Effects of post-hepatic portoenterostomy adjuvant therapy on liver transplantation in children with biliary atresia: A systematic review.

Objectives: Biliary atresia (BA) is a cholangiopathy characterized by obstruction of the intrahepatic and extrahepatic bile ducts. Hepatic portoenterostomy (HPE) is the primary palliative treatment and there is still an urgent need to improve post-HPE management. This study aims to identify post-HPE adjuvant therapies associated with reducing or delaying the need for liver transplantation in children with BA. Methods: This systematic review was registered on the PROSPERO platform (ID: CRD42024553671). Studies were extracted from the following databases: PubMed, Web of Science, Lilacs, and Scielo. Original articles published between 2003 and 2025 in English, Portuguese, and Spanish were included, while studies involving adults, animals, or unrelated topics were excluded. Results: A total of 870 studies were identified, and after applying eligibility criteria, 16 articles were selected. The studies indicated that corticosteroids alone as adjuvant therapy were not associated with improved native liver survival. However, observational studies indicated that corticosteroid therapy combined with antibiotics, anticholestatics, and immunoglobulin may be associated with improvements in jaundice, although only two studies reported increased liver survival. Rectal budesonide demonstrated promising short and long-term results in children with nonsyndromic BA. Conclusions: The use of adjuvant therapy after HPE was associated with improvements in jaundice and cholangitis and, in some studies, with increased liver survival. However, the findings are conflicting and heterogeneous. Further research is needed, with standardized therapeutic approaches to enable more comprehensive analyses.