Journal Of Pediatric Surgery[JOURNAL]
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200 papers
Preventable pediatric trauma transfers in a rural state.
Krinock DJ, Walker SC, Edington M … +4 more , Birisci E, Grappe S, Wolf LL, Wyrick DL
J Pediatr Surg · 2026 Jun · PMID 42331311 · Publisher ↗
BACKGROUND: In rural settings, children with traumatic injuries are often first evaluated by providers at non-pediatric-specialized facilities who determine if the patient's condition warrants transfer. We sought to eval...
BACKGROUND: In rural settings, children with traumatic injuries are often first evaluated by providers at non-pediatric-specialized facilities who determine if the patient's condition warrants transfer. We sought to evaluate potentially preventable transfers (PTs) in the pediatric trauma population of our rural state. METHODS: We performed a single-site retrospective cohort study at a level 1 pediatric trauma center, evaluating patients <18 years who presented as activated trauma transfers from referring facility between 1/2018-12/2023. PTs were defined as patients who were discharged from the Emergency Department or admitted for <24 h without requiring operative or procedural intervention, cross-sectional imaging or ICU admission following transfer. Bivariate analysis compared PTs to patients characterized as unpreventable transfers (UPTs) and multivariable logistic regression determined predictors of PT. RESULTS: We included 762 patients with mean age 12 years (IQR 5,15) and 65% (n = 496) male. Twenty-five percent (n = 191) were characterized as PTs, with 112 patients discharging from the Emergency Department after transfer. PTs had a lower median injury severity score than UPTs (6 versus 17, p < 0.001). Mechanism of injury was not associated with PT (p = 0.172). Injury to the head (p < 0.001), chest (p = 0.009), abdomen (p = 0.012), appendicular skeleton (p < 0.001), and nonaccidental trauma (p = 0.002) were associated with UPT. CONCLUSIONS: One-quarter of activated trauma transfers were classified as potentially preventable, with over half of those patients being discharged from the Emergency Department. Several factors were associated with PTs, most notably injury severity. Telemedicine consultation with a pediatric trauma team may provide a potential method to improve pre-transfer triage for mild and moderately injured children. LEVEL OF EVIDENCE: Level III, Prognostic/Epidemiological.
Enhancing access to a pediatric surgical specialty through virtual care and outreach clinics: A pilot quality improvement initiative at a tertiary children's hospital.
Ibrahimova A, Cheng WK, Albayati H … +10 more , Nguyen AV, Parrotta I, Golzar N, Abrahams B, Hinada P, Afshar K, MacNeily A, Masterson J, Schroeder S, Kim S
J Pediatr Surg · 2026 Jun · PMID 42320744 · Publisher ↗
BACKGROUND: Families in rural and remote communities face travel and financial barriers to pediatric surgical care. This quality improvement initiative aimed to enhance access through virtual and outreach care in pediatr...
BACKGROUND: Families in rural and remote communities face travel and financial barriers to pediatric surgical care. This quality improvement initiative aimed to enhance access through virtual and outreach care in pediatric urology. METHODS: Using statistical process control charts, we compared mean wait times and travel distances before (2018) and after virtual care implementation (2021) for pediatric hydronephrosis and testicular pathology consultations. Cost savings from virtual and outreach care, and travel reductions from outreach care were analyzed. Caregiver surveys assessed satisfaction, accessibility, and perceived cost savings. RESULTS: Among 1,288 patients (618 in 2018; 670 in 2021), X-bar charts demonstrated process stability with a sustained reduction in mean travel distances from 240 km to 143 km (-40.4%). Mean wait times remained stable at 115 days (p=0.42) with decreased variability after virtual care implementation, which yielded an estimated $41,700 CAD in family savings from reduced vehicle and parking costs in 2021. Outreach clinics generated additional savings of $8,600-$10,900 CAD in total, reducing average travel distances by 649-1,445 km (82-88%) per visit. By 2024, average wait times increased to 149 days as consultation volumes rose by 56%. Caregiver satisfaction remained high (81.5% in-person vs 82.4% virtual), with 87.1% reporting cost savings and 82.7% reporting time savings. CONCLUSIONS: Virtual and outreach care reduced travel and financial burdens without compromising satisfaction or wait times, supporting hybrid care as a sustainable approach to pediatric surgical service delivery. Rising 2024 wait times under increased demand underscore the need for workflow optimization and resource scaling to sustain timely access.
Assessment of postoperative outcomes of LAT-HSL in the treatment of unilateral inguinal hernia in boys: A multicenter prospective randomized controlled study.
Hang T, Yu J, Hong ZH … +1 more , Lin H
J Pediatr Surg · 2026 Jun · PMID 42320743 · Publisher ↗
BACKGROUND: Single-port laparoscopic hernia needle extraperitoneal high ligation of the hernia sac (LH) has been widely adopted for indirect inguinal hernia due to its concealed surgical incision and rapid postoperative...
