![]() ![]() This has been linked in some cases to extrinsic factors such as maternal diabetes. There is a syndromic or embryonic form that accounts for 10–20% of cases and is distinguished by other congenital anomalies such as polysplenia, situs inversus, cardiac anomalies (eg, atrial and ventricular septal defects), and absence of the inferior cava, etc (biliary atresia splenic malformation syndrome 3). There appear to be two clinical phenotypes. Type I biliary atresia affects the common bile duct and proximal cystic biliary duct type II biliary atresia affects the common hepatic duct and type III biliary atresia, which is the most common, affects the entire extra hepatic biliary tree. The disease is classified according to the extent of biliary damage. 1, 2 The disease affects both intra and extra hepatic ducts with progressive destruction leading to cholestasis, fibrosis and cirrhosis. It is a rare disease with an incidence of approximately 1:15 000 live births. For those children developing intractable complications of cirrhosis and portal hypertension, liver transplantation provides a 90% chance of achieving normal life.Įxtra hepatic biliary atresia is a disease of unknown aetiology with no proven genetic basis. Over half of infants undergoing portoenterostomy will clear the jaundice and have a greater than 80% chance of a good quality of life, reaching adolescence without transplantation. Centralising surgery to specialised centres has improved survival of this potentially fatal disease to over 90% in the UK. ![]() Medical management consists of antibiotics, ursodeoxycholic acid to encourage bile flow, fat soluble vitamin supplementation and nutritional support. Surgical management is complementary and includes an attempt to restore biliary flow (the Kasai portoenterostomy) and liver transplantation if necessary. Management has been improved by public and professional education to encourage early referral and diagnosis to facilitate initial surgery before 8 weeks of age. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice. Extra‐hepatic biliary atresia occurs in approximately 1:15 000 live births leading to about 50 new cases/year in the UK.
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