OBJECTIVE:This systematic review analyzed laparoscopic repair of primary hiatal hernia (HH) in children, focusing on presentation, diagnosis, surgical techniques and postoperative outcomes. METHODS:Studies published between 2000 and 2025 on laparoscopic repair of primary pediatric HH were identified according to PRISMA guidelines (PROSPERO: CRD420261295203). Due to heterogeneity in study design and outcome reporting, a meta-analysis was not feasible. Data were summarized descriptively. Selected comparisons used Fisher's exact test and odds ratios (ORs) with 95% confidence intervals (CIs)(p < 0.05) Results: Twenty-seven studies reporting 353 patients met the inclusion criteria. The evidence was of low certainty, consisting predominantly of case reports (n=12) and case series (n=10), with only five retrospective cohorts and no prospective studies. Estimated median age was 18.6 months (range 6 days-16 years). Gastroesophageal reflux disease was the most common symptom. Laparoscopic repair was performed in all patients, with 1.4% conversion rate and one intraoperative complication. Recurrence occurred in 21/331 (6.3%). Patch-reinforced repair showed a non-significantly higher recurrence rate than primary repair (OR 2.63, 95% CI 0.54-12.8; p=0.24). Esophageal stenosis was the most frequent postoperative complication (n=40/331; 12.1%) and was more common after Nissen-Rossetti than Thal fundoplication (OR 2.63, 95% CI 1.11-6.25; p=0.027), although heterogenous reporting and missing data limit this comparison. CONCLUSION:Laparoscopic repair of primary pediatric HH appears feasible, with low reported perioperative morbidity and acceptable recurrence rates, but evidence is low certainty. Fundoplication appears reasonable but the observed association of complete wraps with stenosis requires cautious interpretation.. Current evidence does not support routine patch reinforcement.
Background:In pediatric surgical oncology, intraoperative tissue assessment is limited by small specimen size and the absence of real-time histopathology. Ex vivo fluorescence confocal microscopy (FCM) provides rapid histology like imaging of fresh tissue, yet interpretation relies on expert visual assessment. We tested the hypothesis that deep learning applied directly to ex vivo FCM images enables automated, accurate, and spatially interpretable tumor detection in pediatric specimens. Methods:FCM images were prospectively acquired during routine clinical activity from 42 children, yielding 141 surgical and biopsy specimens. For this analysis, anonymized mosaics were retrospectively collected and manually annotated as malignant, benign, or healthy tissue by expert, then decomposed into 256 × 256 pixel tiles at 0.5 µm per pixel. A convolutional neural network was trained, validated, and tested on stratified datasets. Performance metrics were calculated at the tile level using accuracy, sensitivity, specificity, and F1 score. Tile level activation heatmaps were generated to localize regions influencing predictions. Results:Overall, 243,659 tiles were analyzed, including 124,348 malignant, 48,441 benign, and 70,870 healthy tissue tiles. The independent testing dataset comprised 84,751 tiles. Malignant tissue was identified with 91.35% accuracy, 95.88% sensitivity, 86.28% specificity, and an F1 score of 92.13%. Healthy tissue achieved 94.41% accuracy and 95.58% specificity. Benign tissue showed 90.14% accuracy and 91.78% specificity. Heatmaps consistently highlighted at a cellular level the architectural distortion in malignant tiles. Conclusion:This work introduces the first pediatric integration of deep learning with ex vivo FCM, demonstrating the feasibility of rapid and interpretable tissue classification and providing the basis for future prospective intraoperative validation.
Introduction Anorectal malformations (ARMs) represent the most common form of congenital gastrointestinal malformations. ARMs range from mild anal stenosis to complex abnormalities involving the rectum, genitals, and urinary tract. The aim of this study was to investigate prevalence, mortality and associated anomalies of ARMs in the Finnish population. Materials and Methods The data were obtained on all live births, stillbirths and terminations of pregnancy due to fetal anomalies from registers maintained by the Finnish Institute for Health and Welfare, and Statistics Finland. All individuals diagnosed with ARM between January 1, 2004, and December 31, 2017, were included in the study. Results This population-based study identified 646 ARM cases, including 545 (84.4%) live births, 8 (1.2%) stillbirths, and 93 (14.4%) terminations of pregnancy. The total prevalence for ARM in Finland was 8.00 per 10,000 births, with no significant variation in the prevalence rates between 2004 and 2017. Neonatal mortality was 4.6% (n = 25), and infant mortality was 6.0% (n = 33). Neonatal and infant mortality were both associated with congenital heart defects (p = 0.02 and p<0.001) and VACTERL (p = 0.0002 and p<0.001, respectively). Postnatal survival rate for isolated ARM in the study population was 100%. Most common associated anomalies were urinary tract, heart, and limb malformations. Conclusion The overall prevalence of ARMs has remained stable in the Finnish population. Mortality during the neonatal and infant periods remains a significant concern, particularly among patients with severe associated anomalies, multiple congenital abnormalities, and syndromic disorders.
BACKGROUND:The optimal treatment of pilonidal disease remains uncertain, with recurrence rates up to 30% after resection or sinusectomy. This retrospective single-center cohort study evaluates the recurrence rate after graduation from our Pilonidal Care Clinic's (PCC) Minimally Invasive Pilonidal Protocol (MIPP). METHODS:We conducted a retrospective review of a single center's prospectively collected demographic and treatment data for patients with pilonidal disease who completed the MIPP between January 2021 and December 2024. Follow-up appointments were scheduled every 6 to 8 weeks, continuing until disease resolution and completion of laser ablation sessions. The analysis focused on patients who graduated. Patients still receiving care at study completion and those lost to follow-up were excluded. Recurrence was defined as any PCC visit after graduation with pilonidal disease on examination. Graduated patients who had not returned to the PCC were contacted via text message and queried about new symptoms since graduation. RESULTS:Out of the initial 884 patients, 8 patients presented for a second opinion only, 353 were lost to follow-up and 226 were still receiving care, leaving the study group of 297 patients who completed the protocol and graduated. They graduated after an average of 6.4 PCC visits, 2 pit-picking procedures and 5.3 laser epilations. The average disease duration before treatment was 11.4 months. The mean treatment duration was 12.3 months (SD 8), with a median of 10 months (IQR 6-17). The follow-up period for these patients ranged from 4 to 55 months. Of the 297 graduates, 17 patients (5.7%) returned to the PCC with recurrence concerns; however, only 12 (4.0%) showed physical evidence of recurrence: eight of them (66.7%) had new pits, and 4 (33.3 %) had both pits and a nidus. The other 5 showed no signs of pilonidal disease on examination. Recurrence occurred on average 16.3 months after graduating, and 9 of 12 patients with a recurrence were successfully re-graduated, 3 were LTFU. Among the 280 patients who did not revisit the clinic, 72 (25.7%) replied to the text survey: 68 (94.4%) of respondents remained disease-free, while 4 (5.6%) reported recurrence, two of whom sought medical care. CONCLUSION:Completion of the MIPP at this center is associated with a 4.0% recurrence rate within 16 months, with most recurrences mild and again resolved with minimally invasive care.
Introduction:The management of meconium ileus (MI) has yet to reach a unified consensus among pediatric surgeons, particularly regarding diagnostics and variants, conservative treatment strategies, surgical approaches, and clinical outcomes. This topic was therefore selected for the 2024 Consensus Session of the Annual Congress European Paediatric Surgeons' Association (EUPSA). Methods:A structured literature review was conducted across Cochrane, Medline, and EMBASE databases up to April 2024. Nine pediatric surgeons appointed by the European Paediatric Surgeons' Association (EUPSA) Consensus Committee reviewed the evidence and developed topic-specific recommendations relating to (1) diagnostics and variants, (2) Gastrografin® (Bracco Diagnostics Inc, Monroe Township, NJ) application and outcomes, and (3) surgery-related outcomes. Findings were presented at the 2024 EUPSA Annual Congress, with consensus statements formulated through iterative discussion and refinement, incorporating feedback from congress participants. Results:The consensus addresses antenatal and postnatal diagnosis, differentiation between simple and complex MI, conservative management using contrast enemas, surgical strategies, and short- and long-term outcomes, particularly in relation to cystic fibrosis (CF). Key recommendations include the use of contrast enema as the primary diagnostic modality and first-line treatment in uncomplicated cases, with surgical intervention indicated for complicated MI or failed conservative management. Enterostomy remains a widely used surgical approach, with technique guided by intraoperative findings and patient factors. Recommendations regarding stoma reversal emphasize the role of multidisciplinary assessment and preoperative evaluation including CF workup and confirmation of distal bowel patency. Conclusion:A peer-reviewed consensus statement on the diagnosis and management of MI has been developed, informed by current evidence and expert practice. This statement provides pragmatic guidance in the diagnosis and management of MI, including the role of contrast enema in uncomplicated cases and surgical intervention for complicated disease. It is recognized by the EUPSA as a valuable resource to support pediatric surgeons in clinical decision-making for this pathology.
Background:Bracing is the preferred treatment for pectus carinatum. However, few studies have compared outcomes of different bracing methods. We tested the hypothesis that the type of brace has no effect on outcomes. Methods:A retrospective study of children who underwent bracing during a 10-year period (2011-2021) at a multidisciplinary chest wall anomaly center was conducted. The characteristics and outcomes of patients treated with a pressure-guided orthotist-adjusted brace (FMF Dynamic Compression System [DCS]) were compared with those treated with a simpler, less costly, self-adjustable brace (Trulife). Pressure of correction (POC) was used to assess severity. A standardized follow-up protocol was used. Patients who were still in active bracing or had inadequate follow-up were excluded. Results:During the study period, 373 patients started bracing, with 241 (93.7% males) having sufficient data to assess outcomes (170 DCS, 71 Trulife). Bracing succeeded in 217 patients (90%). Of 24 patients who failed, the majority (17) were due to non-compliance. There were no significant differences in POC (DCS 5.27 ± 1.59 vs. Trulife 4.99 ± 1.62 psi, p = 0.219) or any other characteristics between the groups. Bracing success (DCS 89% vs. Trulife 93%, p = 0.329) and active bracing duration (5.41 ± 3.52 vs. 5.37 ± 4.28 months, p = 0.944) were similar between the two groups. Loss to follow-up was significantly higher in the Trulife group (40.7% vs. 23.7%). Use of the Trulife brace resulted in a cost savings of CAD 406,000.00. Conclusion:With the limitations of a retrospective study, a self-adjustable brace resulted in a similar success rate and bracing duration, when compared with a pressure-guided orthotist-adjusted brace, and was associated with substantial cost savings. Level of Evidence:IV.
Aim:The objective of this study is to characterize contemporary international practice in pediatric gastrostomy insertion and perioperative management. Methods:A cross-sectional electronic survey was developed by the European Pediatric Surgeons' Association (EUPSA) Network Office and distributed through the EUPSA network between June 2024 and January 2025. The survey contained 32 primary questions and addressed multiple aspects of the perioperative gastrostomy pathway. Responses were analyzed descriptively. Categorical variables were compared using chi-square or Fisher's exact tests, and continuous variables using the Mann-Whitney U test. Comparisons were performed between European and non-European, and high- and low-/middle-income settings. A two-sided p-value < 0.05 was considered statistically significant. Results:A total of 181 pediatric surgical respondents from 132 cities across 59 countries were included. Of these, 117/181 (64.6%) completed the survey in full. Preferred primary gastrostomy insertion technique varied, with percutaneous endoscopic gastrostomy without laparoscopy preferred most frequently (59/137, 43.1%). Open gastrostomy was reported more frequently in low- and middle-income than high-income settings (11/35, 31.4% vs. 3/102, 2.9%; p < 0.001) and in non-European than European settings (12/45, 26.7% vs. 2/92, 2.2%; p < 0.001). Reported timing of postoperative gastrostomy use varied substantially; enteral feeding was initiated earlier in European than non-European settings and in high-income than low- and middle-income settings (median 8 vs. 24 hours for both comparisons; p < 0.001 for both). Conclusion:Pediatric gastrostomy practice varies substantially across the perioperative pathway, with important differences across geographic and economic settings. These findings highlight persistent uncertainty in key aspects of care and support the need for prospective outcome-based research, prioritization of key areas, and consensus development.
Background:While non-surgical management is the mainstay of low-grade pancreatic trauma treatment, the optimal management of high-grade pancreatic trauma remains unclear. This study evaluates surgical versus non-surgical management and associated clinical outcomes in high-grade pancreatic trauma patients. Methods:This retrospective cohort study, utilizing the American College of Surgeons Trauma Quality Improvement Program Participant Use File (ACS-TQIP-PUF) database from 2017 to 2023, selected pediatric patients (<18 years) with high-grade pancreatic injuries (grade III-V) who underwent surgical versus non-surgical management, then stratified outcomes by hemodynamic status on arrival. Primary outcomes included 24-hour and in-hospital mortality. Secondary outcomes included non-operative management (NOM) failure rate, transfusion volume, complication rates, intensive care unit length of stay (ICU-LOS), and ventilator-free days (VFDs). Results:A total of 223 patients were included. In-hospital mortality occurred only in the surgical group (6.7% vs. 0%; p = 0.041); however, mortality events were infrequent and interpreted cautiously. The overall non-surgical management failure rate was 8.7%. Adjusted analyses demonstrated no statistically significant differences in total transfusion volume (β = 2.313, 95% CI: -15.351-19.976, p = 0.795, standard error [SE] = 8.894), ICU-LOS (β = - 1.871, 95% CI: -7.507-3.765, p = 0.510, SE = 2.829), or VFDs (β = - 4.315, 95% CI: -10.872-2.242, p = 0.191, SE = 3.249) between groups. Findings were consistent across hemodynamically stable and unstable subgroups. Conclusion:Non-surgical management may be a reasonable initial strategy in carefully selected pediatric patients with high-grade pancreatic trauma, with a low observed failure rate and comparable short-term resource utilization outcomes. However, findings should be interpreted cautiously within the constraints of a retrospective database study.
The optimal surgical treatment for achalasia in children remains controversial. Pneumatic balloon dilation (PBD) and Heller's myotomy (HM) are the most commonly used interventions, but comparative evidence is limited and based mainly on small observational studies.This study aimed to compare clinical outcomes of PBD and HM in children with achalasia through a systematic review and meta-analysis.PubMed, EMBASE, and Cochrane were searched through June 2025. Comparative studies evaluating PBD and HM in patients younger than 18 years were included. Outcomes of interest included symptom recurrence, reoperation, gastroesophageal reflux disease (GERD), and complications. Random-effects models were used due to expected heterogeneity.Nine observational studies comprising 317 patients were included (PBD: 184; HM: 133). HM was associated with lower odds of reoperation (odds ratio [OR] = 11.01; 95% confidence interval [CI]: 6.01-20.17; p = 0.0001) and symptom recurrence (OR = 8.00; 95% CI: 1.65-38.75; p = 0.0098). PBD was associated with a lower risk of GERD (OR = 0.14; CI: 0.02-0.98; p = 0.0477). No significant differences were observed in perforation rates. All studies were retrospective cohorts with heterogeneous reporting of techniques and outcomes, and some analyses were based on a limited number of studies.HM was associated with lower recurrence and reoperation rates, while PBD showed a lower risk of GERD. However, evidence is limited by small observational studies, with heterogeneity in treatment techniques, outcome definitions, and follow-up durations. Results should be interpreted cautiously, and prospective multicenter studies are needed to determine the optimal treatment strategy.
Introduction:This study aims to evaluate the incidence and characteristics of Nuss bar infections, identify factors associated with infection, and present our institutional management considerations. Methods:We conducted a single-center retrospective cohort study of patients undergoing the Nuss procedure between 1999 and 2024 at Amsterdam Pectus Center. All patients had a minimum follow-up of 6 months. Primary outcomes included incidence and management of infections. Secondary outcomes included infection characteristics (early- [<30 days] vs. late-onset [>30 days], superficial vs. deep), diagnostics, microbiology, factors associated with infection (sex, age, type of anesthesia, number of bars, bar dislocation, stabilizer position, use of sutures), impact on bar removal, incidence of allergies and terra firma-forme dermatosis. Results:Of 695 patients, 4.0% (n = 28/695) developed Nuss bar infections, at a median of 2.0 (0.0-13.5) months postoperative, presenting with erythema, pain, and exudate. Over time, infections shifted from early- to late-onset (p = 0.03). Deep infections (71.4%, n = 20/28) more often required surgery (p = 0.007); superficial infections were managed with a wide range of antibiotics. Bar dislocations (5.6%, n = 39/695) and stabilizer plate loosening (1.0%, n = 7/695) required reoperation and were associated with infections (p = 0.001). Operative time for bar removal was longer in infected patients (52.0 [32.0-68.0] vs. 35.0 [26.0-49.0] minutes, p = 0.03). One case of cobalt allergy was identified. Terra firma-forme dermatosis (0.4%, n = 3/695) was treated with alcohol wipes. Conclusion:Bar dislocation and stabilizer plate loosening are associated with Nuss bar infections. We present our institutional management considerations based on retrospective data and experience, requiring prospective validation. Accurate diagnostics, tailored antibiotic therapy, and consideration of alternative diagnoses remain essential.
Introduction:Although hypospadias is a common condition and pediatric urology fellowship training programs exist, they have recently been described as insufficient to mediate the necessary skills to master hypospadias surgery. Simulation-based education is increasingly promoted, but training is limited by simulators that typically focus on a single surgical technique. We aimed to develop and demonstrate a single adaptable simulator capable of simulating the six commonly used single-stage hypospadias repair methods. Methods:A model made from lamb tenderloin and chicken skin was used to simulate the six most common single-stage repair techniques: The Meatal-based Flap (Mathieu), the Tubularized Preputial Island Flap, the Island Onlay, the Meatal Advancement and Glanduloplasty (MAGPI) technique, the Koyanagi-Nonomura technique, and the Yoke repair technique. Simple adaptations were employed to modify the model, including sulcus and meatal repositioning, as well as suture- and resection-based skin adaptations. Results:The model could be adapted to simulate six major hypospadias correction techniques on one single platform. The model required minimal resources and preparation, making it accessible and cost-effective. Conclusion:With the animal tissue model presented, the simulation of six commonly used single-stage reconstruction techniques on one adaptable, easily accessible platform becomes possible. Formal validation studies are warranted prior to their incorporation into standardized training curricula.
Background:Telescopic intramedullary nailing is a cornerstone in the surgical management of pediatric osteogenesis imperfecta (OI), providing fracture prevention and deformity correction while accommodating bone growth. Nevertheless, implant-related complications remain a challenge affecting long-term outcomes. Methods:This retrospective single-center study evaluated 13 pediatric patients with OI who underwent femoral fixation using corkscrew-tipped telescopic nails (CTTN) between 2014 and 2021. Demographic characteristics, Sillence classification, surgical indications, radiological outcomes, rod migration, complication, and revision rates were analyzed. Implant survival was assessed using Kaplan-Meier analysis. Results:The study consisted of 7 female (53.8%) and 6 male (46.2%) patients, with a mean age of 9.2 ± 3.8 years. Most patients were classified as Sillence type IB (84.6%), with one patient each classified as Type IA and Type III. Rod migration occurred in three patients (23.0%) without distal joint penetration. Limited telescoping was observed in two femurs (15.2%). The revision rate was 23.0%, and the overall complication rate was 38.4%. Mean rod survival was 4.8 years (95% confidence interval [CI], 3.9-5.7). Conclusion:CTTNs appear to be a feasible option for femoral fixation in pediatric patients with OI, avoiding joint arthrotomy and demonstrating acceptable complication and revision rates. However, lateral rod migration remains a notable limitation, indicating that adjunct stabilization techniques may be required.
