Optimizing Post-Kasai Management in Biliary Atresia: Balancing Native Liver Survival and Transplant Timing.

Area of Science:

• Pediatric Surgery
• Hepatology
• Transplantation Medicine

Background:

• Biliary atresia (BA) is a leading cause of pediatric liver transplantation.
• Kasai portoenterostomy (KPE) is a palliative surgery to restore bile flow.
• While some patients achieve jaundice clearance post-KPE, long-term native liver survival (NLS) can be affected by chronic liver disease sequelae.

Purpose of the Study:

• To provide a comprehensive overview of salvage procedures performed after KPE in BA patients.
• To discuss the indications and limitations of these interventions for prolonging NLS.
• To analyze outcomes of various salvage procedures in BA patients.

Main Methods:

• Literature-based search of PubMed for interventions after KPE to prolong NLS.
• Inclusion of data from case reports, retrospective studies, and registries.
• Analysis of surgical and radiological interventions, including redo-Kasai's, procedures for bile lakes and cholangitis, and shunts for portal hypertension.

Main Results:

• Fifteen studies encompassing 794 patients undergoing post-KPE salvage procedures were analyzed.
• Interventions included redo-Kasai's (n=710), procedures for bile lakes/cholangitis (n=14), shunt surgery (n=49), and TIPS (n=21).
• Patient ages at intervention varied widely, from neonates to adults, highlighting the long-term applicability of some procedures.

Conclusions:

• Salvage procedures can support NLS in BA patients with post-KPE complications.
• Indications for salvage procedures are specific, and liver transplantation is the ultimate solution for most.
• Procedures for bile lakes and portal hypertension offer viable options for select patients, requiring multidisciplinary expert team evaluation.