BACKGROUND: Congenital lung malformation (CLMs) is a series of developmental lung abnormalities that may persist into adulthood. Some centres would advocate an observation approach rather than opting for surgical excisio...BACKGROUND: Congenital lung malformation (CLMs) is a series of developmental lung abnormalities that may persist into adulthood. Some centres would advocate an observation approach rather than opting for surgical excision. However, the potential impact of environmental factors, particularly cigarette smoke exposure, on CLM-affected lung tissue remains poorly understood. AIM: This study aimed to evaluate the effects of cigarette smoke extract (CSE) on paired normal and diseased airway organoids (AOs) derived from pediatric lung tissue diagnosed as intralobar sequestration, using N-[N-(3,5-Difluorophenacetyl)-L-alanyl]-S-phenylglycine t-butyl ester (DAPT), a γ-secretase inhibitor, to modulate airway epithelial differentiation. METHOD: AOs were established from paired normal and diseased regions of pediatric lung tissue. Organoids were cultured under two conditions: without DAPT or with DAPT pretreatment, followed by 4 % CSE exposure. Mucus viscosity, MUC5AC protein expression, PAS staining, and RT-qPCR analysis of airway epithelial cell markers, tight junction proteins (E-cadherin, ZO-1), and proinflammatory cytokines were measured. RESULTS: Disease-derived AOs exhibited higher mucus secretion compared to normal tissue-derived AOs. CSE exposure enhanced mucus viscosity and MUC5AC expression in both groups, with more pronounced effects in diseased organoids. CSE-induced upregulation of mucins and pro-inflammatory cytokines was particularly evident in disease-derived organoids without DAPT treatment. DAPT pretreatment attenuated these CSE-induced changes, while tight junction protein expression remained stable across conditions. CONCLUSION: Our findings demonstrate that diseased airway organoids show enhanced susceptibility to cigarette-smoke -induced mucus hypersecretion and inflammatory responses, suggesting that patients with congenital airway malformations may be particularly vulnerable to the effects of cigarette smoke exposure.
BACKGROUND: Esophageal button battery impactions are pediatric emergencies with clear guidelines for pre-extraction management, but post-extraction care remains highly variable. Common practices include ICU admission, pr...BACKGROUND: Esophageal button battery impactions are pediatric emergencies with clear guidelines for pre-extraction management, but post-extraction care remains highly variable. Common practices include ICU admission, prolonged NPO, antibiotics, and extensive imaging, though the supporting evidence is unclear. METHODS: We performed a scoping review, searching PubMed, Embase, and Scopus for pediatric esophageal button battery injuries reporting post-extraction management. Variables included imaging strategy and impact, ICU admission, feeding practices, and complications. Case reports, case series, and retrospective cohorts were included. Variables included imaging strategy and impact, ICU admission, feeding practices, and complications. Case reports, case series, and retrospective cohorts were included. RESULTS: Of 37 studies with 713 patients, complications included 47 esophageal perforations, 37 tracheoesophageal fistulas, 28 esophageal strictures, 26 mediastinitis, 23 esophageal stenoses, 11 vocal cord injuries, 4 aortoesophageal fistulas, and 3 deaths. Post-extraction imaging was reported in 83.8 % of studies with 610 patients; esophagram was most common. In studies that reported imaging details, imaging influenced postoperative management in 12/23 studies using esophagram, 5/12 studies using CXR, 4/6 studies using CT, and 2/3 studies using MRI/MRA. (These were study-level proportions as patient-level counts could not be consistently extracted and modalities overlapped).Time to feeding ranged from 1 h to 233 days via multiple routes. Among 24 studies with known timing, 12/18 resuming <7 days and 2/6 resuming ≥7 days reported morbidity or mortality. Antibiotics were explicitly given in 24 studies. CONCLUSION: Routine ICU admission, prolonged NPO, universal antibiotics, and multi-modality imaging are not standard after esophageal button battery removal. We propose a risk-stratified framework for imaging, ICU use, and feeding that reserves advanced imaging, ICU level care, and delayed feedings for patients at highest risk for complications based on their presentation and injury severity.