Introduction:Long-gap esophageal atresia (LGEA) is challenging due to the substantial distance between the proximal and distal esophageal pouches. Traditionally, it includes Gross types A and B, while inclusion of long-gap type C cases remains debated. In type C esophageal atresia (EA), the distal tracheoesophageal fistula (TEF) varies in location; when situated at the carina, the effective pouch distance may be markedly increased, making it one of the most complex anatomical subtypes. Introduction:We hypothesized that early primary anastomosis in type C with carinal fistula (TEFC) is feasible using thoracoscopic mobilization and intraoperative internal traction, and aimed to compare outcomes with type C cases where the TEF is higher on the tracheal wall (TEFT). Materials and Methods:We analyzed 134 EA patients treated thoracoscopically at the Wilhelmina Children's Hospital in Utrecht between 2012 and 2024, including 25 TEFC and 109 TEFT patients. TEF location was determined by perioperative tracheobronchoscopy, and the surgical approach was tailored accordingly. TEFC patients underwent thoracoscopic esophageal mobilization with intraoperative internal traction, while TEFT patients received conventional primary thoracoscopic anastomosis. Primary outcomes were early successful anastomosis, preservation of the native esophagus, and anastomotic leakage. Secondary outcomes included postoperative morbidity and clinical results during childhood. Results:All patients underwent successful thoracoscopic repair shortly after birth, without requiring esophageal replacement. In the TEFC group, single-stage anastomosis was achieved in 23 patients; two required temporary external traction followed by delayed repair. TEFC patients showed higher rates of anastomotic leakage (40% vs. 20.2%), recurrent stenosis (24% vs. 10.1%), and need for anti-reflux surgery (26.1% vs. 11.3%). Early feeding difficulties affected weight gain in both groups, with 10 to 20% underweight at 6 to 12 months and no significant group difference. By 5.5 years, growth improved markedly, with no TEFC patients and 8.6% of TEFT patients remaining underweight. Conclusion:Although TEFC represents a more complex subgroup with higher complication rates, early thoracoscopic anastomosis with preservation of the native esophagus is achievable in these long-gap type C patients. Routine tracheobronchoscopy may support accurate classification and optimized surgical planning.
Abstract:Tissue engineering represents a promising and innovative strategy for the treatment of complex congenital and acquired esophageal disorders, particularly in patients for whom conventional therapies have failed or current options for organ replacement remain inadequate. Current approaches have explored the use of synthetic and biological scaffolds, cell-based therapies, or combinations of both to promote tissue regeneration and restoration of function. Increasing evidence suggests that, in order to achieve a functional esophagus, hybrid strategies incorporating exogenous cell delivery may further enhance regenerative outcomes while simultaneously modulating inflammatory responses. Abstract:Importantly, the feasibility and effectiveness of esophageal tissue engineering are strongly influenced by the extent and depth of the defect. Encouraging results achieved in partial-thickness defects and patch repair models have supported early clinical translation. Reconstruction of full-thickness circumferential esophageal defects, as required in long-gap esophageal atresia, remains a challenge, primarily due to luminal stenosis, anastomotic leakage, and incomplete regeneration of organized muscular and neuromuscular structures. Recently, the first preclinical demonstration of secondary peristalsis and stent independence in a circumferential, cell-seeded esophageal graft in a growing large-animal model has provided important proof of concept, though translation to longer grafts and clinical application remains to be established. Further preclinical and translational research is required to develop safe, reproducible, and standardized strategies for circumferential esophageal replacement, supported by transparent and comprehensive reporting of experimental outcomes. Abstract:Consequently, further preclinical and translational research is required to develop safe, reproducible, and standardized strategies for circumferential esophageal replacement. Such efforts must be supported by transparent and comprehensive reporting of experimental outcomes to facilitate meaningful comparison and clinical translation. In this review, we summarize current esophageal tissue engineering strategies relevant to esophageal atresia, critically evaluate the available evidence, and discuss future directions in the field.
