Prognosis

First-line treatment is hepatoportoenterostomy (HPE), which provides transplant-free survival for at least 2 years in about half of children.[77] If total bilirubin is less than 34.2 micromoles/L (<2 mg/dL) at 3 months post-HPE, then the chance of being transplant-free at 2 years of age is 84%.[77] In infants with normal serum bilirubin 6 months post-HPE, those with low serum bile acids are less likely to develop portal hypertension, ascites, gastrointestinal bleeding, or progress to requiring liver transplantation.[90] Clinical variables can be used to predict outcomes at later time points in biliary atresia.[91]​​​

The focus of care following HPE is fat-soluble vitamin replacement, nutrition rehabilitation, prevention of cholangitis, and minimisation of the sequelae of portal hypertension.[78]​ Overall, it is thought that 70% or more of affected children will ultimately require liver transplantation.[76]

In the near future, programs implementing newborn screening for biliary atresia may result in earlier intervention and improved outcomes.

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