BACKGROUND: Recurrent tracheoesophageal fistula (rTEF) is a rare but serious complication after esophageal atresia (EA) repair. Although various surgical and endoscopic techniques are available, the optimal management re...BACKGROUND: Recurrent tracheoesophageal fistula (rTEF) is a rare but serious complication after esophageal atresia (EA) repair. Although various surgical and endoscopic techniques are available, the optimal management remains controversial due to limited data and heterogeneous outcomes. PURPOSE: To systematically review and compare the outcomes of surgical and endoscopic interventions for rTEF in pediatric patients. METHODS: A systematic literature search was conducted in PubMed, MEDLINE, and EMBASE for studies published between January 2015 and January 2025. Twenty-seven eligible studies involving 789 children with rTEF were analyzed. Treatment modalities were classified as open surgical management (OSM), thoracoscopic surgical management (TSM), or endoscopic management (EM). Data on demographics, treatment type, and outcomes were descriptively summarized. RESULTS: The overall incidence of rTEF was 7.17 %. The success rates were 92.9 % for OSM, 86.4 % for TSM, and 67.2 % for EM. Major risk factors were esophageal stricture and anastomotic leakage. Re-recurrence rates ranged from 8.9 % to 21.7 % in surgically treated patients. EM frequently required repeated sessions, and a substantial proportion of patients ultimately underwent surgical repair due to persistent fistula or recurrent symptoms. CONCLUSIONS: Open and thoracoscopic repair remain the most effective treatments for rTEF, while endoscopic methods serve as minimally invasive adjuncts. Standardized definitions and multicenter prospective studies are required to establish an evidence-based treatment algorithm and to improve either short- or long-term outcomes in children with rTEF.
BACKGROUND: Indocyanine green (ICG) fluorescence imaging is increasingly used intraoperatively to assess tissue perfusion and guide surgical decision-making. While its qualitative use has been associated with improved ou...BACKGROUND: Indocyanine green (ICG) fluorescence imaging is increasingly used intraoperatively to assess tissue perfusion and guide surgical decision-making. While its qualitative use has been associated with improved outcomes, including reduced anastomotic leak rates, interpretation remains subjective and lacks standardization. Quantitative fluorescence analysis may improve reproducibility, precision, and broader clinical applicability. METHODS: Handheld intraoperative ICG fluorescence video from a representative bowel perfusion assessment was analyzed using three quantitative approaches: (1) a static region-of-interest (ROI) "steady lasso," (2) a manually adjusted "moving lasso," and (3) a semi-automated computational method. ROIs were applied to well- and poorly-perfused bowel segments, and mean pixel intensity over time was measured. RESULTS: The steady lasso method demonstrated substantial signal misalignment due to tissue and camera motion, limiting reliability. The moving lasso improved spatial accuracy and demonstrated earlier signal rise and higher peak intensity in well-perfused bowel compared with poorly perfused tissue. The semi-automated method enabled high-throughput analysis and detailed characterization of fluorescence signal dynamics. Large ROIs demonstrated lower signal variability compared with small ROIs, particularly in poorly perfused tissue. Across methods, visually well-perfused tissue demonstrated faster time-to-peak and higher peak fluorescence. DISCUSSION: Quantitative analysis of ICG fluorescence in handheld surgical video is feasible and reveals measurable differences in fluorescence signal behavior. While manual techniques improve accuracy over static approaches, semi-automated methods offer a higher degree of scalability. These findings highlight the need for automated ROI tracking and standardized acquisition protocols to support reproducible, real-time quantitative perfusion assessment in surgery.
