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

Background:  Kasai procedure (KPE) is a palliative intervention in infants with biliary atresia (BA) aiming to restore biliary drainage. While the measure of success in BA is the post-Kasai native liver survival (NLS), BA remains the most frequent indication for liver transplantation in children. While a considerable amount of children fail to clear their jaundice following KPE, resulting in early liver failure and transplantation, some children become jaundice-free after "successful" KPE. However, sequelae of chronic liver disease might affect those children, becoming a later risk for NLS. While liver transplantation is inevitable in the majority of children, various salvage procedures have been recently described to maintain NLS. This article provides a comprehensive overview of procedures performed after KPE to prolong NLS in BA patients from early childhood to late adulthood and discusses their indications and limitations.

Methods:  A literature-based search for surgical and radiological interventions performed in BA patients after KPE to prolong NLS (salvage procedures) was performed using PubMed. Data from case reports, retrospective studies, and registries were included.

Results:  Fifteen studies included 794 patients who underwent post-KPE salvage procedures. The Oxford Centre for Evidence-Based Medicine levels were IIc to IV.Interventions included redo-Kasai's (n = 710) for cessation of bile flow post-KPE, surgical and radiological procedures (n = 14) for bile lakes, and recurrent cholangitis, shunt surgery (n = 49), and transjugular intrahepatic portosystemic shunt (n = 21) for portal hypertension in BA patients. Age at the time of salvage interventions varied considerably, including redo-Kasai's at 27 days of life and percutaneous biliary interventions in a 35-year-old.

Conclusion:  Salvage procedures can maintain NLS after KPE in BA patients with disease sequelae. However, indications remain scarce and liver transplantation is ultimately unavoidable in the majority of patients. While redo-Kasai numbers are globally decreasing with the advances in liver transplantation, procedures for bile lakes and portal hypertension can be viable options for patients with complications but otherwise stable liver function. Discussion on those procedures should be held by multidisciplinary expert teams, involving pediatric hepatologists, pediatric surgeons, and transplant surgeons to elaborate on the potential of maintaining NLS or proceeding with transplantation.