Background: Budd-Chiari syndrome (BCS) is a rare disorder characterized by the obstruction of hepatic outflow veins. This results in backup of blood in the liver, causing abdominal pain, hepatomegaly and ascites. The choice of treatment offered to the patients with BCS depends on the type and extent of hepatic injury. Liver transplantation (LT) is considered as the definitive treatment option when the liver injury is extensive and irreversible, or when other treatment options are not considered effective. This study evaluates the outcomes of living donor liver transplantation (LDLT) in pediatric patients with decompensated liver disease secondary to BCS.
Methods: This case-series study was conducted on four male pediatric patients (mean age: 9.75 years, range: 6–15) who underwent LDLT at Pakistan Kidney and Liver Institute and Research center (PKLI & RC) Lahore, Pakstan. Clinical characteristics, surgical parameters, and post-transplant outcomes were assessed.
Results: Out of 103 pediatric liver transplants performed between July 2021 and February 2025, four patients (3.8%) underwent LDLT for Budd-Chiari syndrome. All patients were male, with a mean age of 9.75 years (range: 6–15 years). Protein C deficiency was found the most common cause (100%) cases. The postoperative survival rate was 75%, with one mortality (25%) occurring at 9 months due to sepsis and liver failure. The mean hospital stay was 27 days (range: 16–57). Portal vein thrombus was found in one patient (25%) at the time of transplantation. All patients had retro hepatic IVC involvement, with one case of extensive fibrosis requiring IVC replacement with cadaveric common iliac vein. All the patients had hepatomegaly and ascites as the most common presentation, with Diuretic resistant ascites documented in 2 patients (50%) cases. Preoperative complications included hematemesis (50%), melena (50%), ascites (100%), and jaundice (50%). Three patients (75%) received left lobe grafts, while one (25%) received right lobe graft. Cold and warm ischemia times averaged 28.5 and 24.5 minutes, respectively. Post-operative Portal vein thrombosis occurred in one patient (25%) requiring exploration, and liver rejection was documented in another (25%), which was managed with adequate immunosuppression. Biliary anastomotic strictures was reported in one patient (25%), requiring ERCP.


Conclusion: LDLT remains a viable option for pediatric patients with decompensated liver disease due to BCS, offering favorable survival outcomes. However, post-transplant complications, including vascular, biliary issues and liver rejection necessitate careful monitoring and long-term management. Further studies with larger cohorts are needed to optimize perioperative care and improve long-term survival in this patient population.