BACKGROUND: Single-port laparoscopic hernia needle extraperitoneal high ligation of the hernia sac (LH) has been widely adopted for indirect inguinal hernia due to its concealed surgical incision and rapid postoperative recovery. Laparoscope-Assisted Trans-scrotal Hernia Sac ligation (LAT-HSL) is an alternative surgical approach for high ligation of the indirect inguinal hernia sac performed via a scrotal approach under laparoscopic assistance. However, head-to-head comparative data on the clinical outcomes of LAT-HSL versus LH for pediatric inguinal hernia remains limited. OBJECTIVE: To Comparing the efficacy, safety and economic outcomes of the LAT-HSL and LH procedures in the treatment of unilateral indirect inguinal hernia in boys aged 1-3 years. METHODS: This multicenter, single-blnd, prospective randomized controlled trial was conducted at the pediatric surgery departments of two tertiary children's medical centers in China. Male pediatric patients aged 1-3 years with primary unilateral indirect inguinal hernia (confirmed by abdominal ultrasonography) admitted between April 2024 and June 2025 were enrolled, with a total of 104 patients included in the final analysis. Eligible patients were randomly allocated to either the LH group (n = 53) or the LAT-HSL group (n = 51), with a 6-month postoperative follow-up period. The primary outcomes were postoperative hernia recurrence rate and overall complication rate within 6 months of surgery. Secondary outcomes included intraoperative parameters (pneumoperitoneum duration, operative time, mean end-tidal carbon dioxide [ETCO], and incidence of intraoperative hypocapnia [ETCO < 35 mmHg]), postoperative recovery indicators (length of hospital stay, Face, Legs, Activity, Cry, Consolability [FLACC] pain scores at 12 and 24 h postoperatively, and Manchester Scar Scale (MSS) scores at 1 week, 2 weeks, and 1 month postoperatively), and medical costs. Normally distributed continuous data were expressed as mean ± standard deviation and compared using the t-test. Non-normally distributed continuous data were presented as median (interquartile range [IQR]) and compared using the Mann-Whitney U test. Categorical variables were analyzed using Pearson's chi-square test and Fisher's exact test. Univariate and multivariate regression models were employed to identify independent factors associated with intraoperative parameters. RESULTS: A total of 104 patients were included in the study, with 53 cases (20.0 [16.0,31.5] months])in the LH group and 51 cases (16.0 [12.0,23.0] months) in the LAT-HSL group. Compared to the LH group, the LAT-HSL group had significantly longer operative time (30.0 [24.0,42.0] min vs 24.0 [18.0,30.0] min, P = 0.001) and hospital stay (1.0 [1.0,2.0] days, 1.0 [1.0,1.0] days, P = 0.003). While, LAT-HSL group had significantly shorter pneumoperitoneum time (1.5 [1.0,2.5] min vs 24.0 [18.0,30.0] min, P < 0.001) and smaller proportion of normal-low ETCO values (2 cases [3.9%] vs 12 cases [22.6%], P = 0.005). At Jiaxing University Affiliated Jiaxing Women and Children Hospital where disposable hernia needles are utilized, the consumable cost in the LAT-HSL group was significantly lower than that in the LH group (1569.1 [1569.1,1569.1]CNY vs 1168.5 [1078.8,1569.3]CNY, P = 0.001). CONCLUSION: LAT-HSL and LH have comparable short-term safety and efficacy for the treatment of unilateral indirect inguinal hernia in boys aged 1-3 years. LAT-HSL is associated with a significantly shorter pneumoperitoneum duration and lower risk of intraoperative hypocapnia, and may reduce medical consumable costs in centers using disposable laparoscopic hernia needles. Therefore, LAT-HSL represents a safe, effective, and economical surgical option for this patient population.
Clinicopathological predictors of cervical lymph node metastases and disease persistence in pediatric papillary thyroid carcinoma: A single-center cohort study.
Carreño M, Canestrari M, Korman L … +11 more , Cotignola L, Zanon E, Dujovne N, Gazek N, Formia I, Donato M, Dardanelli E, Orozco E, Tedesco E, Lopez J, Ayarzábal V
J Pediatr Surg · 2026 Jun · PMID 42309483 · Publisher ↗
BACKGROUND: Papillary thyroid carcinoma (PTC) in children frequently presents with advanced locoregional disease, including cervical lymph node metastases. Identifying clinicopathological factors associated with nodal in...
BACKGROUND: Papillary thyroid carcinoma (PTC) in children frequently presents with advanced locoregional disease, including cervical lymph node metastases. Identifying clinicopathological factors associated with nodal involvement and disease persistence may help refine surgical management and postoperative surveillance. METHODS: We conducted a retrospective cohort study of pediatric patients undergoing total thyroidectomy with or without lymph node dissection at a tertiary referral center. Clinicopathological variables including tumor size, histological subtype, multifocality, extrathyroidal extension, and lymphovascular invasion were analyzed for their association with cervical lymph node metastases and disease-free survival. RESULTS: The cohort included 83 patients with a mean age of 12.2 ± 2.8 years, with a predominance of females (73.5%). Cervical lymph node metastases were identified in 61.5% of patients, most commonly involving both central and lateral compartments. Tumor size, multifocality, extrathyroidal extension, lymphovascular invasion, and diffuse sclerosing variant were significantly associated with nodal metastases (p < 0.01). Patients younger than 10 years demonstrated a higher frequency of lateral neck metastases (p = 0.014). During a median follow-up of 1.9 years, structural disease persistence occurred in 37.3% of patients. In the univariate Cox regression analyses, lymphovascular invasion (HR 4.84; 95% CI 1.46-15.98; p = 0.01) and central lymph node metastasis (HR 3.28; p = 0.028) were associated with reduced disease-free survival. CONCLUSIONS: In pediatric papillary thyroid carcinoma, several pathological features such as tumor size, multifocality, extrathyroidal extension, lymphovascular invasion, and diffuse sclerosing variant are strongly associated with cervical lymph node metastases. Lymphovascular invasion and central compartment nodal involvement were significant predictors of reduced disease-free survival, highlighting their potential value for risk stratification and postoperative management. LEVEL OF EVIDENCE: Level III (retrospective cohort study).
A comparison of the modified Duckett versus conventional Duckett procedure for severe hypospadias: A retrospective single-centre cohort study.
Liu P, Fan Y, Zhang S
J Pediatr Surg · 2026 Jun · PMID 42309482 · Publisher ↗
OBJECTIVE: To compare the operative outcomes of the modified and conventional Duckett procedures for severe hypospadias, and to evaluate the clinical value of the modified Duckett (M-Duckett) procedure. METHODS: We condu...
OBJECTIVE: To compare the operative outcomes of the modified and conventional Duckett procedures for severe hypospadias, and to evaluate the clinical value of the modified Duckett (M-Duckett) procedure. METHODS: We conducted a comparative study of 132 paediatric patients with severe hypospadias who had a minimum follow-up of 24 months and underwent different surgical procedures at the Department of Paediatric Surgery, The First Affiliated Hospital of Zhengzhou University, between January 2018 and February 2023. Based on the surgical technique, 75 patients constituted the M-Duckett group, while the remaining 57 comprised the Duckett group. The M-Duckett procedure incorporates refinements to the conventional technique, primarily pertaining to the anastomosis between the native urethral meatus and the neourethra, as well as the architectural reconstruction of the external urethral meatus. Preoperative baseline data, perioperative parameters, and postoperative outcomes were compared between the two groups. RESULTS: No significant differences were observed in preoperative baseline data and perioperative parameters between the two groups. However, the incidence of postoperative urethral stricture was significantly lower in the M-Duckett group (9.3%) than in the Duckett group (22.8%; χ = 4.573, P = 0.032). Furthermore, a bell-shaped urinary flow curve was observed in a significantly higher proportion of patients in the M-Duckett group (69.4%) compared to the Duckett group (45.8%; P = 0.048). No other postoperative outcomes showed significant intergroup differences. CONCLUSION: In comparison to the Duckett procedure, the M-Duckett procedure is associated with a lower rate of postoperative urethral stricture and a higher proportion of bell-shaped uroflow curves, without increasing the risk of other postoperative complications or leading to an undesirable penile appearance.
Bedside predictors of in-hospital mortality in neonatal gastric perforation: development of a clinical scoring system.
Bao Ngan PC, Mai DN, Tram NQ … +2 more , Chieu VM, Nguyen TT
J Pediatr Surg · 2026 Jun · PMID 42303091 · Publisher ↗
BACKGROUND: Neonatal gastric perforation has high mortality, and bedside tools for risk stratification at diagnosis remain limited. OBJECTIVE: To develop and internally validate a clinical prediction model for mortality...
BACKGROUND: Neonatal gastric perforation has high mortality, and bedside tools for risk stratification at diagnosis remain limited. OBJECTIVE: To develop and internally validate a clinical prediction model for mortality using variables available at diagnosis, and to characterize post-operative vasoactive inotropic score (VIS) trajectories. METHODS: In this retrospective cohort study (2015-2025), neonates with surgically confirmed gastric perforation were included. A multivariable logistic regression model was developed and internally validated using bootstrap resampling (1000 iterations). Clinical utility was evaluated using decision curve analysis. A simplified bedside score was derived, and survival analyzed using Kaplan-Meier methods. VIS trajectories were examined in a secondary analysis. RESULTS: Mortality was 41.5% (27/65). The final model included delivery room intubation (OR 3.26, 95% CI 1.04-10.94), onset-to-diagnosis interval (OR 1.03 per hour, 95% CI 1.00-1.05), and shock at diagnosis (OR 4.18, 95% CI 1.11-20.48). Discrimination was moderate (AUC 0.747; optimism-corrected 0.719), with acceptable calibration. The simplified score stratified patients into low- and high-risk groups (22.9% vs 63.3%, p = 0.001). Decision curve analysis demonstrated positive net benefit across clinically relevant threshold probabilities (approximately 25-60%). In secondary analyses, non-survivors showed marked escalation in post-operative vasoactive support, with higher VIS1 (110 [IQR 58-165] vs 9 [0-30]) and ΔVIS (65 [40-125] vs 0 [-2 to 9]). CONCLUSION: A three-variable bedside model enables early risk stratification in neonatal gastric perforation. Post-operative VIS dynamics describe hemodynamic deterioration and may inform clinical reassessment.
Epidemiology of necrotizing enterocolitis in multi-birth preterm infants: A 22-year retrospective study in Switzerland (2000-2021).
Milosevic M, Stalder T, Haefeli S … +7 more , Kidszun A, Krause T, Starvaggi C, Berger S, Adams M, Kessler U, Swiss Neonatal Network
J Pediatr Surg · 2026 Jun · PMID 42297234 · Publisher ↗
BACKGROUND: Necrotizing enterocolitis (NEC) is a life-threatening gastrointestinal condition predominantly affecting preterm infants. Although multiple births are increasingly common, their specific contribution to NEC r...
BACKGROUND: Necrotizing enterocolitis (NEC) is a life-threatening gastrointestinal condition predominantly affecting preterm infants. Although multiple births are increasingly common, their specific contribution to NEC risk remains unclear. OBJECTIVE: To assess the incidence and mortality of NEC in preterm multiple-birth infants in Switzerland over two decades (2000-2021), and to determine whether multiplicity contributes to NEC risk. METHODS: We conducted a retrospective cohort study of 15,635 infants born before 32 weeks' gestation, using prospectively collected data from the Swiss Neonatal Network. RESULTS: Of the cohort, 32.8% (n = 5133) were multiple births. The overall NEC incidence was 2.84%, with no statistically significant difference between singletons (2.9%) and multiples (2.7%). NEC-related mortality was high (35.8%) and similar across groups (35.3% in multiple births vs. 36% in singletons). However, within twin pairs, if one twin developed NEC, the second twin's odds increased sixfold (OR 6.1, 95% CI 2.4-16.2). NEC incidence remained stable over the study period. CONCLUSION: Over a 22-year period, incidence and mortality of NEC in preterm multiple-birth infants in Switzerland remained stable and did not differ significantly from those in singletons. Multiple gestation was not a risk factor for NEC. However, within twin pairs, the diagnosis of NEC in one twin was associated with a sixfold increased odds (OR 6.1, 95% CI: 2.4-16.2) of NEC in the co-twin, raising the estimated risk from 2.7% to 15%. This clustering suggests shared environmental or biological triggers. These findings underscore the importance of vigilant surveillance in the co-twin of an affected infant.
Postoperative benefits of cryoanalgesia in minimally invasive repair of pectus excavatum: Results from a tertiary referral center.
Papadakis L, Shnawa G, Masiakos PT … +8 more , Cameron D, Pietrantonio NM, McDevitt G, El Abdellaoui Y, Gauthier L, Ryan DP, Doody DP, Griggs CL
J Pediatr Surg · 2026 Jun · PMID 42288292 · Publisher ↗
INTRODUCTION: Minimally invasive repair of pectus excavatum (MIRPE) is associated with significant postoperative pain, and opioid use remains common despite Enhanced Recovery After Surgery (ERAS) protocols. Cryoanalgesia...
INTRODUCTION: Minimally invasive repair of pectus excavatum (MIRPE) is associated with significant postoperative pain, and opioid use remains common despite Enhanced Recovery After Surgery (ERAS) protocols. Cryoanalgesia has emerged as a promising adjunct to improve pain control, but its comparative effectiveness within established ERAS pathways remains incompletely defined. We aimed to evaluate opioid use and recovery outcomes following cryoneurolysis compared to a thoracic epidural-based ERAS protocol. METHODS: We performed a retrospective cohort study of pediatric and young adult patients undergoing MIRPE from 2017 to 2024. Outcomes included inpatient opioid use and outpatient opioid prescribing (MME/kg), length of stay (LOS), and postoperative care utilization. Groups were compared using appropriate univariate tests, and multivariable models adjusted for age, Haller index, and calendar year of surgery. RESULTS: Among 245 patients (median age 16.0 years), 124 (50.6%) received cryoanalgesia. Baseline characteristics were similar between groups. Cryoanalgesia was associated with significantly reduced opioid use and prescribing (inpatient: 6.10 vs. 18.55 MME/kg; outpatient: 1.70 vs. 7.91 MME/kg; both P < 0.0001) and shorter LOS (2.01 vs. 3.25 days, P < 0.0001), with these associations remaining significant after adjustment for age, Haller index, and calendar year of surgery. Routine outpatient care needs and surgical complication rates were similar between groups. Cryoanalgesia was associated with a modest increase in unplanned postoperative visits, without a difference in the proportion of patients affected (28.1% without cryoanalgesia vs 22.6% with cryoanalgesia, P = 0.322). CONCLUSION: Cryoneurolysis was associated with substantial reductions in opioid use and shorter hospitalization without increased routine follow-up. A modest increase in unplanned visits warrants further study. These findings support cryoanalgesia as an effective adjunct to multimodal pain management in MIRPE. IRB APPROVAL STATEMENT: This protocol was approved by the Institutional Review Board at Mass General Brigham, and a waiver of informed consent for medical records review was obtained (Protocol #: 2025P001162).
Esophageal atresia and parental psychological distress in the neonatal period.
Arntzen T, Mikkelsen A, Birketvedt K … +4 more , Pripp AH, Haugen G, Emblem R, Diseth T
J Pediatr Surg · 2026 Jun · PMID 42285283 · Publisher ↗
BACKGROUND: Esophageal atresia (EA) is a rare congenital malformation requiring neonatal surgery and intensive care. Parents may experience distress, but there are few studies on influencing factors. This study aimed to...
BACKGROUND: Esophageal atresia (EA) is a rare congenital malformation requiring neonatal surgery and intensive care. Parents may experience distress, but there are few studies on influencing factors. This study aimed to assess psychological distress in parents of neonates with EA and identify predictors of parental distress. METHODS: Parents of neonates with EA born 2019-2025 were invited. Psychological distress was assessed with GHQ-30 and traumatic stress with IES-R at a median of 9 days after birth. Clinical data were collected from medical records. RESULTS: Out of 126 invited parents, 113 participated (58 mothers, 55 fathers). Rates of GHQ-30 cases (score≥6) were higher in mothers (82%) than fathers (70%) (p < 0.005), with higher mean Likert scores (43.0 vs 36.8, p = 0.027). Mothers reported higher traumatic stress than fathers (31.1 vs 24.8, p = 0.036), with 46% vs 30% > 33, indicating symptoms of probable posttraumatic stress reactions or adjustment disorder. Maternal traumatic stress was associated with older age and primiparity, while paternal traumatic stress was linked to pregnancy complications and longer time from birth to admission to the surgical ward. Paternal GHQ-cases were characterized by lower gestational age (p = 0.034) and lower paternal age (p = 0.036), whereas no significant characteristics for maternal GHQ-30 cases were identified. CONCLUSION: Both mothers and fathers experienced substantial psychological distress after birth. Mothers reported higher distress than fathers. We advise that all EA parents are offered consultation with mental health professionals, in addition to extra attention being given to older and first-time mothers, and younger fathers of premature neonates.
Disparities in engagement with an artificial intelligence-based chat intervention post-appendectomy.
Lin AM, Rachwal B, Asti L … +2 more , Zettler-Greeley CM, Berman L
J Pediatr Surg · 2026 Jun · PMID 42276356 · Publisher ↗
PURPOSE: Artificial intelligence (AI)-based text messaging, or "chat," in post-appendectomy care has been shown to decrease preventable emergency department visits. As digital health grows, it is important to assess the...
PURPOSE: Artificial intelligence (AI)-based text messaging, or "chat," in post-appendectomy care has been shown to decrease preventable emergency department visits. As digital health grows, it is important to assess the impact of technology-based interventions across patients with diverse backgrounds and socioeconomic risk factors. The purpose of this study was to evaluate engagement with an AI-based texting platform for patients with risk factors related to social determinants of health (SDoH). METHODS: An AI chat was designed with input from pediatric surgeons and specialty nurses to address postoperative concerns after appendectomy. It was implemented from March 2022 to December 2023. Caregivers or patients could respond to chats. Mann-Whitney U tests compared continuous variables, and Fisher's exact tests compared categorical variables. An ordinal logistic regression model was fit to determine which patient demographics and SDoH-related risk factors (area deprivation index [ADI] and four social vulnerability index [SVI] themes) were associated with chat engagement. RESULTS: Of the1023 chat eligible post-appendectomy patients, 497 caregivers/patients did not engage, 229 caregiver/patient engaged with the chat once, and 297 engaged with more than one chat. Independent factors associated with decreasing engagement were increasing patient age (odds ratio, 95% confidence interval; 0.96 [0.93-0.99]), higher SVI socioeconomic score (0.28 [0.16-0.49]) and having Medicaid insurance (0.68 [0.52-0.88]). However, higher SVI housing & transportation score (2.08 [1.26-3.43]) was associated with increased engagement. CONCLUSION: Lower chat engagement was observed among non-White patients and those with lower socioeconomic status. These are the same groups who may be at higher risk for complications. Future work should focus on optimizing feasibility and accessibility of technological interventions for patients with SDoH-related risk factors.
Regulatory gene analysis in enteric neural crest cells stratified by GDNF activation status: An insight into Hirschsprung disease pathophysiology.
Randall S, Giron A, Shaul D … +4 more , Yu P, Flyer Z, Ahmad H, Schomberg J
J Pediatr Surg · 2026 Jun · PMID 42269811 · Publisher ↗
BACKGROUND: Incomplete migration of enteric neural crest cells (ENCCs) into the colon underlies the pathogenesis of Hirschsprung disease, resulting in aganglionosis and disordered intestinal motility. Although activation...
BACKGROUND: Incomplete migration of enteric neural crest cells (ENCCs) into the colon underlies the pathogenesis of Hirschsprung disease, resulting in aganglionosis and disordered intestinal motility. Although activation of the glial cell line-derived neurotrophic factor (GDNF) pathway has been implicated in neurosphere differentiation, specific regulatory genes governing ENCC differentiation and proliferation remain poorly characterized. This study seeks to elucidate regulatory genes and pathways involved in neural crest cell differentiation, furthering our understanding of GDNF's role in enteric nervous system development. METHODS: Mus musculus (mice) ENCC gene expression data were obtained from Gene Expression Omnibus (GEO), a public functional genomics repository, to compare GDNF (n = 13) and non-GDNF (n = 12) samples in dataset GSE34208. We calculated repressive tendency scores using TRIAGE R to rank genes based on their typical epigenetic repression and identify unique regulatory genes in GDNF+ and GDNF- neurospheres. Regulatory pathways were then characterized using Gene Ontology (GO) database. RESULTS: TRIAGE-based prioritization revealed greater similarity among GDNF + samples than between GDNF+ and GDNF- samples. GO enrichment analysis of 300 top-ranked genes from GDNF + samples demonstrated significant enrichment in biological processes relevant to ENS development. Homeobox (HOX) transcription factors emerged as a key regulatory gene class in the GDNF + condition, appearing consistently across pathways related to gut patterning and neuronal differentiation. CONCLUSION: Our analysis clarifies the regulatory mechanisms governing neural crest cell differentiation and proliferation and establishes a foundation for future research into the molecular regulation of enteric neural crest cell development and Hirschsprung disease pathophysiology. LEVEL OF EVIDENCE: III.
Patient reported outcomes after subtotal colectomy in pediatric therapy-resistant constipation: A combined retrospective and cross-sectional study.
Jonker CAL, de Jong JR, Pronk CEM … +3 more , Koppen IJN, Benninga MA, Gorter RR
J Pediatr Surg · 2026 Jun · PMID 42264138 · Publisher ↗
BACKGROUND: Current literature on outcomes after subtotal colectomy for therapy-resistant constipation (TRC) in the pediatric population is scarce. We aimed to review clinical outcomes, postoperative complications and he...
BACKGROUND: Current literature on outcomes after subtotal colectomy for therapy-resistant constipation (TRC) in the pediatric population is scarce. We aimed to review clinical outcomes, postoperative complications and health-related quality of life (HRQoL) after subtotal colectomy in children with TRC. METHODS: All children <18 years who underwent a subtotal colectomy for TRC from January 2011 till June 2024 were included and data were extracted from their medical files. Follow up by telephone was done in 2024. Clinical outcomes, postoperative complications and HRQoL after subtotal colectomy were evaluated using the Patient-Reported Outcomes Measurement Information System (PROMIS). Treatment success was defined as a defecation frequency >2 times per week and a fecal incontinence frequency <1 per 2 weeks. RESULTS: Forty children with TRC were included (78% female). Median age at subtotal colectomy was 16 years [IQR 13-17]. Treatment success following subtotal colectomy was achieved in 68% of patients at a median follow-up of 68 months [IQR 27-129]. Despite subtotal colectomy, 47% of patients still reported abdominal pain. Postoperative complications were observed in 63% of the patients, with major complications (Clavien-Madadi grade III-IV) occurring in 48% of them. HRQoL after subtotal colectomy was preserved in all PROMIS domains across all age groups. CONCLUSION: Treatment success after subtotal colectomy was achieved in 68%, however, approximately half of patients still reported postoperative abdominal pain and postoperative complications rates were high. Subtotal colectomy was associated with a favorable HRQoL across PROMIS domains.
Short term complications of persistent cloaca reconstruction - A single-institution review of 53 cloacal reconstructions.
Newman T, Staniorski C, Hisam B … +8 more , Bowser M, Ho C, Feng C, Badillo A, Mayhew A, Meyers MR, Levitt MA, Varda B
J Pediatr Surg · 2026 Jun · PMID 42259485 · Publisher ↗
PURPOSE: To describe short-term complications following cloaca repair and evaluate their relationship with cloaca complexity. METHODS: A prospective institutional registry of patients evaluated for persistent cloaca from...
