title: Maximizing Kasai portoenterostomy in the treatment of biliary atresia: medical and surgical options.
abstract: Biliary atresia (BA) remains one of the most challenging conditions in paediatric surgery. It has several possible causes, resulting in a range of different clinical scenarios. The current therapeutic approach is almost entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai-type portoenterostomy. Liver transplantation (cadaveric or living donor) is usually reserved for failure or for infants presenting late with end-stage cirrhosis. The role of adjuvant medical therapy is unclear and evidence of benefit is lacking. Nonetheless, the use of post-operative steroids, prophylactic antibiotics and choleretic agents such as ursodeoxycholic acid is common. Ideally, the entire pathway should be complementary and seamless with few infants dying of end-stage liver disease or uncorrectable associated congenital malformations. Experience from high-volume centres suggests that clearance of jaundice can be achieved in 50 - 60% of infants with 10-year native liver and real survival rates of 45% and 90%, respectively.
authors.affiliation: Department of Paediatric Surgery, Kings College Hospital, London, UK. markdav2@ntlworld.com
authors.forename: Mark
authors.initials: M
authors.lastname: Davenport
authors.forename: Andrew
authors.initials: A
authors.lastname: Grieve
mesh_terms: D001656:Biliary Atresia / Q000601:surgery* / Q000628:therapy*; D002761:Cholangitis / Q000517:prevention & control; D006801:Humans; D007223:Infant; D018529:Nutritional Support; D011171:Portoenterostomy, