Medical imaging is crucial for surgical planning, yet surgeons struggle with mental transformation of 2D images into 3D representations, particularly in complex pediatric pelvic anatomy. This study evaluated perceived benefits of 3D imaging with tractography compared to conventional 2D MRI in pediatric pelvic tumor surgery. A nationwide study assessed three groups: non-medical personnel (n = 30), medical trainees (residents and fellows; primary analysis n = 61, excluding 3 medical students), and senior pediatric surgeons (n = 12). Using 3-Tesla MRI with specialized protocols including high-resolution CoroT2cube and diffusion tensor imaging, participants evaluated five clinical cases in both 2D and 3D formats using 7-point Likert scales. Statistical analysis employed Wilcoxon paired tests with Bonferroni correction. All groups showed significant improvements in perceived understanding with 3D imaging. Non-medical personnel scores increased from 4.24 (± 0.69) to 6.27 (± 0.28) (p < 0.001), particularly in understanding disease and surgical objectives. Medical trainees improved from 5.08 (± 0.61) to 6.42 (± 0.49) (p < 0.001), with enhanced understanding of surgical objectives and anatomical relationships. Senior surgeons’ scores increased from 5.02 (± 0.69) to 6.33 (± 0.52) (p < 0.001), showing significant improvements in preoperative planning and family communication. Effect sizes were substantial across groups (Cohen’s d: 2.80, 1.90, and 1.52 respectively), though the within-subject design likely contributes to effect size inflation. This study provides preliminary evidence for perceived 3D imaging value in pediatric pelvic tumor surgery. Improved anatomical comprehension among non-medical personnel may benefit informed consent, while enhanced visualization aids surgical education and planning. High surgeon acceptance (92%) suggests strong acceptability, though these exploratory findings require validation before implementation recommendations can be made. Prospective studies evaluating objective clinical outcomes, workflow integration and cost-effectiveness require further study.
Communication skills are essential non-technical competencies in pediatric surgery, yet formal training programs remain limited and inconsistent. This perspective article examines the critical role of communication across pediatric surgical subspecialties and proposes frameworks for implementing structured communication training in residency programs. We performed a narrative review of published literature on communication skills in pediatric surgery and synthesized existing communication frameworks applicable to surgical training. Expert perspectives were gathered from an international group of pediatric surgeons representing diverse subspecialties including prenatal counseling, neonatal surgery, pediatric urology, and pediatric surgical oncology. We identify specific communication challenges unique to pediatric surgery, including prenatal counseling, neonatal intensive care discussions, sensitive urological conditions, and pediatric oncology. Existing frameworks such as SPIKES for delivering bad news and EMPATHY for non-verbal communication provide evidence-based tools adaptable to pediatric contexts. Current training approaches remain largely informal and mentor-dependent, with significant variability between programs. Pediatric surgery training programs should integrate structured communication curricula with defined learning objectives, simulation-based practice, and regular feedback mechanisms to prepare trainees for the unique communication demands of caring for children and their families.
BACKGROUND:"Textbook Outcome" (TO) is a composite surgical quality measure encompassing intraoperative/postoperative complications, length of stay, resection completeness, and perioperative mortality. While established in adult surgical oncology for standardized outcome reporting, TO has not been employed in pediatric surgery. This study evaluated TO for neuroblastoma resections involving image-defined risk factors (IDRFs). METHODS:Children with neuroblastoma and IDRFs who underwent resection at our institution from 2012-2022 were included. Textbook Outcome was defined as achieving >90% total resection without intraoperative/postoperative complications, 30-day readmission, length of stay exceeding the 75th percentile, or mortality. Patient data, perioperative information, and postoperative outcomes were extracted from institutional records. RESULTS:Among 69 patients analyzed, 32 (46.4%) achieved TO. Specific IDRFs negatively impacted TO achievement: duodenopancreatic block infiltration (p < 0.001), porta hepatis infiltration (p = 0.001), vena cava encasement (p = 0.01), and >3 IDRFs (p = 0.007). High-risk neuroblastoma patients showed increased risk of not achieving TO (p = 0.003). The primary barrier to achieving TO was postoperative complications, which affected 81.1% of those who did not achieve TO, with 23% classified as Clavien-Dindo grade 3 and 6.7% as grade 4. Median length of stay was 7 (range 4-73) days; no intraoperative mortality occurred. CONCLUSIONS:Textbook Outcome achievement was <50%, highlighting the complexity of care in this population. Textbook Outcome may provide healthcare providers and families new perspectives on pediatric cancer center quality. As a potentially useful but exploratory quality measure, TO assessment for neuroblastoma requires validation in larger multicenter studies before implementation across institutions to enhance surgical standardization and to improve overall care quality.