Objective:Previous studies exploring long-term outcomes of patients with omphalocele and gastroschisis suggest an ongoing burden of gastrointestinal symptoms but also highlight substantial heterogeneity in methodology and outcomes measurement, limiting comparability. Therefore, the aim of this study was to systematically evaluate patient-reported long-term gastrointestinal symptoms, using a validated questionnaire, and compare the scores to those of healthy controls. Methods:All children treated for omphalocele and gastroschisis between 1999 and 2022 were invited to complete the Pediatric Quality of Life Inventory GI Module, consisting of twelve gastrointestinal domains. Parent proxy-reports or child self-reports were sent by mail, appropriate for the participants' age. The scores were compared with a predefined American healthy cohort (n = 513). Significance threshold was p < 0.004 (Bonferroni-corrected). Results:In total, 45/131 (34.4%) respondents completed the questionnaire, of whom 20 had omphalocele (50.0% male, median age 10.4 years [IQR: 6.4-16.4]), and 25 had gastroschisis (56.0% male, median age 9.9 years [IQR: 6.2-16.8]). Omphalocele patients reported a similar mean total score (86.5 [ ± 15.9] vs. 88.6 [ ± 12.9], d = 0.15, p = 0.566) and similar domain scores (d < 0.35, p > 0.01) compared with healthy controls. Gastroschisis patients also reported a similar mean total score compared with healthy controls (84.9 [SD ± 10.9], d = 0.31, p = 0.112). However, they scored worse on the domains "gas and bloating" (d = 0.78, p = 0.001), "diarrhea" (d = 0.74, p = 0.002), and "constipation" (d = 0.51, p = 0.026). Conclusion:Omphalocele patients reported a low burden of long-term gastrointestinal symptoms, indicating that routine follow-up may not be required. In contrast, gastroschisis patients reported a higher prevalence of gas and bloating, diarrhea, and constipation compared with healthy controls, highlighting the importance of early recognition and targeted management of these symptoms.
Purpose:Giant omphaloceles (GOs) pose significant surgical challenges. Two common strategies to close the defect include applying a topical epithelializing agent with delayed closure and the Schuster staged closure, which approximates the abdominal wall within 2 weeks of life. Although patient selection is critical for the Schuster staged closure, it remains unclear whether the rapid rise in intra-abdominal pressure increases abdominal hernia risk. We hypothesized that abdominal hernia rates differ between closure strategies. Methods:We conducted a single-institution, retrospective cohort study comparing abdominal hernia rates in patients undergoing Schuster staged closure versus delayed closure between 2012 and 2022. Results:Among 21 patients who met the inclusion criteria, 9 underwent the delayed closure, and 12 underwent the Schuster staged closure. Demographics were similar, but birth weight and prenatal lung volumes differed between groups, with greater values in the Schuster group. Abdominal hernias occurred more frequently after Schuster closure (n = 9, 75%) versus delayed closure (n = 2, 22%; p = 0.03) within 2 years. Bilateral inguinal hernias were most common. Conclusion:Infants undergoing Schuster staged closure for GOs had a higher incidence of abdominal hernias than those with delayed closure. Given underlying physiologic differences between groups, these findings should be interpreted as hypothesis-generating rather than causal.
