Researchers from the University of Helsinki have published a review that provides new insights into long-term liver injury following Kasai portoenterostomy in children with biliary atresia (BA). The study, which appeared in the World Journal of Pediatric Surgery on December 30, 2025 (DOI: 10.1136/wjps-2025-001098), explores how liver fibrosis progresses after surgery and examines current assessment methods.
Biliary atresia is a condition marked by early destruction of bile ducts, leading to cholestasis, inflammation, and rapid fibrosis soon after birth. The Kasai portoenterostomy procedure can restore bile flow in many infants and delay the need for liver transplantation. However, some patients continue to develop progressive liver fibrosis despite initially successful surgery. Current assessment tools rely on invasive biopsies or non-invasive markers that lack accuracy. The biological factors driving fibrosis after surgery also appear different from those present at disease onset.
The review highlights that post-surgical liver fibrosis can follow various paths. While more than half of patients eventually develop cirrhosis, some experience stable or even regressing fibrosis if bile flow is efficiently restored. Molecular studies show that although inflammation decreases after surgery, gene activity related to fibrogenesis and extracellular matrix production continues. A key factor is ductular reaction—an abnormal increase in bile duct-like cells—which correlates strongly with both the severity of fibrosis and survival with the native liver.
Advanced imaging and artificial intelligence-assisted histological analysis suggest these ductular cells play an active role in matrix remodeling rather than being part of a passive repair process. Elevated serum bile acids are identified as important predictors for progression of fibrosis and long-term outcomes, possibly by stimulating ductular reaction and myofibroblast activation. Non-invasive markers such as elastography and serum biomarkers are helpful for detecting advanced disease but have limited sensitivity for early-stage fibrosis.
The authors state: "Understanding fibrosis progression after surgery requires shifting focus from short-term bilirubin normalization to long-term tissue-level changes." They add: "Persistent ductular reaction and bile acid dysregulation represents active disease processes rather than residual damage." According to them: "Improved risk stratification, informed by molecular and histological markers, is essential for identifying patients who may benefit most from emerging antifibrotic or bile acid-modulating therapies."
The findings suggest that reliable non-invasive biomarkers could reduce dependence on repeated biopsies while enabling earlier identification of high-risk patients. Targeting pathways involved in bile acid signaling or ductular reaction may provide new therapeutic options to slow down fibrosis progression and extend native liver survival. The review concludes that long-term management strategies should be based on underlying mechanisms rather than viewing surgical success as an endpoint.
