Pediatric cholecystitis and cholelithiasis management is heterogeneous. We surveyed European centers to map current practices, training exposure, and outcomes of pediatric biliary cholecystectomy. A 24-item cross-sectional international survey was developed by the European Union of Medical Specialists (UEMS) Section of Paediatric Surgery and distributed to centers in 31 UEMS member states. Items covered institutional resources, indications and timing, surgical approach and adjuncts (ERCP, ICG), training exposure, and center-level outcomes; results are reported as n (

Objectives: To revise the 2018 European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies (ERNICA) clinical guideline for the management of rectosigmoid Hirschsprung's disease (HSCR) based on new evidence and evolving clinical priorities, ensuring continued relevance, trustworthiness, and consistency in care. Methods: The update followed a structured five-step process: identifying new evidence (2018-2024), prioritizing recommendations using the Update Priority Tool, and revising selected topics using the Grading of Recommendations Assessment, Development and Evaluation-Evidence to Decision (GRADE EtD) framework. A multidisciplinary panel of pediatric surgeons, patient representatives, and methodologists from 12 countries oversaw the process. Revised recommendations were developed through evidence review and consensus meetings. Results: Ten prioritized clinical topics were revised. Key updates include refined recommendations on histopathological staining in diagnosis, preoperative screening for renal anomalies and malnutrition, postoperative anastomotic calibration, individualized dietary guidance, sexual and fertility health during follow-up, transition to adult care, and the role of botulinum toxin in managing Hirschsprung's-associated enterocolitis (HAEC). Routine preventive anal dilatations were no longer recommended. The guideline highlights the importance of structured, multidisciplinary, and patient-centered care throughout the lifespan and identifies several research gaps, including nutrition, transition of care, fertility, and HAEC definitions. Conclusion: This updated guideline reflects current evidence and expert consensus, supporting equitable, patient-centered HSCR care. Implementation will be supported via the European Pediatric Surgical Audit (EPSA), and future updates will be informed by registry data and ongoing research.

Kirberg et al. described in 2016 a one-step gastrostomy tube placement, the 'pull-introducer' technique, designed for small-for-gestational-age neonates, combining two endoscopic techniques. Nine infants (weight 3.4-8.1 kg) in our centre required a gastrostomy placement (two infants) or a combined gastrostomy placement and Nissen fundoplication (seven infants). A single-stage balloon gastrostomy was preferred in all cases because of a significant anaesthetic risk. The pull-introducer method was chosen because the commercially available one-step balloon gastrostomy gastropexy devices were considered too large. There were no immediate or long-term complications (except local infection). All nine infants were discharged from the hospital as planned. After 6 months, the balloon gastrostomy tube was replaced with a balloon button or balloon gastrostomy tube.

Background and aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability. This current study seeks to stratify and assign a score to each IDRF based on its impact on surgical risk. Methods: This collaborative study entailed retrospective collection of patient data from NB operations conducted between 2016 and 2020, together with the computation of a Surgical Complexity Index (SCI) for every patient. The SCI values obtained were correlated with preoperatively identified IDRFs. Mann-Whitney statistical test was employed to assign a relevance score to the association between individual IDRFs and SCI. Results: 14 centers contributed 427 neuroblastoma index cases meeting study inclusion criteria. 303 patients had 1 or more IDRFs. The presence and number of IDRFs in all patients significantly correlated with the SCI value (p < 0.0001). The most frequently encountered IDRF was renal pedicle involvement. There was a significant association observed between clusters of abdominal IDRFs and the occurrence of post-operative complications (p < 0.05), while a weak association link was found with intra-operative complications. A relevance score for individual IDRFs allowed their stratification based on surgical risk. The most relevant IDRFs were extension within two anatomical body compartments, infiltration of the hepato-pancreatic block, encasement of the superior mesenteric artery and coeliac axis, and tumor compressing the trachea. Conclusions: This current study has facilitated the assignment of a relevance score to each IDRF, correlating it with surgical risks. Considering this stratification of surgical risk alongside oncologic risk as defined by the neuroblastoma treatment patient risk grouping should facilitate a more precise definition of surgical objectives and the optimal conditions favoring gross tumor resection. (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

Very rare tumors are usually divided into two groups: One includes tumors that are rare among both children and adults; the other one encompasses tumors that frequently occur in adults but are rarely observed in children. In this review, we focus on adrenocortical tumors, neuroendocrine tumors of the appendix, pheochromocytoma and paraganglioma, pancreatoblastoma and solid pseudopapillary tumors of the pancreas, with special attention to the role of surgery as main curative intervention or as part of the multimodal treatment.

