Prognosis

If untreated then 98% of children will die before the age of two years.

In the UK survival is over 90% (1)

• in the majority of children (60%) biliary drainage will be achieved after Kasai portoenterostomy
  • serum bilirubin will return to normal within 6 months (2)
  • 80% of children will reach adolescence without the requirement for liver transplantation (2)
  • study evidence suggests (3) that it is possible for patients with biliary atresia to survive more than 20 years on their native liver after undergoing the Kasai operation during early infancy - in this study 88% of patients survived for more than 20years
    • however, 60.5% of the long-term survivors alive on their native liver end up suffering from progressive liver-related complications

If liver transplantation is required (1)

• provides a 90% chance of achieving a normal life

Reference:

• Kelly DA, Davenport M; Current management of biliary atresia. Arch Dis Child. 2007 Dec;92(12):1132-5.
• Hartley JL, Davenport M, Kelly DA; Biliary atresia. Lancet. 2009 Nov 14;374(9702):1704-13.
• Bijl EJ et al.The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review. Neth J Med. 2013 May;71(4):170-3.