BackgroundHigh mortality in children awaiting liver transplantation is a significant concern. At Cipto Mangunkusumo Hospital, the only active and established pediatric living-donor liver transplantation (LDLT) center in Indonesia, similar challenges persist. This study aims to determine the mortality rate, time to death or transplantation and causes of death in children listed for LDLT at our center.MethodsThis prospective single-center cohort study was conducted from January 2023 to December 2024. Consecutive sampling included all eligible children age 0–18 listed for LDLT. Participants were monitored from listing until pretransplant death, transplantation, loss to follow-up or study completion. The primary outcome was all-cause mortality on the LDLT waiting list.ResultsSixty-two children were enrolled; 64.51% (n=40) were female with biliary atresia accounting for 72.58% of diagnoses. Malnutrition was identified in 70.97% (n=44) and 35.48% (n=22) had a history of cytomegalovirus (CMV) infection. Median duration from listing to death was 87.5 days. Median time to transplantation was 263.5 days. During follow-up, 22 children died with sepsis as the leading cause, 22 underwent LDLT, 13 were lost to follow-up and 5 remained on the waiting list. Donor scarcity contributed substantially to prolonged listing and loss to follow-up.ConclusionsChildren listed for liver transplantation experience a high waiting list mortality at our center. This is primarily due to infection, malnutrition and socioeconomic factors. Sepsis persists as the principal cause of death. LDLT remains the only feasible option for children requiring liver transplantation in Indonesia.

This study aimed to evaluate the safety and efficacy of three-port thoracoscopic surgery for patent ductus arteriosus (PDA) ligation in children. A retrospective analysis was conducted on 50 pediatric patients with PDA (median age: 30 months; median weight: 12.00 kg; median ductal diameter: 2.50 mm) treated with three-port thoracoscopic surgery in the Department of Cardiothoracic Surgery at Kunming Children's Hospital from January 2021 to June 2024. All 50 patients successfully underwent surgery without conversion to open thoracotomy; there was no mortality. The median hospitalization cost was 25 211.00 CNY. Postoperatively, three (6.00%) patients developed chylothorax and three (6.00%) patients had residual shunts. During the 6-36 month follow-up period, no instances of ductal recanalization or other complications were reported. In conclusion, three-port thoracoscopic PDA ligation is a safe and effective approach, offering cosmetic incisions, definitive efficacy, and low complication rates. However, meticulous handling during ductus arteriosus ligation is essential to minimize complications and residual shunts.

Biliary atresia (BA) is a progressive cholangiopathy of infancy and the leading cause of pediatric liver transplantation. Despite surgical intervention with the Kasai portoenterostomy, long-term outcomes remain poor, with many patients progressing to cirrhosis. Emerging evidence implicates the gut microbiota—a dynamic ecosystem crucial to immune development and liver homeostasis—in BA pathogenesis and clinical progression. This review synthesizes current literature on gut microbiota composition in BA before and after the Kasai procedure, highlighting consistent patterns of dysbiosis, including pathobiont expansion and depletion of beneficial microbes such as Bifidobacterium. The review explores associations between microbial profiles and clinical outcomes—highlighting potential mechanisms involving bile acid metabolism, microbial translocation, and immune modulation. Further understanding of gut–liver–microbiota interactions in BA may inform microbiome-targeted therapies to improve native liver survival.

This study aimed to evaluate the feasibility and efficacy of laparoscopic management in neonates diagnosed with congenital gastric muscular layer defects and gastric perforation. A retrospective analysis was conducted on four male neonates (median gestational age: 36.79 weeks; median birth weight: 2460 g) who underwent laparoscopic repair at Shanxi Provincial Children’s Hospital between January 2022 and October 2024. All neonates presented with symptoms of vomiting, feeding intolerance, and abdominal distension, with preoperative imaging confirming pneumoperitoneum. Laparoscopic surgery was successfully completed in all cases without conversion to open surgery. The median operative time was 102 min, and no surgery-related complications such as anastomotic leakage or hemorrhage were observed. One neonate was transferred to the department of internal medicine due to postoperative feeding difficulties, failure, and persistent oxygen dependence. The median hospital stay was 27.5 days. At the 6-month postoperative follow-up, three neonates exhibited satisfactory growth and development, while one exhibited mild growth delay. Our findings indicate that laparoscopic repair offers significant advantages, including mitigated surgical trauma, quicker recovery, and lower incidence of intestinal adhesion. Provided that surgical indications are strictly assessed and the surgeon possesses proficient laparoscopic skills, it is feasible for specialized surgeons to manage this disease using laparoscopy.

10.1136/wjps-2025-001032.video016376273975112BMJ Journals Video Playerwjps2025001032media1.

