Diagnostic and management guidelines for biliary atresia in 2025

Biliary atresia (BA) is a severe pediatric biliary disorder characterized by the progressive obstruction of liver bile ducts. In the absence of treatment, fibrosis advances rapidly in most affected children. In recent years, there has been significant progress in the management of BA, but many challenges remain. This guideline provides expert opinions on the screening, diagnosis, treatment, and follow-up of BA, aiming to assist and guide clinical practice.

The guideline steering group, guideline development group, and guideline review group were formed to formulate clinical questions, develop recommendations, and draft guidelines using the GRADE Grid method, nominal group technique (NGT), and Delphi voting method. Three offline meetings were held on November 5, 2022, June 10, 2023, November 10, 2023, and October 27, 2024, respectively, to vote on the recommendations and solicit comments and suggestions from all participating experts. All experts from the United States, Europe, and Asia contributed significantly to this consensus guideline.

After summarizing high-quality literature on clinically encountered issues such as early screening, complementary checkup, treatments, follow-up, vaccinations, growth, development, and neurocognition, 23 observations were made and strength of recommendations were given.

International guidelines for BA can guide surgeons, patients, medical societies, hospital administrators, and relevant community groups in their current practice. The diagnosis and treatment of BA remains controversial internationally and more research evidence is needed.