Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR).

In the literature many different decision algorithms exist for BA-associated cholangitis underlining the clear need for standardizing diagnostic criteria. As an illustration, in 2015 the Swiss center introduced structured guidelines to diagnose BA-associated cholangitis. Upon retrospective analysis of SBAR data (2010–2022) we noted significant differences in disease management before and after implementation: The number of multiple diagnosed cholangitis episodes was reduced and we observed a reduced transplantation rate. Building on this, the Biliary Atresia and Related Diseases consensus has developed key diagnostic and management guidelines with objective criteria that have yet to undergo prospective validation. Indeed, prospective multicenter assessment is clearly needed to enhance acceptance and improve outcomes. Further, rigorous evaluation of prophylactic and therapeutic interventions, including antibiotics and immunomodulatory treatments, seems essential, with randomized trials required to establish their effectiveness and explore microbiota modulation in cholangitis management.

The BA-community should strive to establish and adopt a widely accepted protocol for the diagnosis, management and prevention of BA-associated cholangitis. The implementation of such protocols is expected to significantly improve clinical outcomes for these patients.