2025 Volume 61 Issue 2 Pages 158-165
Purpose: In the revision of the clinical practice guidelines (CPGs) for biliary atresia, we investigated the actual state of medical practice before and after the publication of the CPGs and examined its impact on standardizing medical practice.
Methods: A survey was conducted among facilities affiliated with the Japanese Biliary Atresia Society to examine clinical practices and CPGs usage before and after its publication.
Results: Responses were collected from 76 facilities before and 84 after the publication of the CPGs. Over the past five years, 24% of the facilities had no surgical experience, whereas 43% managed 1–4 cases and 27% handled 5–14 cases. Over 80% of the respondents that reported using CPGs expressed satisfaction with the clinical questions, appropriateness, clarity, and usefulness of the algorithm. The use of the stool color card, recommended by the CPGs, increased; moreover, the number of facilities conducting liver biopsies, despite being “not recommended” preoperatively, increased. The importance of early surgery was more widely recognized after publication, with 41% supporting earlier surgery and 19% supporting surgery within 30 days of birth. Laparoscopic surgery remained rare, with only 3% of facilities performing it universally, whereas 92% did not perform it at all. Postoperative steroid use, advised against by the CPGs, decreased slightly from 89% to 82%. The number of facilities using the pediatric end-stage liver disease (PELD) score for primary liver transplantation has also decreased.
Conclusions: The CPGs is being utilized, and future revisions incorporating additional evidence are required to further standardize care and improve treatment outcomes.