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.
BACKGROUND:Textbook Outcome (TO) has emerged as a composite quality metric for benchmarking perioperative performance in adult hepatobiliary surgery. Its role in pediatric oncologic liver surgery remains insufficiently explored and TO has never been defined for hepatoblastoma resection. METHODS:Single-center cohort study including children undergoing curative-intent liver resection for hepatoblastoma. Textbook Outcome after hepatoblastoma resection (TO-HB) was defined as an all-or-none composite comprising: (i) no postoperative complications (CCI = 0), (ii) no prolonged length of stay (≤procedure-specific 75th-percentile), (iii) no reoperation within 90 days, (iv) no unplanned readmission within 90 days, and (v) no 90-day mortality. Case-mix adjustment was performed using ridge-penalized logistic regression with pre-specified covariates. Model performance was evaluated in terms of overall accuracy and calibration, including the Brier score and calibration-in-the-large, with bootstrap internal validation, to support risk-adjusted benchmarking analyses. RESULTS:TO-HB was achieved in 60/108 patients (55.6%). Failure to achieve TO-HB was predominantly driven by postoperative complications (38/48; 79%) and prolonged length of stay (18/48; 38%). TO-HB rates decreased with increasing surgical complexity, including higher PRETEXT stage and more extensive hepatectomy types. Risk-adjusted predictions were narrowly distributed around the overall TO rate (mean predicted probability 0.556), with preserved population-level calibration. The observed-to-expected ratio for TO-HB was 1.00, corresponding to a risk-standardized TO-HB rate of 55.6% (95% CI 42.4-71.5%). CONCLUSIONS:TO-HB is a feasible and stringent benchmark for perioperative quality in hepatoblastoma surgery. Failure to achieve TO-HB is predominantly driven by postoperative morbidity rather than mortality or reintervention, highlighting postoperative complications as target for quality improvement.
BACKGROUND:Extrahepatic Portal Vein Obstruction (EHPVO) is the leading cause of prehepatic portal hypertension in children, causing gastrointestinal (GI) bleeding, splenomegaly, and thrombocytopenia. Meso-Rex Bypass (MRB) restores hepatopetal flow, but long-term outcomes remain inconsistently reported. We conducted the first systematic review of long-term outcomes after MRB in pediatric EHPVO. METHODS:Following PRISMA guidelines, six databases were systematically searched. Prospective and retrospective studies on pediatric EHPVO patients (0-18 years) undergoing MRB were included. Technical aspects, complications, and functional outcomes were analyzed. RESULTS:Forty studies (1998-2024), encompassing 1403 MRB operations, were reviewed. Shunt patency was achieved in 87% (1229/1403), with thrombosis in 9% and stenosis in 5%. Recurrent GI bleeding occurred in 13% (92/730). Median follow-up was 30 months (range 1-134). Improvements were observed in platelet counts (24/24 studies), spleen size (decreased in 18/18 studies), leukocytes (8/8), and hemoglobin (4/4). Portal pressures decreased in all eight studies evaluating it, though mean postoperative values remained above 10 mmHg. Growth studies (n = 4) showed maintained or improved anthropometric parameters. Five patients (0.3%) died, with one MRB-related death. CONCLUSIONS:MRB is an effective, durable treatment for pediatric EHPVO with high patency and low mortality. It reverses hypersplenism and improves hematologic parameters while restoring physiologic portal flow. Future research should standardize long-term, multidisciplinary follow-up protocols.