Purpose Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs do not all carry the same weighting in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize a surgical complexity (Surgical Complexity Index, SCI) in NB resection. Methods A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. A shared agreement included the achievement of at least 75% consensus focused on a single or two close risk categories. Results After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient. Conclusions A consensus on a SCI to stratify the risks related to neuroblastoma tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.

OBJECTIVE:To create the first structured surgical report form for NBL with international consensus, to permit standardized documentation of all NBL-related surgical procedures and their outcomes. SUMMARY OF BACKGROUND DATA:NBL, the most common extracranial solid malignant tumor in children, covers a wide spectrum of tumors with significant differences in anatomical localization, organ or vessel involvement, and tumor biology. Complete surgical resection of the primary tumor is an important part of NBL treatment, but maybe hazardous, prone to complications and its role in high-risk disease remains debated. Various surgical guidelines exist within the protocols of the different cooperative groups, although there is no standardized operative report form to document the surgical treatment of NBL. METHODS:After analyzing the treatment protocols of the SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology pediatric cooperative groups, important variables were defined to completely describe surgical biopsy and resection of NBL and their outcomes. All variables were discussed within the Surgical Committees of SIOP Europe International Neuroblastoma Study Group, Children's Oncology Group, and Gesellschaft fuer Paediatrische Onkologie und Haematologie - German Association of Pediatric Oncology and Haematology. Thereafter, joint meetings were organized to obtain intercontinental consensus. RESULTS:The "International Neuroblastoma Surgical Report Form" provides a structured reporting tool for all NBL surgery, in every anatomical region, documenting all Image Defined Risk Factors and structures involved, with obligatory reporting of intraoperative and 30 day-postoperative complications. CONCLUSION:The International Neuroblastoma Surgical Report Form is the first universal form for the structured and uniform reporting of NBL-related surgical procedures and their outcomes, aiming to facilitate the postoperative communication, treatment planning and analysis of surgical treatment of NBL.

Button battery (BB) ingestion is a preventable pediatric health hazard with important morbidity and mortality due to complications. We present 3 pediatric patients with a complicated course after BB ingestion and discuss current guidelines. Urgent endoscopic removal is necessary for every BB impacted in the esophagus. A new strategy before endoscopic removal is the administration of honey or sucralfate. During endoscopy, rinsing the esophageal mucosae with acetic acid can neutralize the alkalic environment and prevent late complications. Prevention of ingestion needs to be pursued by increasing awareness and changing legislation of packaging of BB.

Introduction: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. Materials & Methods: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. Results: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. Conclusions: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours. (C) 2021 Elsevier Ltd, BASO similar to The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.

BACKGROUND AND STUDY AIMS:Transanal irrigation (TAI) is used in children to treat constipation and incontinence. Belgium has 2 systems available: Colotip® (cheaper, however not designed for TAI) or Peristeen®.PATIENTS AND METHODS:This patient-control switch study is the first to compare 2 TAI systems. Children regularly using Colotip® for TAI were asked to participate, after consent, a visual analogue scale (VAS) rating the system and a 2-week diary (fecal continence, self-reliance, time spent on the toilet, pain, Bristol stool scale, irrigation volume and frequency of enema) were completed. Non-parametric statistics were used.RESULTS:Out of 26 children using Colotip®, 18 (69%) children participated and 5 refused (fear n=1, satisfaction Colotip® system n=7). Of these 18 children (interquartile range: 3-18 years, median 12.5 years, 9 girls) 5 patients stopped Peristeen® (pain n=1, fear n=1 and balloon loss n=3) and 2 were lost from follow up. Dropouts and included patients showed no statistical difference. In the 11 remaining patients, pseudo-continence (p 0.015), independence (p 0.01) and VAS score (p 0.007) were significantly better with Peristeen®, no difference was found in time spent on the toilet (p 0.288) and presence of pain (p 0.785).CONCLUSIONS:In children Peristeen® offered significantly higher pseudo-continence and independency. 30% refused participation because of satisfaction with the Colotip® and 30% spina bifida patients reported rectal balloon loss due to sphincter hypotony. To diminish Peristeen® failure, a test-catheter could be of value. Considering Colotip® satisfaction, both systems should be available. Patient selection for Peristeen® needs further research.