OBJECTIVES: Barrett's esophagus (BE) is a late sequela after esophageal atresia (EA) repair. In order to assess the need for an endoscopic BE surveillance program, we studied evolution of BE in 71 adults with C-type EA i...OBJECTIVES: Barrett's esophagus (BE) is a late sequela after esophageal atresia (EA) repair. In order to assess the need for an endoscopic BE surveillance program, we studied evolution of BE in 71 adults with C-type EA in two successive endoscopies. METHODS: Endoscopic Prague Classification of BE was used. Endoscopic BE was graded as True BE (with goblet cell metaplasia) or BE without goblet cell metaplasia. RESULTS: The median patient age was 36 (IQR 28-43) years at E1 (index endoscopy) and 50 (41-58) years at E2 (follow-up endoscopy) with median interval of 16 (12-17) years. Prevalence of endoscopic BE increased from 15 % to 42 % (p = 0.002) and of true BE from 1.4 % to 15 %, (p = 0.04). Dysplasia and cancer were not observed. De novo true BE (n = 10) developed from previous BE without goblet cell metaplasia (n = 4) or from normal epithelium (n = 6). In four (5.6 %) patients true BE extended ≥ 3 cm. True BE was predicted by previous endoscopic BE (RR = 9.2; 95%CI 2.0-4-1, p = 0.004) and esophagitis (RR = 5.8; 95%CI 1.4-38, p = 0.02). CONCLUSIONS: The prevalence of endoscopic and true BE increased by 3- and 10-fold, respectively, between median ages of 36 and 50 years. High-risk BE was rare and no dysplasia or cancer was found. Esophagitis and endoscopic BE predisposed to true BE. Endoscopic surveillance of patients with EA before the age 50 years seems unnecessary.
BACKGROUND: Pediatric intestinal pseudo-obstruction (PIPO) is a severe gastrointestinal motility disorder with considerable genetic heterogeneity. Nevertheless, genetic etiology in a substantial proportion of the patient...BACKGROUND: Pediatric intestinal pseudo-obstruction (PIPO) is a severe gastrointestinal motility disorder with considerable genetic heterogeneity. Nevertheless, genetic etiology in a substantial proportion of the patients remains unknown. This study aimed to identify the underlying genetic cause in a child with unexplained PIPO and to establish a novel disease gene associated with this condition. METHODS: Whole-exome sequencing was performed in a PIPO pedigree negative for mutations in known causative genes. The effects of the variant identified on protein expression were evaluated by Western blotting. A knock-in mouse model bearing the patient-specific variant was generated using CRISPR/Cas9. Intestinal motility was evaluated by carmine red transit assay. The contractile function of primary mouse intestinal smooth muscle cells (MISMCs) was assessed by collagen gel contraction assays. RESULTS: We identified an extremely rare, evolutionarily conserved de novo missense variant (c.20A > G, p.N7S) in the patient's CNN2 gene. This gene encodes a protein belonging to the calponin (CaP) family, which plays a critical regulatory role in smooth muscle contraction. Cnn2 mice exhibited significantly prolonged gastrointestinal transit time, recapitulating the intestinal dysmotility phenotype. Functional analyses revealed that the variant significantly impaired MISMC contractile function in a gene dosage-dependent manner. CONCLUSION: Our study revealed that a variant in CNN2 causes intestinal dysmotility in a PIPO patient and mouse model, establishing CNN2 as a novel disease gene. These findings provide a molecular diagnosis for the affected patient, expands the genetic spectrum of PIPO, and reveals an essential role of calponin 2 in the regulation of intestinal motility.
