Background: Crigler-Najjar Syndrome (CNS) is a rare genetic disorder causing severe unconjugated hyperbilirubinemia due to UDP-glucuronosyltransferase deficiency. Auxiliary Partial Orthotopic Liver Transplantation (APOLT) presents a promising alternative to whole liver transplantation in pediatric CNS patients, allowing native liver preservation. This study evaluates the safety, efficacy, and early outcomes of APOLT in pediatric CNS patients at a single center.
Methods: A retrospective analysis was conducted using a prospectively maintained database of seven pediatric patients who underwent APOLT at Pakistan Kidney and Liver Institute Lahore, from February 2022 to March 2025. Left lateral sector grafts from living donors were used in all cases. Preoperative and postoperative bilirubin levels, perioperative complications and clinical outcomes were analyzed. Statistical analysis was performed using paired t-tests or Wilcoxon signed-rank tests for comparing pre- and post-transplant bilirubin levels. Categorical variables were analyzed using Fisher’s exact test or chi-square test where applicable. Descriptive statistics, including mean ± standard deviation (SD), were used for continuous variables.
Results: All seven recipients (mean age 5.4 ± 3.2 years, 71.4% male) survived with no graft loss. Preoperatively, patients exhibited severe hyperbilirubinemia (24.6 ± 7.9 mg/dL) and Child-Pugh B cirrhosis (MELD-Na 18.0 ± 2.0). Rapid metabolic correction was achieved, with bilirubin levels declining to 1.0 ± 0.5 mg/dL by day 10 and 0.7 ± 0.5 mg/dL by day 30. No major complications (hepatic artery thrombosis, biliary leaks, or rejection) occurred. One patient (14.3%) developed tacrolimus-related neurotoxicity, resolved with dose adjustment. Two patients (28.6%) needed transfusions (mean blood loss 214.3 ± 111.8 mL).  To optimize graft perfusion and minimize portal venous steal, portal flow modulation was performed in all cases thereby improving blood flow to the graft. While hepaticojejunostomy is the commonly used biliary reconstruction technique in pediatric liver transplantation, duct-to-duct anastomosis was successfully performed in 3 out of 7 patients. The remaining 4 patients underwent hepaticojejunostomy.  Immunosuppression was well-tolerated, with no episodes of acute rejection. Donors (71.4% female, mean age 32.9 ± 8.4 years) demonstrated excellent safety profiles, with low blood loss (235.7 ± 57.7 mL). Graft parameters were favorable (mean volume 399.7 ± 39.2 mL, GRWR 2.0 ± 0.7), and ischemic times were minimal (cold: 32.0 ± 32.4 min; warm: 31.7 ± 12.5 min).


Conclusion: APOLT using left lateral sector grafts is a safe and effective treatment for pediatric CNS, providing rapid metabolic correction, minimal complications, and excellent donor outcomes. This approach preserves the native liver for potential future therapies while ensuring stable graft function with standard immunosuppression.