Aim The aim of this study was to search for any association between demographic, clinical, and therapeutic characteristics of patients with a low-type anorectal malformation (ARM) and their long-term bowel function. Methods In this retrospective study, 108 patients were contacted, of which 80 patients (74%) were included. Demographic, clinical, and therapeutic information was obtained from the patients’ medical records. The standardized Rintala questionnaire (resulting in a bowel function score (BFS)) was used to evaluate bowel function. Data was analyzed with SPSS 26 (significance level = 0.050). Results 80 patients (21 boys (26.3%)) were included, 70 (87.5%) presented with a rectoperineal fistula, 9 (11.3%) with a rectovestibular fistula, and 1 (1.2%) with anus imperforatus. The Mann-Whitney U test showed a significant negative impact of trimming of the anorectum (p = 0.003) and the presence of a developmental disorder (p = 0.013) on bowel function. Patients with sacral/spinal anomalies also showed a negative trend on the BFS score (p = 0.086), but was not significant probably due to a small patient group. The other characteristics (sex, prematurity, time of diagnosis, preoperative interventions, dehiscence, time of operation, and ARM type) were not significant. Multiple linear regression confirmed these results. Conclusions Trimming and the presence of a developmental disorder have a significant negative impact on the bowel function of patients with a low-type ARM. A negative trend was observed in patients with sacral/spinal anomalies. These results may help the clinician in determining the prognosis for patients with low-type ARM, and, if confirmed by later studies, may result in a different surgical approach.

Diffuse peritoneal and especially periportal invasion of Burkitt lymphoma is very rarely seen. We present a case of a 16-year-old boy presenting with abdominal pain and constipation, without improvement after enemas. Abdominal ultrasound revealed a diffuse infiltrating peritoneal mass, omental caking, ascites, and a periportal mass, confirmed by computed tomography (CT). No lymphadenopathies were seen. Biopsy was performed and the diagnosis of Burkitt lymphoma was made. Although very rare, it is important to include lymphoma in the differential diagnosis of diffuse peritoneal and periportal invasion in children, as it can mimic peritoneal carcinomatosis.

Background/Aims : In childhood, clinical presentation of intestinal polyps is variable. Painless rectal red blood loss is the most common presenting sign. Most polyps are sporadic, isolated and benign. However, it is important to correctly identity exceptions. Hare inherited polyposis syndromes need to be recognized because of their increased risk of intestinal and extra-intestinal malignancies. Furthermore, a correct diagnosis and treatment of rare gastro-intestinal malignancies is crucial. Methods : Between 2016 and 2018 we encountered 4 different types of intestinal polyps. A database search was performed and patient tiles were checked for clinical manifestations and histopathology. Literature was searched to recapitulate red flags for these syndromes, probability of underlying genetic disorders and diagnostic criteria. Results : Between 2016 and 2018, 28 patients presented at the Ghent University Hospital with 30 juvenile polyps. Furthermore, we diagnosed juvenile polyposis syndrome, Li Fraumeni syndrome and familial adenomatous polyposis (FAP) in 1 patient each, whilst 2 FAP patients were in follow-up. Each of these diagnoses has a different lifetime risk of (extra)-intestinal malignancy and requires a different approach and follow-up. Histopathology and genetic testing play an important role in identifying these syndromes in pediatric patients. Conclusion : Although most intestinal polyps in childhood are benign juvenile polyps that require no follow-up, rare inherited syndromes should be considered and correctly diagnosed since adequate follow-up is necessary to reduce morbidity and mortality from both gastrointestinal and extraintestinal complications and malignancies.

Introduction/Aim: Children experience important anxiety before surgery. Anxiety and pain are positively correlated. Serious gaming is a non-pharmacological intervention to prepare children and parents for an operation. We aimed to evaluate the effectiveness of the serious game CliniPup (R) on anxiety and pain in children undergoing ambulatory surgery. Method: A prospective randomized controlled pilot trial in 72 children aged 5 to 11 years old scheduled for day-care surgery (general surgery, dentistry, otorhinolaryngology, urology) was performed. Participants were randomly assigned into 3 groups: A (CliniPup (R)), B ("Empty game" without educational information), or C (no game, oral information at the outpatient clinic, current standard of care). Anxiety, pain, and behaviour were evaluated by validated instruments at six time-points: T0: baseline, T1: 1 week preoperatively, T2: at hospital admission, T3: before discharge, T4: 1 week postoperatively, T5: 1 month postoperatively. Results: After playing the game (T1), the estimated mean anxiety score (VASa) was lower in Group A (1.9 units) versus Group B (2.7 units). The estimated mean VASa at T1 for Group A was 2.6 units lower compared to Group C (p = 0.003). For Group B, VASa levels were 1.8 units lower than in Group C (p = 0.045). After correction for "surgery type", Group A continued to show a significantly lower VASa compared to Group C (p = 0.044). On the other time points, no difference in anxiety and pain were observed, nor in post-hospitalization behaviour. Conclusion: Children that played the CliniPup (R) game one week before surgery had a significant reduction in preoperative anxiety after playing the game, but not on the other time points. No differences on peri-operative pain were observed during the different time points. Type of Study: Randomized Trial. (C) 2019 Elsevier Inc. All rights reserved.

PURPOSETo evaluate the impact of surgeon-assessed extent of primary tumor resection on local progression and survival in patients in the International Society of Pediatric Oncology Europe Neuroblastoma Group High-Risk Neuroblastoma 1 trial.PATIENTS AND METHODSPatients recruited between 2002 and 2015 with stage 4 disease > 1 year or stage 4/4S with MYCN amplification < 1 year who had completed induction without progression, achieved response criteria for high-dose therapy (HDT), and had no resection before induction were included. Data were collected on the extent of primary tumor excision, severe operative complications, and outcome.RESULTSA total of 1,531 patients were included (median observation time, 6.1 years). Surgeon-assessed extent of resection included complete macroscopic excision (CME) in 1,172 patients (77%) and incomplete macroscopic resection (IME) in 359 (23%). Surgical mortality was 7 (0.46%) of 1,531. Severe operative complications occurred in 142 patients (9.7%), and nephrectomy was performed in 124 (8.8%). Five-year event-free survival (EFS) ± SE (0.40 ± 0.01) and overall survival (OS; 0.45 ± 0.02) were significantly higher with CME compared with IME (5-year EFS, 0.33 ± 0.03; 5-year OS, 0.37 ± 0.03; P < .001 and P = .004). The cumulative incidence of local progression (CILP) was significantly lower after CME (0.17 ± 0.01) compared with IME (0.30 ± 0.02; P < .001). With immunotherapy, outcomes were still superior with CME versus IME (5-year EFS, 0.47 ± 0.02 v 0.39 ± 0.04; P = .038); CILP was 0.14 ± 0.01 after CME and 0.27 ± 0.03 after IME (P < .002). A hazard ratio of 1.3 for EFS associated with IME compared with CME was observed before and after the introduction of immunotherapy (P = .030 and P = .038).CONCLUSIONIn patients with stage 4 high-risk neuroblastoma who have responded to induction therapy, CME of the primary tumor is associated with improved survival and local control after HDT, local radiotherapy (21 Gy), and immunotherapy.

Abstract Introduction No data are available on surgical management and outcomes of oesophageal atresia (OA) in Belgium. In collaboration with the Belgian Association of Paediatric Surgery (BELAPS) a retrospective study was organized to analyze data from children born with OA, with a 1-year follow-up. Materials and Methods Out of 17 centers in Belgium, 10 participated in this study. A novel checklist to collect data from OA patients born between the 1st of January 2010 and the 1st of January 2015 was created. Gross type A, B, C and D OA were included. Results One hundred children were included. Gross type C OA was seen in 86%. Associated malformations were present in 73%, most frequently cardiac malformations (52%). There was consensus regarding preoperative investigations, anastomotic technique, and transanastomotic tube placement. There was no consensus regarding genetic counseling, placement of a chest tube, and start of enteral feeding. There were six deaths in the first year of life. The majority of patients needed rehospitalization during the first year of life (85.9%) and there was a high occurrence of anastomotic strictures (51.6%). Conclusions This is the first study about management and outcome of OA in Belgium. Although the 1-year survival rate is high (93.8%), these data illustrate that OA has an important morbidity. Multidisciplinary follow-up and prospective studies/registries with longer follow-up are needed.