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Budd-Chiari syndrome (BCS) presents with various degrees of liver damage, and the choice of treatment depends on the type and extent of hepatic injury. Liver transplantation (LT) is considered as the final treatment option when other interventions are not feasible and when the liver injury is irreversible. We report a case of a pediatric patient with BCS who underwent liver transplantation from a living donor in the context of thrombophilic disorder., Case Presentation: A 14-month-old girl was admitted to the hospital with ascites. She was malnourished, and an abdominal CT scan confirmed significant ascites with no visualization of the hepatic veins and retrohepatic inferior vena cava (IVC). A liver biopsy revealed fibrosis, necrosis, and parenchymal hemorrhage. Patient's portal hypertension was managed with prophylactic beta-blocker Propranolol and endoscopic esophageal variceal ligation. However, she was hospitalized four times due to gastrointestinal bleeding from ruptured esophageal varices. Protein C deficiency was found as probable etiology of BCS. The patient underwent liver transplantation at 3 years and 8 months old with a liver from a parental living donor. The surgery and postoperative course were uneventful, and the patient was discharged 25 days after the transplant., Clinical Discussion: Hypercoagulability is often the underlying cause of BCS. Maintaining anticoagulation/thrombophilic balance postoperatively contributed to the successful liver transplantation in this pediatric patient., Conclusion: Liver transplantation is a safe and effective treatment for pediatric patients with BCS who meet the criteria for the procedure., (© 2025 Wiley Periodicals LLC.)

Background: The concept of failure to rescue (FTR) has been used to evaluate the quality of care in several surgical specialties but has not been well-studied after living donor liver transplantation (LDLT) in children., Methods: This study retrospectively reviewed 500 pediatric LDLT performed at a single center between 1993 and 2022. The recipient outcomes were assessed by means of patient and graft survival rates, retransplantation rates, and arterial/portal/biliary complication rates. Graft and patient losses secondary to these complications were calculated regarding FTR for patients (FTRp) and grafts (FTRg)., Results: Overall 1- and 5-year patient survival rates were 94.5% and 92.1%, respectively, the corresponding figures for graft survival being 92.7% and 89.8%. One-year hepatic artery complication rate was 3.6% (n = 18 cases), the respective rates for portal vein complications and biliary complications being 5.7% (n = 57) and 15.6% (n = 101). One-year FTRp rates for hepatic artery thrombosis, portal vein thrombosis, anastomotic biliary stricture, and intrahepatic biliary stricture were 28.6%, 9.4%, 3.6%, and 0%, respectively. The corresponding FTRg rates being 21.4%, 6.3%, 0%, and 36.4%., Conclusion: Such novel analytical method may offer valuable insights for optimizing quality of care in pediatric LDLT., (© 2024 Wiley Periodicals LLC.)

Background: ND2 in Ho Chi Minh City is currently the only public center that performs PLT in Southern Vietnam. In 2005, the first PLT was successfully performed, with support from Belgian experts. This study reviews the implementation of PLT at our center and evaluates the results and challenges., Methods: Implementation of PLT at ND2 required medico-surgical team building and extensive improvement of hospital facilities. Records of 13 transplant recipients from 2005 to 2020 were studied retrospectively. Short- and long-term complications, as well as the survival rates, were reported., Results: The mean follow-up time was 8.3 ± 5.7 years. Surgical complications included one case of hepatic artery thrombosis that was successfully repaired, one case of colon perforation resulting in death from sepsis, and two cases of bile leak that were drained surgically. PTLD was observed in five patients, of whom three died. There were no cases of retransplantation. The 1-year, 5-year, and 10-year patient survival rates were 84.6%, 69.2%, and 69.2%, respectively. There were no cases of complication or death among the donors., Conclusion: Living-donor PLT was developed at ND2 for providing a life-saving treatment to children with end-stage liver disease. Early surgical complication rate was low, and the patient survival rate was satisfactory at 1 year. Long-term survival decreased considerably due to PTLD. Future challenges include surgical autonomy and improvement of long-term medical follow-up with a particular emphasis on prevention and management of Epstein-Barr virus-related disease., (© 2022 Wiley Periodicals LLC.)

Objectives: Cirrhotic children wait-listed for liver transplant are prone to bleeding from gastrointestinal varices. Grade 2-3 esophageal varices, red signs, and gastric varices are well-known risk factors. However, the involvement of hemostatic factors remains controversial because of the rebalanced state of coagulation during cirrhosis., Methods: Children suffering from decompensated cirrhosis were prospectively included while being on waitlist. Portal hypertension was assessed by ultrasound and endoscopy. Coagulopathy was evaluated through conventional tests, thromboelastometry, and platelet function testing. The included children were followed up until liver transplantation, and all bleeding episodes were recorded. Children with or without bleeding were compared according to clinical, radiological, endoscopic, and biological parameters. In addition, validation of a predictive model for risk of variceal bleeding comprising of grade 2-3 esophageal varices, red spots, and fibrinogen level <150 mg/dL was applied on this cohort., Results: Of 20 enrolled children, 6 had upper gastrointestinal bleeding. Significant differences were observed in fibrinogen level, adenosine diphosphate, and thrombin-dependent platelet aggregation. The model used to compute the upper gastrointestinal bleeding risk had an estimated predictive performance of 81.0%. Platelet aggregation analysis addition improved the estimated predictive performance up to 89.0%., Conclusions: We demonstrated an association between hemostatic factors and the upper gastrointestinal bleeding risk. A low fibrinogen level and platelet aggregation dysfunction may predict the risk of bleeding in children with decompensated cirrhosis. A predictive model is available to assess the upper gastrointestinal bleeding risk but needs further investigations. Clinicaltrials.gov number: NCT03244332., (© 2019 Wiley Periodicals, Inc.)