05). No statistically significant differences were found between the AI models regarding suggestions for diagnostics and primary diagnosis. Overall, the recommendations of LLMs were rated as average by pediatric surgeons.This study reveals significant limitations in the performance of AI models in pediatric surgery. Although LLMs exhibit potential across various areas, their reliability and accuracy in handling clinical decision-making tasks is limited. Further research is needed to improve AI capabilities and establish its usefulness in the clinical setting.

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.