Extra-hepatic biliary atresia is a rare disease which occurs with the frequency of approximately 1:15,000 live births leading to about 50 new cases / year in the UK. The presentation is with prolonged jaundice, usually in a term baby, who develops signs of obstructive jaundice. The management has been improved by public and professional education to encourage early referral and diagnosis in order to facilitate initial surgery before 8 weeks of age. The surgical management is complementary and includes an attempt to restore biliary flow, the Kasai portoenterostomy, together with liver transplantation for those where this palliative approach fails. Medical management consists of antibiotics, ursodeoxycholic acid to encourage bile flow, fat soluble vitamin supplementation and nutritional support.
Realisation of the need to centralise surgery to specialised centres has improved the current survival of this potentially fatal disease to over 90% in the UK. Over half of infants undergoing a portoenterostomy will clear the jaundice and have an >80% chance of a good quality of life, reaching adolescence without transplantation. For those children who develop intractable complications of cirrhosis and portal hypertension, liver transplantation provides a 90% chance of achieving normal life.
- biliary atresia
- kasai portoenterostomy
- liver transplantation