Auxiliary partial orthotopic liver transplantation (APOLT) for Crigler-Najjar syndrome: A retrospective analysis

Abstract

Background

Crigler-Najjar syndrome is an ultra-rare hereditary disorder characterized by severe jaundice and risk of neurological complications. Current treatments, such as phototherapy, have limitations, and liver transplantation is often necessary. Auxiliary partial orthotopic liver transplantation (APOLT) is a potential treatment option. Still, its safety and efficacy in Pakistani patients with Crigler-Najjar syndrome Type I (CNS-I) have not been well established.

Methods

This retrospective study reviewed the outcomes of five pediatric patients with CNS-I who underwent APOLT at a tertiary care center in Pakistan. Patient demographics, surgical details, postoperative course, complications, and follow-up data were analyzed. The primary endpoint was the feasibility and safety of APOLT, while secondary endpoints included improvement in serum bilirubin levels, neurological symptoms, and survival rates.

Results

Among five patients diagnosed with CNS-I, APOLT was performed without intraoperative complications. During the median follow-up period of 6 months, there were no cases of relaparotomy, graft rejection, biliary complications, or portal venous thrombosis. One patient developed a portal venous stricture, but his symptoms were controlled with conservative measures. Postoperative liver function tests showed a significant improvement, with an average reduction of 90% in serum bilirubin levels. There was some improvement in neurological symptoms, and the overall patient and graft survival rate was 100%.

Conclusion

This study suggests that APOLT is a feasible and safe treatment option in patients with CNS-I. It improves liver function, bilirubin levels, and neurological symptoms. Further research with larger sample sizes is warranted to confirm these findings and evaluate the long-term outcomes of APOLT in this patient population.