BACKGROUND:Xanthogranulomatous pyelonephritis (XGP) is a rare chronic destructive granulomatous pyelonephritis. Paediatric experience is limited and mostly historical. The largest published series reported nephrectomy in 95.4 %. Whether this radical-only paradigm still reflects best practice in the cross-sectional imaging and minimally invasive surgery era is unclear. MATERIAL AND METHODS:We retrospectively reviewed all paediatric patients (≤16 years) treated for XGP at four tertiary paediatric-surgery centres of Assistance Publique - Hôpitaux de Paris (AP-HP), the university hospital trust of the greater Paris region (Bicêtre, Robert-Debré, Necker-Enfants Malades and Trousseau), between January 2005 and December 2024. Diagnosis required a compatible cross-sectional imaging pattern and/or histological confirmation. Demographics, prior uropathy, clinical and microbiological findings, imaging, treatment strategy (radical, parenchyma-sparing, or purely conservative), perioperative course and follow-up were analysed. RESULTS:Twelve children were included (9 male, 3 female; M:F 3:1; median age 47 months, range 10 months-6 years). The left kidney was involved in 11/12 (91.7 %). Pre-existing uropathy was documented in 7/12 (58.3 %). All patients had fever; nephrolithiasis was identified in 8/12 (66.7 %), with Proteus mirabilis being the predominant isolate (4/12, 33.3 %). All received broad-spectrum intravenous antibiotics. Nine children (75 %) underwent nephrectomy - by open approach (n = 4), retroperitoneoscopy (n = 4) or robot-assisted laparoscopy (n = 1) - while three (25 %) were managed without nephrectomy (percutaneous drainage; ureteric stent and delayed ureteroscopy; Cohen reimplantation for severe vesicoureteric reflux). Intra-operative complications occurred in 3/9 surgical patients (33.3 %; 25 % of the whole cohort). At median follow-up of 47 months, compensatory contralateral hypertrophy was universal, no recurrent infection occurred in the parenchyma-sparing group, and one child (8.3 %) progressed to stable CKD stage 3. CONCLUSIONS:In this contemporary multicentre cohort, paediatric XGP affected very young, predominantly male children with left-sided disease and was diagnosed earlier than in historical series. Nephrectomy remains the cornerstone for diffuse, non-functional disease, but minimally invasive approaches were feasible and a parenchyma-sparing or conservative strategy appeared feasible and safe in highly-selected children with focal disease, preserved differential function, or a clearly correctable underlying uropathy. We propose a pragmatic decision framework integrating differential function, focality and the presence of a treatable causal lesion. This series challenges the historical paradigm of systematic nephrectomy in paediatric XGP and supports a more individualized approach guided by renal function and disease focality.
Management of liver tumors in children is well-defined, with clear treatment protocols established by organizations such as the International Society of Pediatric Oncology-Liver group (SIOPEL), the Children's Oncology Group (COG), and the Japanese Study Group for Pediatric Liver Tumors (JPLT). However, no structured approach exists for hilar lymph node (LN) resection. Surgical practices vary among centers, reflecting the adult setting. This systematic review aims to examine the current evidence on hilar lymphadenectomy in pediatric patients with primary liver tumors and to assess LN positivity rates and their associations with recurrence and survival. METHODS:A structured literature search was conducted on MEDLINE, Embase, and Web of Science (2004-January 2025) using keywords: lymph node, hepatoblastoma, hepatocellular carcinoma, and liver tumor. Patients under 21 years were included. The study protocol was registered on PROSPERO (CRD42024501895), and Rayyan software supported screening and review. RESULTS:Eight studies (880 patients) were included. Diagnoses were hepatoblastoma (HB, n = 277), hepatocellular carcinoma (HCC, n = 507), and other tumors (n = 96). LN dissection details were available for 431 patients: 349 (80.9%) had no metastases. In the HB group, 43% underwent hilar LN dissection with 0% positivity. In HCC, 33.6% had positive nodes. In other tumors, 7.8% showed LN involvement. Data on survival impact were limited. CONCLUSIONS:In HB, routine LN dissection may be unnecessary due to universally negative nodes. For HCC, the one-in-three positivity rate supports further evaluation of lymphadenectomy. Evidence remains limited for other tumors. Prospective multicenter studies using standardized protocols are needed to define the role of LN evaluation beyond HB.