BACKGROUND: Laparoscopic-assisted Anorectoplasty (LAARP) for male recto-bulbar fistula (RBF) remains debated, with early reports describing approach-specific complications. Prior meta-analyses pooled heterogeneous Anorec...BACKGROUND: Laparoscopic-assisted Anorectoplasty (LAARP) for male recto-bulbar fistula (RBF) remains debated, with early reports describing approach-specific complications. Prior meta-analyses pooled heterogeneous Anorectal malformation (ARM) subtypes, limiting conclusions for RBF. We conducted an RBF-specific systematic review and meta-analysis comparing LAARP with posterior sagittal anorectoplasty (PSARP). METHODS: PubMed/MEDLINE, Embase, Scopus, and Google Scholar were searched (2000-November 2025) for comparative studies of LAARP versus PSARP in male children with RBF. Risk of bias was assessed using the ROBINS-I tool. Random-effects meta-analyses in Jeffreys's Amazing Statistics Program (JASP) used log effect sizes for dichotomous outcomes and mean differences for operative time. Sensitivity analyses excluded studies with zero events in both arms. RESULTS: Six observational cohort studies (278 patients; LAARP 154, PSARP 124) were included. No intraoperative complications or conversions to open surgery were reported. Postoperative urethral diverticulum (UD) was not reported in five studies; one study reported 4 cases (15.38 %) after LAARP and none after PSARP, precluding meta-analysis. Primary analyses showed no statistically significant differences between LAARP and PSARP for residual fistula (RSF) (pooled log odds ratio (OR) 0.68, 95 % confidence interval (CI) -0.86 to 2.22), anal stenosis (log OR -0.36, 95 % CI -1.35 to 0.63), or rectal prolapse (log OR 1.03, 95 % CI -0.03 to 2.09). Reoperation was infrequent but higher after LAARP in the pooled analysis (log OR 1.23, 95 % CI 0.02 to 2.44); this persisted in event-positive studies (log OR 1.45, 95 % CI 0.18 to 2.72). Operative time was longer with LAARP (mean difference (MD) 66.74 min, 95 % CI 27.48 to 106.00), with substantial heterogeneity (I 97.57 %). Wound infection was uncommon and inconsistently reported; quantitative pooling was not undertaken. CONCLUSIONS: LAARP is a feasible and safe option for male RBF, with outcomes comparable to those of PSARP for major postoperative complications; however, the certainty of the evidence remains limited, underscoring the need for high-quality prospective studies.
BACKGROUND: The decision to operate on asymptomatic children who have ingested multiple magnetic objects is based on a subjective assessment of magnet progression via serial abdominal radiographs at non-standardised time...BACKGROUND: The decision to operate on asymptomatic children who have ingested multiple magnetic objects is based on a subjective assessment of magnet progression via serial abdominal radiographs at non-standardised time-points. We aimed to develop an objective tool to identify non-progression on serial abdominal radiographs. METHOD: Children (aged ≤16 years) who ingested two or more magnetic objects in one episode were identified from four UK paediatric surgery centres. Asymptomatic children who had undergone two or more abdominal radiographs, without radiological evidence of obstruction or pneumoperitoneum, were included. Each abdominal radiograph was divided into a 3x3 grid and progression was defined as movement of magnets from one grid region to another between two serial radiographs. Magnet-related injuries were recorded. RESULTS: Thirty-one children, ingesting a median of 3 magnets (range: 2-20), were included. Of these 10/31 (32 %) underwent a surgical intervention with 5/31 (16 %) confirmed to have a magnet-related injury. Timing of radiographs varied with 21/31 (68 %) undergoing at least one follow-up radiograph within 24 h and 29/31 (94 %) in the first 48 h. Children with a magnet-related injury had non-progression identified earlier. No child with two consecutive episodes of progression developed a magnet-related injury, whereas two consecutive episodes of non-progression were strongly associated with magnet-related injury. CONCLUSION: A 3x3 grid system overlaying the abdominal radiograph provides an objective tool to evaluate magnet progression. We suggest an imaging protocol consisting of at least three abdominal radiographs within the first 36-48 h, with risk-based adjustment of imaging frequency thereafter. Combined with standardisation of radiograph timing, the grid system may facilitate earlier intervention in those with non-progression, safe early discharge in those without injury, and overall reduced radiation exposure.
BACKGROUND: Pre-circumcision genital screening enables early detection of anomalies that contraindicate the procedure and require specialist management. In Pakistan, where most circumcisions are performed by untrained pr...BACKGROUND: Pre-circumcision genital screening enables early detection of anomalies that contraindicate the procedure and require specialist management. In Pakistan, where most circumcisions are performed by untrained providers, such screening is rarely practiced. This study evaluated the effectiveness of training health workers (HWs) to identify genital anomalies through a structured Safe Circumcision Program (SCP). METHODS: A retrospective data review was conducted of all male infants presenting to the safe circumcision clinic at the Indus Hospital & Health Network (IHHN) Karachi between June 2016 to June 2022. Trained HWs screened infants prior to circumcision, referring those with suspected anomalies to pediatric surgeons. Concordance between HW and specialist diagnoses was analyzed using Cohen's Kappa. RESULTS: Of 9,898 infants screened, 382 (3.9 %) had suspected genital anomalies. Among 310 infants reviewed by specialists, 353 anomalies were confirmed. The most common anomalies were webbed penis (n = 98, 27.8 %), hydrocele (n = 74, 21.0 %), hypospadias (n = 41, 11.6 %), undescended testes (n = 35, 9.9 %), and chordee (n = 34, 9.6 %). Overall concordance between HWs and specialists was 73.1 %. Discordance was mainly due to false positives, particularly in cases of webbing and hypospadias. CONCLUSION: With structured training HWs were able to competently identify genital anomalies prior to circumcision, demonstrating feasibility and safety of task-shifting in low-resource settings. Pre-circumcision screening by trained HWs can prevent unsafe procedures and facilitate early referral for specialist care in countries lacking routine newborn screening programs.