PURPOSE: To describe short-term complications following cloaca repair and evaluate their relationship with cloaca complexity. METHODS: A prospective institutional registry of patients evaluated for persistent cloaca from 2020 to 2025 was used to identify patients undergoing primary repair at our institution. Early complications (prior to scheduled examination under anesthesia at 6-12 weeks) were graded by Clavien-Madadi scale to determine complication rate, severity, setting and type. RESULTS: Among 104 patients evaluated, 53 met inclusion criteria, undergoing cloacal reconstruction at median age of 0.7 years (IQR 0.6-1.5). 40% had complex cloaca (common channel ≥3 cm, 21/53) and 55% underwent urogenital separation (29/53). Median operative time was 307 min (ΙQR 206-414). Median length of stay was 4 days (IQR 2-7). Patients with complex cloaca had higher usage of urogenital separation (86%[18/21] v. 34%[11/32], p = 0.001) with longer operative time (445 v. 238 min, p < 0.001) and hospital stays (7 vs 2 days, p = 0.002). 39 complications occurred in 24 patients (45%). Patients with complex and moderate anatomy had similar complication rates (57% v. 38%, p = 0.16). Most complications were ≤Clavien-Madadi 3a (31/39, 79%) and occurred at similar rates in the intra-operative, inpatient and post-discharge settings. Most were related to surgery (34/39, 87%) with 38% of all complications (15/39) involving the genitourinary system (injury, infection or retention). Transfusion was the only complication associated with complex anatomy (33% v. 0%, p = 0.001). CONCLUSION: Early complications occurred in nearly half of patients following cloacal repair. Most events were minor and frequently involved the genitourinary system. These data can inform counseling, management and surgical techniques. IRB APPROVAL: Pro00015991.
Beyond the pull-through: A multi-institutional study of long-term surgical and functional outcomes in total colonic hirschsprung disease - A pediatric colorectal and pelvic learning consortium (PCPLC) study.
Ahmad H, Joachim A, Urrutia Gonzalez AY … +23 more , Shaul D, Austin K, Badillo A, Calkins CM, Crady RC, Durham MM, Frischer J, Gayer C, Grabowski JE, Harris JC, Hong CR, Kawaguchi AL, Langer JC, Nandivada P, Rana A, Reeder RW, Rentea RM, Rollins MD, Rosen N, Saadai P, Smith CA, Speck KE, Wood RJ
J Pediatr Surg · 2026 Jun · PMID 42251888 · Publisher ↗
PURPOSE: Total colonic Hirschsprung disease (TCHD), a severe Hirschsprung disease variant (∼5-10% of cases), is defined by aganglionosis of the entire colon. This study evaluates short-term surgical outcomes and long-ter...
PURPOSE: Total colonic Hirschsprung disease (TCHD), a severe Hirschsprung disease variant (∼5-10% of cases), is defined by aganglionosis of the entire colon. This study evaluates short-term surgical outcomes and long-term functional results in children with TCHD. METHODS: A cross-sectional retrospective cohort study was conducted on patients with TCHD with at least 4 years of follow-up enrolled at the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) institutions from 2017 to July 2025. Demographics, surgical details, and functional outcomes-including bowel management, toilet training, and stooling accidents-were collected. RESULTS: Of 75 patients meeting inclusion criteria, 63 underwent a pull-through procedure, with 32 occurring at a PCPLC site; demographics and associated diagnoses did not influence whether patients received pull-through. Among PCPLC patients, the most common procedures were Duhamel (n = 12, 37.5%), Yancey-Soave (n = 8, 25.0%), and Swenson (n = 8, 25.0%), with 59.4% performed laparoscopic-assisted. Four patients required redo pull-through (12.5%), 22 patients (68.8%) had 1 or more additional operations, and 1 (3.1%) had an anastomotic leak. Among non-diverted patients, rates of toilet training ranged from 47.2% at ages 4-7 to 92.9% at 12+ years, although up to 38.5-55.0% of patients continued to have stooling accidents. Common bowel management plans included botulinum toxin injection, fiber, and stool diversion. CONCLUSIONS: Pull-through procedures of patients with TCHD yielded generally favorable early and mid-term outcomes, with most patients achieving toilet training by 8-11 years. However, a subset required re-diversion or redo pull-through, and many continued to experience stooling accidents, highlighting ongoing functional challenges despite specialized colorectal care.
Impact of Trisomy 21 on post pull-through enterocolitis and bowel function in children with Hirschsprung disease: A PCPLC analysis.
Wallace MW, Short SS, Crady RC … +20 more , Reeder RW, Ahmad H, Austin K, Badillo A, Calkins CM, Durham MM, Fuchs JR, Gayer C, Grabowski JE, Harris JC, Hong CR, Rana A, Rentea RM, Rosen N, Saadai P, Smith CA, Speck KE, Velazco CS, Wood RJ, Rollins MD
J Pediatr Surg · 2026 Jun · PMID 42251887 · Publisher ↗
INTRODUCTION: We evaluated whether Trisomy 21 (T21) affected bowel management use, ability to toilet train for stool, and rates of Hirschsprung-Associated Enterocolitis (HAEC) in children with Hirschsprung disease (HD)....
INTRODUCTION: We evaluated whether Trisomy 21 (T21) affected bowel management use, ability to toilet train for stool, and rates of Hirschsprung-Associated Enterocolitis (HAEC) in children with Hirschsprung disease (HD). We hypothesized that T21 would be associated with increased rates of HAEC, bowel management program (BMP) use, and delayed toilet training for stool. METHODS: Children with HD in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database who underwent primary pull-through were included and stratified by T21 status: HD only versus HD + T21. Children requiring a redo pull-through, initially managed with a stoma, or those who had inadequate follow-up were excluded. HAEC episodes, toilet-training status, and BMP data recorded during individual PCPLC sites' clinical visits were analyzed. RESULTS: 339 children with HD and 48 with HD + T21 were included. No significant differences in patient demographics, age at diagnosis, transition zone, age/weight at pull-through, type of pull-through, or child opportunity index (COI) were identified. No significant differences in BMP or number of HAEC episodes were found. Children with HD + T21 were less likely to be toilet trained at their 4-7-year visit (22.2% vs. 52.6%, p = 0.002) and their 8-11-year visit (22.2% vs. 80.9%, p < 0.001). On multivariable regression, T21 independently predicted lower odds of toilet training for stool at age 4-7 years. CONCLUSIONS: Children with HD and T21 have delayed toilet training but have similar bowel management needs and HAEC rates to children with HD only, suggesting that differences in continence outcomes are not explained by increased enterocolitis burden or greater bowel management intensity. LEVEL OF EVIDENCE: Level 3 - Retrospective Comparative Study.