To compare the risks, complications, and feasibility of minimally invasive and open surgery for resection of Wilms tumor, we systematically reviewed comparative studies assessing the oncologic outcomes of minimally invasive (MIS) versus open surgery and performed both a meta-analysis and a critical assessment of the MIS surgical landscape in the field of pediatric urology. A systematic review of current literature was performed using PubMed, EMBASE, Google Scholar, Cochrane Collection, and Web of Science databases. Of 310 articles screened, a total of 9 studies (5 retrospective, 2 prospective, and 2 database/population-level retrospective reviews) were included in the meta-analysis. Heterogeneity was considered minimal between studies, and adjustments were made when necessary. Rates of intra-operative tumor spillage, positive margins, complications, recurrence, and survival were similar between MIS and classical open surgery. Mean length of hospital stay and operative times were significantly lower in MIS, with a mean difference of.96 days and 22.48 min, respectively (p < 0.001). The mean number of retrieved lymph nodes was significantly lower in MIS vs open surgery, with a difference of 3 nodes recorded (p = 0.003). Although lymph-node retrieval sampling was found to be reduced in MIS Wilms tumor resection, there were no differences in complications or post-operative outcomes when comparing MIS vs classical open surgery. We conclude that outcome metrics are considered equivalent in MIS and classical open surgery, though operative surgical times and total hospital stay are shorter with MIS resection. Further evidence-based prospective studies and quality reporting of lymph-node retrieval are crucially warranted as MIS becomes increasingly adopted.
Objective: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. Methods: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. Results: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. Conclusion: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.
Adrenocortical carcinoma (ACC) is a rare, aggressive pediatric malignancy. Advanced ACC requires multimodal treatment, including surgery and systemic chemotherapy including cisplatin, etoposide, doxorubicin, and mitotane. This is associated with significant gastrointestinal toxicity, resulting in many patients being unable to complete scheduled therapy. Often, supplemental nutrition is required if oral intake during treatment is poor. We assessed the frequency of nutritional supplement use in pediatric patients treated for advanced ACC. This was a retrospective observational study of patients with ACC treated at St. Jude Children’s Research Hospital over 10 years (2012–2022). Patient demographics, treatment received, and the need for supplemental enteral or parenteral nutrition were reviewed. A total of 18 patients with ACC were treated from 2012 to 2022, with 11 having advanced ACC. 54.5
Hepatoblastoma is the most common primary liver cancer in children, with an incidence of approximately 1.5 cases per million children per year. Most cases are sporadic, typically presenting at a median age of 18 months, with only 5% occurring after 4 years of age. Clinical presentation often includes an abdominal mass and, less commonly, abdominal pain, weight loss, jaundice and precocious puberty. Low birth weight is a significant risk factor, along with genetic conditions such as Beckwith-Wiedemann syndrome, Simpson-Golabi-Behmel syndrome, familial adenomatous polyposis and trisomy 18. Screening protocols for hepatoblastoma are recommended for children with predisposing conditions. Medical imaging is crucial for hepatoblastoma diagnosis and staging, with abdominal ultrasonography being the initial modality of choice, followed by abdominal contrast MRI for detailed evaluation and monitoring. Chest computer tomography is indicated to evaluate potential lung metastases. The Pretreatment Extent of Disease (PRETEXT) system is employed for hepatoblastoma staging and for guiding treatment strategies such as surgical resection and chemotherapy. Patients with advanced hepatoblastoma may require liver transplantation. Advancements in surgery and chemotherapy have improved survival rates, with 5-year survival rates exceeding 80-90% in localized disease. However, challenges remain in treating individuals with high-risk and metastatic hepatoblastoma. Ongoing research into treatment stratification, the introduction of novel therapies, including targeted and immune therapies, and the application of otoprotectants are essential to address refractory or recurrent hepatoblastoma and to increase the overall survival of patients. Long-term quality of life and the management of treatment-related sequelae are becoming increasingly important as survival rates improve.