BACKGROUND: Liver trauma is a leading cause of pediatric mortality, yet consensus on optimal intervention in children remains unclear. Angioembolization (AE), widely adopted in adults, remains underutilized in children....BACKGROUND: Liver trauma is a leading cause of pediatric mortality, yet consensus on optimal intervention in children remains unclear. Angioembolization (AE), widely adopted in adults, remains underutilized in children. We hypothesized that AE would be associated with lower in-hospital mortality than surgical repair (SR). METHODS: We conducted a retrospective cohort study using the American College of Surgeons Trauma Quality Improvement Program database (2018-2021) to identify pediatric patients (<18 years) with liver trauma undergoing AE or SR (N = 817). The primary outcome was in-hospital mortality; secondary outcomes included discharge disposition, ICU/hospital length of stay, early blood product use, and complications. One-to-one propensity score matching and multivariable logistic regression adjusted for confounding. RESULTS: In the matched cohort, AE (N = 152) was associated with lower mortality (7.2 % vs 19.1 %; p = 0.002) than SR (N = 152). AE patients were more often discharged home (68.4 % vs 40.1 %; p < 0.001) and required fewer transfusions within 4 h: packed red blood cells (56.5 % vs 77.3 %; p < 0.001), plasma (39.5 % vs 67.8 %; p < 0.001), and platelets (19.7 % vs 50.7 %; p < 0.001). In multivariate analysis, AE remained independently associated with reduced mortality (OR = 0.34, 95 % CI 0.15-0.73; p = 0.01), with Injury Severity Score (ISS) predicting mortality (OR = 1.06, 95 % CI 1.03-1.10; p < 0.01). Age, sex, and penetrating injury were not significant predictors (AUC = 0.791). CONCLUSION: AE was associated with lower in-hospital mortality and reduced blood product requirements compared to SR in select pediatric liver trauma patients, suggesting that AE may be an effective adjunct to nonoperative management in hemodynamically stable patients.
PURPOSE: The present nationwide survey combined with a scoping review aimed to provide an overview of current paediatric robotic-assisted surgery (RAS) training practices in France, focusing on simulation tools and evalu...PURPOSE: The present nationwide survey combined with a scoping review aimed to provide an overview of current paediatric robotic-assisted surgery (RAS) training practices in France, focusing on simulation tools and evaluation methods. METHODS: A cross-sectional national survey was conducted among all French paediatric surgery centres with existing or upcoming RAS programmes. Answers were collected using Likert scales. A scoping review was conducted following PRISMA-ScR guidelines, screening publications from 2000 to 2025 on paediatric robotic surgery and simulation. RESULTS: Eighteen surgeons from 13 centres responded. Two centres had exclusive access to robotic equipment, whereas others shared platforms with adult teams. Centres reported a mean 4.2 training days per month and 37.5 RAS procedures per year. Twelve respondents reported that a dedicated paediatric RAS curriculum would be more appropriate. Fifteen (82 %) engaged in additional simulation outside their initial training. Three independent paediatric RAS simulation programmes were identified, differing in format and target audience. Only three participants used objective performance-assessment tools. The scoping review included 8 articles from 361 records. All focused on procedural robotic simulation in paediatric settings; only one addressed non-procedural interprofessional simulation. The included studies demonstrated benefits of simulation in accelerating the learning curve, improving trocar placement, and strengthening team performance. CONCLUSIONS: These findings support the need for structured, paediatric-specific RAS simulation programmes integrated into training pathways, supported by structured evaluation frameworks and institutional investment.