Association of small intestinal atresia and Hirschsprung disease in a multi-institutional colorectal registry.
Read Ivaturi M, Crady RC, Hussaini SF … +27 more , Ahmad H, Austin K, Badillo A, Calisto JL, Calkins CM, Darcy D, Durham MM, Fuchs JR, Gayer C, Grabowski JE, Harris JC, Hong CR, Kawaguchi AL, Rana A, Reeder RW, Rentea RM, Rollins MD, Rosen N, Saadai P, Smith CA, Speck EK, Velazco CS, Halaweish I, Gasior AC, Criss C, Wood RJ, Pruitt LCC
J Pediatr Surg · 2026 Jun · PMID 42251886 · Publisher ↗
PURPOSE: Unlike colonic atresia, concomitant jejuno-ileal atresia (JIA) and Hirschsprung disease (HSCR) is an under-recognized association. We used a multi-institutional patient registry to describe the incidence, clinic...
PURPOSE: Unlike colonic atresia, concomitant jejuno-ileal atresia (JIA) and Hirschsprung disease (HSCR) is an under-recognized association. We used a multi-institutional patient registry to describe the incidence, clinical characteristics, management, and outcomes of patients with both conditions. METHODS: We performed a retrospective review of patients with HSCR in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. Additional data was collected from participating sites regarding atresia management and outcomes. RESULTS: Of 1459 eligible patients with HSCR, 15 (1.0%) also had JIA. Median age at initial surgical consult and atresia diagnosis was 1 day (IQR 0.5-3.0 and 0.0-3.0, respectively). Median age at HSCR diagnosis was 58.5 days (IQR 16-147.5) compared to a median of 7 days (IQR 3.0-60.0) for patients with HSCR alone. Most patients had ileal atresia (N = 10, 66.7%), and total colonic HSCR (N = 9, 60.0%), although HSCR segment length was not consistently related to atresia location. After primary atresia surgery, 46.7% (N = 7) of patients had a complication within 30 days. At one year, 53.3% (N = 8) of patients required ongoing nutritional support, 55.6% (N = 5) had short bowel syndrome, 80.0% (N = 12) had at least one readmission, and 100% a reoperation. Twelve patients (80.0%) have undergone pull-through. CONCLUSION: Patients with both small intestinal atresia and HSCR demonstrated a higher likelihood of delayed HSCR diagnosis and an increased incidence of total colonic HSCR. A high index of suspicion is critical for timely diagnosis and management of this under-recognized population.
Does the need for early cutaneous vesicostomy correlate with poor urinary outcomes in girls with cloacal malformation?
Bain A, Tillman A, Sion A … +9 more , Read M, Chen R, Rachwal B, Bricker J, Thomas E, Jayanthi VR, Wood RJ, DaJusta DG, Fuchs ME
J Pediatr Surg · 2026 Jun · PMID 42251885 · Publisher ↗
INTRODUCTION: In the neonatal period, children with cloacal malformation may require bladder drainage with a vesicostomy. Little is known if vesicostomy creation in these patients affects long-term urinary outcomes, spec...
INTRODUCTION: In the neonatal period, children with cloacal malformation may require bladder drainage with a vesicostomy. Little is known if vesicostomy creation in these patients affects long-term urinary outcomes, specifically volitional voiding rates and continence rates. Our objective was to determine whether the requirement of vesicostomy at birth is a predictor of worse urinary outcomes for patients with cloacal malformation. METHODS: A retrospective review was performed of all patients with cloacal malformations being managed at a single institution from 2013 to 2024. Anatomic details and clinical outcomes were collected and compared between patients who required a vesicostomy in the neonatal period versus those who did not. RESULTS: A total of 159 patients were included. Patients with a vesicostomy were more likely to have a long common channel (54.8% vs 15.1%, p < 0.0001), higher rates of hydronephrosis (79.2% vs 44.7%, p = 0.0007) and hydrocolpos (87.5% vs 41.3%, p < 0.0001) at birth. Only 11.4% of patients in the vesicostomy cohort achieved volitional voiding and were dry at last clinic encounter, compared to 66.2% in the no vesicostomy cohort (p < 0.0001). Patients in the vesicostomy cohort had a higher likelihood of developing chronic kidney disease, requiring bladder augmentation and relying on clean intermittent catheterization to achieve continence. CONCLUSION: The need for a vesicostomy at birth in those with cloacal malformation is associated with decreased likelihood of achieving volitional voiding and worse renal function. This information will be helpful for counseling families on long-term urinary outcomes for these patients.
Malignant transformation of sacrococcygeal teratoma versus presacral teratoma in Currarino syndrome: Results of 'The SCT-study'.
van Heurn LJ, Derikx JPM, Hall NJ … +14 more , AbouZeid AA, de Campos Vieira Abib S, Chirdan LB, Fumino S, Kim SC, Muensterer O, Olivos M, StPeter SD, Twisk J, Vinit N, Yang T, Zani A, Ernst van Heurn LW, SCT-study consortium
J Pediatr Surg · 2026 Jun · PMID 42250912 · Publisher ↗
BACKGROUND: The risk of malignant transformation and recurrence of Sacrococcygeal Teratoma (SCT) is relatively high, while it is possibly lower in cases associated with Currarino Syndrome (CS). However, the existing lite...