INTRODUCTION:Surgery is pivotal in the management of hepatoblastoma (HB) although approaches and techniques may vary. The Liver Group of the International Society of Pediatric Oncology (SIOPEL) and the International Pediatric Surgical Oncology (IPSO) group collaborated to develop an online survey to investigate specific technical aspects and challenging indications for HB surgery, with the goal of establishing shared guidelines. METHODS:An electronic survey with 50 items was developed to address preoperative assessment and specific surgical issues. All questions were multiple-choice, allowing for multiple answers in complex cases. The survey was distributed via the SIOPEL and IPSO mailing lists. RESULTS:Overall, 10% of the mailing lists participated (52 respondents), including 28 transplant (LT) surgeons and 24 non-LT surgeons from 25 countries, globally. Around 40% (20/52) work in high-volume centers (> 10 hepatic resections/year, and > 10 liver transplants/year). For a mass at the origin of the suprahepatic veins, LT is preferred over resection (48 vs. 12), while a mass with satellite nodules favors liver resection (52 vs. 15). Non-LT surgeons prefer LT for masses at the hilar plate, whereas LT surgeons choose resection (p < 0.05). IVC replacement is favored by transplant surgeons in case of infiltration (p = 0.043), mostly with heterologous venous grafts (p = 0.004). DISCUSSION:The heterogeneity of responses underscores the lack of a standardized approach to the various surgical scenarios encountered in the surgical management of HB. Further analysis will enhance the understanding of how different management strategies affect outcomes and promote the creation of evidence-based guidelines for HB resection practices.
BACKGROUND:Central venous access (CVA) is an integral part of the management of various pediatric diseases. One of the commonly used CVAs is subcutaneous ports (SP). The adequate catheter tip location has been described ideally to be placed at the junction of the superior vena cava and right atrium (known as Cavo-atrial junction (CAJ)) confirmed by intraoperative fluoroscopy. In the pediatric population, the intravascular catheter length estimation is challenging due to the wide variance in body habitus and it is very important to properly place the CVC to eliminate the risk of complication. Our study aims at accomplishing an accurate formula to preoperatively predict the CVA length based on the insertion site and patient's height. METHODS:This is a prospective study including 134 patients less than 21 years old who underwent placement of SP at St Jude Children's Research Hospital during the period from August 2022 to July 2023, either using the right internal jugular vein (RIJV) or left subclavian veins (LSCV). The intravascular catheter length (in cm) was measured using the grading on the catheter lines. Formulas were developed using the line measurement and patient height for each of the techniques, and the formulas were compared to previously published formulas. These formulas were tested to validate their reliability and accuracy. RESULTS:The formula developed for the RIJV demonstrated a predictive accuracy within 1 cm for 50% of the CVCs, while that for the LSCV notably surpassed existing methods, achieving a high prediction within 1 cm for 71.5% of CVCs. CONCLUSION:Pediatric intravascular CVC length can be estimated by formulas suggested by the present study based on the insertion side and patient height, limiting x-ray exposure during CVC insertion and decreasing potential complications. LEVEL OF EVIDENCE:III.
BACKGROUND:Post-operative muscle paralysis aims to reduce anastomotic complications following primary esophageal atresia (EA) repair. This study evaluates the impact of paralysis on outcomes in children enrolled in the EUPSA Esophageal Atresia Registry (EAR). METHODS:Patients with type B, C, and D EA enrolled in the EAR between 2014 and 2017 who underwent primary EA repair were included. They were divided into two groups based on the use of paralysis (Group P) or not (Group NP). Comparisons included demographics, associated malformations, surgical approach, complications, and hospital stay. Multivariate logistic regressions analyses were performed. RESULTS:Of 316 patients, 126 were in Group P and 190 in Group NP. Group P had significantly lower gestational age and birth weight. Ventilation duration (7.9 ± 10.4 vs. 4.0 ± 4.4 days; p < 0.001) and hospital stay (43.7 ± 93.7 vs. 27.5 ± 31.8 days; p < 0.001) were significantly longer in Group P. The overall complication rate was higher in Group P (39.7 % vs. 28.4 %; p = 0.036), but mortality rates did not differ significantly. Post-operative paralysis was not associated with a significant reduction in anastomotic leaks or strictures. CONCLUSIONS:Post-operative paralysis may provide stability in high-risk cases, such as neonates with low birth weight or associated malformations, but it does not significantly reduce surgical complications and may prolong recovery. Its use should be carefully considered and limited to individualized scenarios where the benefits outweigh the risks.