INTRODUCTION: Optimal age for definitive pull-through in Hirschsprung disease (HD) is debated. Both early and delayed interventions may influence short- and long-term outcomes, but current guidelines do not provide a cle...INTRODUCTION: Optimal age for definitive pull-through in Hirschsprung disease (HD) is debated. Both early and delayed interventions may influence short- and long-term outcomes, but current guidelines do not provide a clear recommendation. METHODS: A systematic review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Nineteen studies published between 1998 and 2025, including 3980 pediatric patients with HD undergoing definitive pull-through surgery, were included. Age at surgery was stratified using predefined thresholds (neonatal, <3 months, <6 months, <12 months, <4 years, and above). Short- and long-term outcomes were extracted and synthesized. Risk of bias was assessed using the Newcastle-Ottawa Scale. RESULTS: Short-term outcomes were reported in 15 studies. Hirschsprung-associated enterocolitis (HAEC) was the most frequently reported complication (0-69.1 %), occurring more often in neonates than in non-neonates (22.4 %, 78/348 vs. 15.5 %, 125/808) and in patients operated before 3 months of age compared with those operated later (20.7 %, 68/328 vs. 13.6 %, 60/442). Neonatal surgery was also associated with higher rates of anastomotic leakage (7.1 %, 10/140 vs. 1.5 %, 6/412). Long-term outcomes were assessed in 14 studies. Patients operated during the neonatal period had higher rates of fecal incontinence (30.0 %, 55/183 vs. 17.8 %, 56/314) and constipation (25.2 %, 39/155 vs. 12.8 %, 43/336). Similarly, surgery performed before 3 months of age was associated with increased rates of fecal incontinence (18.0 %, 36/200 vs. 8.6 %, 20/232) and constipation (23.5 %, 47/200 vs. 13.8 %, 32/232) at long-term follow-up. CONCLUSIONS: Timing of definitive surgery in Hirschsprung disease is associated with differences in postoperative outcomes. Neonatal surgery and surgery performed during early infancy (<3 months) were more often associated with higher complication rates, whereas procedures performed during infancy showed more balanced results.
BACKGROUND: Post-hepatectomy liver failure (PHLF) is a major concern after extensive liver resection. Adult volumetric rules (20/30/40 rules) and International Study Group of Liver Surgery (ISGLS) definitions are often a...BACKGROUND: Post-hepatectomy liver failure (PHLF) is a major concern after extensive liver resection. Adult volumetric rules (20/30/40 rules) and International Study Group of Liver Surgery (ISGLS) definitions are often applied to children, but their relevance is unclear. We evaluated PHLF incidence and course in a pediatric surgical series, with attention to salvage liver transplantation (SLTx). METHODS: Retrospective review of 57 consecutive pediatric patients (<18) undergoing initial curative hepatectomy for hepatoblastoma (HB) at our institution (2003-2025). PHLF was assessed using adult criteria (ISGLS, 50:50, and bilirubin >7 mg/dL). Clinical outcomes, volumetric parameters, and chemotherapy exposure were analyzed. RESULTS: Twenty-eight patients (49 %) underwent liver resection, leaving <3 Couinaud segments. All patients had FLR/TLV ≥30 %. Six patients (10.5 %) met adult PHLF criteria, but none required SLTx during the early postoperative period. One patient developed late-onset hepatic failure several months after liver resection, leaving <3 Couinaud segments despite adequate FLR/TLV and required SLTx. Histology revealed fibrosis progression likely attributable to cumulative chemotherapy, radiotherapy, and perioperative complications. Overall, liver resection leaving <3 Couinaud segments was associated with more frequent postoperative complications (P < 0.05), but not with higher early PHLF incidence. CONCLUSION: Adult-based PHLF criteria may capture transient dysfunction in pediatric HB patients but fail to identify those at risk for late, clinically significant hepatic failure requiring SLTx. Beyond volumetry, cumulative therapy-related hepatic injury, perioperative complications, and baseline fragility appear central to PHLF pathogenesis in children. Careful preoperative assessment and centralized surgical management with access to transplantation backup are essential.