BACKGROUND: The risk of malignant transformation and recurrence of Sacrococcygeal Teratoma (SCT) is relatively high, while it is possibly lower in cases associated with Currarino Syndrome (CS). However, the existing literature gives contradictory results. We aimed to examine the risk of malignant transformation in a large cohort of SCT and CS patients. METHODS: In a global retrospective cohort study, data of consecutive SCT patients and CS patients with presacral teratoma was obtained from 132 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence, or death due to malignancy was analysed for SCT and CS patients. The rate of malignant transformation was analysed with log-rank test and compared between groups. RESULTS: Of 3612 patients with presacral teratoma, 3388 entered analysis; 3183 SCT and 205 CS patients. The percentage of patients with malignant transformation at initial resection was higher in the SCT versus the CS group, 10·3 % and 31·9 % after one and two years, versus 4·2 % in CS patients after two years, respectively (p < 0.001). Histology in recurrent teratoma was malignant in 35·4 % (n = 114) of SCT patients and 5·9 % (n = 1) of CS patients (p = 0.005). Survival in both groups was equivalent at 94·9 % in SCT patients versus 96·9 % in CS patients (p = 0·343) CONCLUSION: The SCT-study shows that malignancy is more often present in SCT than in CS patients with an increasing risk of malignant transformation with age compared to CS patients in whom malignancy is rare. Recurrence after resection was more often malignant in the SCT group. LEVEL OF EVIDENCE: level III.
Trans-anastomotic tube feeding following repair of congenital duodenal obstruction: A 15-year single-centre cohort study.
Nowak A, Rex D, Shetty S … +1 more , Kulkarni A
J Pediatr Surg · 2026 Jun · PMID 42250629 · Publisher ↗
AIM: To evaluate the association between trans-anastomotic tube (TAT) placement and feeding outcomes following surgical repair of congenital duodenal obstruction (CDO). METHODS: A retrospective single-centre cohort study...
AIM: To evaluate the association between trans-anastomotic tube (TAT) placement and feeding outcomes following surgical repair of congenital duodenal obstruction (CDO). METHODS: A retrospective single-centre cohort study of neonates undergoing CDO repair between 2010 and 2024, grouped by intraoperative TAT placement. The main outcome was time to full enteral feeding (≥150 ml/kg/day). Secondary outcomes included time to full oral feeding, postnatal growth, postoperative complications, long line use, parenteral nutrition duration and length of stay with estimated hospital cost. Time-to-event outcomes were analysed using Kaplan-Meier methods and Cox proportional hazards regression adjusted for surgical era, gestational age, birth weight, and associated anomalies. No institutional protocol governed feed advancement or aspirate management in either group during the study period. RESULTS: 71 infants were included, of whom 25 (35%) received a TAT. Time to full enteral feeding was similar between groups (median 13 vs 12 days; adjusted HR 0.98, 95% CI 0.44-2.21). As a secondary outcome, time to full oral feeding was also similar (median 22 vs 16 days; adjusted HR 0.74, 95% CI 0.31-1.77). Parenteral nutrition duration was similar (median 5 vs 6 days). Growth and complication rates were similar. After adjustment, TAT use was not independently associated with hospital cost. Unadjusted higher costs in the TAT group were driven by longer HDU stay. CONCLUSION: In this cohort, TAT placement following CDO repair was not associated with earlier enteral feeding, improved postnatal growth, or reduced complications. These findings likely reflect the absence of an optimised nutritional strategy rather than an intrinsic limitation of TAT. A formal alongside nutritional protocol has been developed and is currently undergoing institutional approval prior to prospective evaluation at our centre.
Clinical outcome and long-term quality of life in patients treated for gastroschisis - Results from a national study.
Røkkum H, Sæter T, Treider MA … +2 more , Ertresvåg K, Bjørnland K
J Pediatr Surg · 2026 Jun · PMID 42250628 · Publisher ↗
BACKGROUND: Gastroschisis is the most common type of congenital abdominal wall defects. The survival rate is close to 100% in high-income countries, but knowledge on long-term outcomes is limited. Particularly, long-term...
BACKGROUND: Gastroschisis is the most common type of congenital abdominal wall defects. The survival rate is close to 100% in high-income countries, but knowledge on long-term outcomes is limited. Particularly, long-term quality of life (QoL) is scarcely reported. The aim of this study was to explore long-term clinical outcomes and to investigate patient-reported gastrointestinal and generic QoL in gastroschisis patients. METHODS: This is a cross-sectional long-term follow-up study of patients treated for gastroschisis 2009-2022 at the two tertiary referral hospitals for pediatric surgery in Norway. Eligible patients were invited to participate in a questionnaire survey. Gastrointestinal and generic QoL were assessed with the PedsQL Gastrointestinal Symptoms Scales and the PedsQL Generic Core Scales. Results were compared to those from healthy controls. Perioperative data, medical history and complications were collected from a study-specific questionnaire and electronic patient records. Ethical approval was obtained. RESULTS: Of 172 eligible patients, 82 (48%) responded. Sixteen (20%) underwent at least one unplanned laparotomy, most commonly due to mechanical intestinal obstruction. At follow-up, nearly one third had significant symptoms of gas and bloating, diarrhea, and constipation. Impaired outcomes for school functioning were registered in 11 (13%) patients. Eleven (13%) respondents reported unsatisfactory follow-up, particularly regarding management of gastrointestinal complaints and inadequate information about gastroschisis. Furthermore, 14 (17%) wanted assessment regarding surgical correction of the scar. CONCLUSION: Significant long-term gastrointestinal complaints and impaired school functioning occur frequently in gastroschisis patients, and parents often experience insufficient qualified follow-up. This knowledge is important for parental information and when planning follow-up.
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