BackgroundHirschsprung’s disease (HSCR) is a functional obstruction of the gastrointestinal tract characterized by abdominal distension, constipation, and vomiting. The protein zonulin serves as a biomarker for intestinal permeability. We sought to explore the changes in plasma zonulin levels in patients with HSCR and to assess its predictive role in the development of postoperative Hirschsprung-associated enterocolitis (HAEC).MethodsThere are 60 patients with HSCR were recruited for this study, categorized into short-segment disease (S-HSCR) (n=33), long-segment disease (L-HSCR) (n=15), and total colonic aganglionosis (TCA) (n=12). Venous blood samples were taken from all participants before and after pull-through surgery. Plasma concentrations of zonulin were determined using an ELISA. Immunohistochemistry (IHC) analysis was conducted to evaluate the expression of zonula occludens-1 (ZO-1) and occludin in colonic tissues. Hematoxylin and Eosin stained (H&E-stained) sections were used to evaluate the degree of inflammation in the dilated (ganglionic) segment. Postoperative outcomes were assessed through a combination of online questionnaires and telephone interviews. Diagnostic threshold for HAEC was based on clinical symptom sets and the HAEC scoring system developed previously.ResultsPreoperative zonulin levels in patients with TCA were statistically lower than those in patients with S-HSCR (p=0.008) and L-HSCR (p=0.028). The incidence of postoperative HAEC was 16.7%, 57.1%, and 14.3% in TCA, L-HSCR, and S-HSCR groups, respectively. Patients who experienced an increase in plasma zonulin levels of more than 1.5 times those on the first day after surgery had a higher risk of developing HAEC (p=0.005). IHC staining further confirmed decreased expression of ZO-1 and occludin in colonic tissues of patients with HSCR who experienced postoperative HAEC.ConclusionPreoperative zonulin levels were lowest in the TCA group. The change in zonulin levels on the first day after surgery can serve as a useful indicator for predicting the risk of postoperative HAEC occurrence.

Organoids are three-dimensional and self-organizing cell cultures of various lineages that resemble structures and functions of an organ in many ways, and they are versatile tools in disease modeling and patho-mechanistic study of human diseases affecting their tissues of origin. Biliary atresia (BA), a cholangiopathy affecting the bile ducts of the liver, is a heterogeneous and multifaceted liver disease of complex pathogenesis. Cholangiopathies refer to a category of liver diseases that affect the cholangiocytes, the epithelial cells lining the lumen of the biliary trees. Biliary organoids consist of cholangiocytes in a spherical monolayer epithelium, which favorably resembles the structures and functional properties of the bile duct cholangiocytes. Biliary tissue-derived cells, pluripotent stem cells or embryonic stem cells, and hepatic progenitor cells are capable of generating biliary organoids. In the last decade, a considerable advancement has been made in the generation of biliary organoids for modeling liver physiology and pathophysiology. Using biliary organoids, scientists have advanced our knowledge underlying the pathogenic roles of genetic susceptibility, dysregulated hepatobiliary development/structure, environmental factors, and dysregulated immune-inflammatory responses to an injury in BA. This review will summarize and discuss the derivation and the use of biliary organoids in the disease modeling and patho-mechanistic study of BA.

Multisystem inflammatory syndrome in children (MIS-C) is a disease that emerged during the COVID-19 pandemic. Patients exhibit symptoms mimicking the clinical presentation of an acute abdomen, representing a novel differential diagnosis, particularly in the young generation. This study aims to investigate the current level of awareness of MIS-C among surgeons and medical students. We conducted an anonymous online questionnaire among members of the Swiss Surgical Society and Swiss medical students. The questionnaires collected participants' baseline demographics and their awareness regarding MIS-C. Both students and surgeons obtained very low scores in the self-assessment and had a low score in the section with awareness questions (true score). In medical students, we observed a positive correlation between self-assessment and true scores (ρ=0.422, p=0.001), while surgeons had a negative correlation (ρ=-0.243, p<0.001). Furthermore, there was a positive correlation between gender and self-assessment (ρ=0.245, p<0.001), depicting a higher self-assessed score in female surgeons (median female self-assesment=5, IQR: 2-7). Likewise, board-certified pediatric surgeons and surgeons treating both children and adults had a higher self-assessment compared with non-pediatric surgeons. In both populations (surgeons and students), the true scores were similar in all the variables analyzed. Two-thirds of surgeons stated that MIS-C should be considered a differential diagnosis, and about half of surgeons indicated MIS-C being part of their differential diagnosis already. The results show an insufficient level of awareness concerning MIS-C among students and surgeons, warranting implementation in students' curriculum and surgeons' continuous training.

Focal nodular hyperplasia (FNH)-like lesions of the liver are rare in the pediatric population and are seldom reported as congenital lesions. The differential diagnosis between these lesions and pure-fetal hepatoblastoma (HBL) is challenging. We present a case of a congenital FNH-like hepatic lesion, managed with a right hepatectomy due to suspected fetal HBL. Additionally, a review of all published cases of congenital FNH-like lesions of the liver was carried out.MRI