Hirschsprung disease (HD) is a rare malformation that requires complex corrective surgery. Low caseload, heterogeneous patient cohorts, and difficulties in linking long-term outcomes to the initial corrective surgery limit the available direct evidence for a volume-outcome relationship. Indirect evidence from adult colorectal cancer (CRC) surgery may be transferable, given the partial similarity of the procedures.Using the Institute for Quality and Efficiency in Health Care (IQWiG) V24-07 rapid report's methodology, this study evaluated the transferability of the volume-outcome relationship from adult CRC surgery to corrective surgery for HD in children. Differences between the two populations were identified and rated for their effect on the transferability of the volume-outcome relationship by an expert panel, following predefined IQWiG V24-07 categories.Consensus was reached for 6 out of 12 criteria. For "disease characteristics," "comorbidities," "intervention," "follow-up," and "concomitant treatments," differences were deemed irrelevant to transferability. For "diagnosis," differences were considered to increase procedural complexity in HD, thereby reinforcing the volume-outcome relationship. Tissue sampling and frozen-section pathology were identified as key challenges. Differences of opinion arose for the remaining criteria, particularly concerning whether the transferability of evidence is limited to rectal resections in adults or includes all colorectal resections. No consensus could be reached on the appropriate caseload threshold for corrective surgery for HD in children.Using the IQWiG V24-07 framework, this study provides strong support for the transferability of the volume-outcome relationship from adult colorectal surgery to corrective surgery for HD. Despite population differences, the underlying mechanisms linking surgical complexity and institutional experience are comparable, reinforcing the relevance of volume-based quality control in pediatric surgery.
Objective:Focal forms of congenital hyperinsulinism (FoCHI) are rare pediatric conditions managed using established metabolic and surgical protocols. To date, the use of a robotic approach for this surgery in children has not been described. We present our initial experience with robotic pancreatectomy, compared with the open approach, for the management of FoCHI. Methods:We conducted a retrospective, single-center study involving 25 children who underwent pancreatectomy for CHI between 2011 and 2024. Collected data included patient demographics, surgical details, complications, and post-operative outcomes. The Da Vinci Xi robotic system was used for all robotic procedures. Results:Ten patients underwent robotic pancreatectomy, and 10 underwent open surgery; five children treated laparoscopically were excluded. There were no significant differences between the robotic and open groups in median weight at surgery (7.7 kg vs. 7.3 kg, p = 0.7), median age (7 months vs. 5.9 months, p = 0.48), median operative time (298 minutes vs. 285 minutes, p = 0.5), length of stay (14 days vs. 14.5 days, p = 0.26), or time to postoperative feeding resumption (4 days vs. 4.5 days, p = 0.68). Intraoperatively, 80% of lesions were visible on the pancreatic surface. Two cases of incomplete resection occurred in each group; after multidisciplinary review, both patients in the robotic group required a second procedure. No intraoperative bleeding or conversions were reported. The overall cure rate was 90% in the robotic group and 80% in the open surgery group. Conclusion:This preliminary study suggests that robotic pancreatectomy for congenital hyperinsulinism may be both safe and feasible. The robotic approach provides enhanced visualization of small vessels, enabling limited dissection and safe resection. Given the sample size, further studies are required to confirm these findings.
The European Paediatric Surgeons' Association (EUPSA) and the European Reference Network for Rare Inherited and Congenital Anomalies (ERNICA) conducted a survey to assess the current surgical management and care practices for esophageal atresia (EA) in order to assess changes over the past decade.An online survey consisting of 56 questions was administered to EUPSA members and ERNICA representatives between March and September 2025. The questionnaire covered seven domains: center structure, preoperative assessment, surgical management of esophageal atresia and tracheoesophageal fistula (EA-TEF) patients and long-gap EA, postoperative care, long-term follow-up, and the management of complications. Results were compared to practices reported in a previous EUPSA Network Office survey in 2013.There were a total of 202 respondents from 41 countries with 60% from European Union countries. Compared with previous surveys, the routine use of preoperative bronchoscopy and the use of trans-anastomotic tubes were found to be significantly more common (both p < 0.001). A marked increase in preference for thoracoscopic techniques for EA-TEF was observed (p < 0.001). The routine use of chest drains, elective paralysis, and contrast studies prior to feeding initiation also rose significantly over the decade (p ≤ 0.004). Yet for many areas of care, there remains variation between surgeons, and structured long-term follow-up arrangements are not universal.The findings demonstrate increased standardization and suggest increased adherence to recommendations in the management of EA-TEF. However, significant variation persists in long-term follow-up, transition-to-adult-care programs, and structured quality-of-life assessment, highlighting areas for future harmonization across European centers.
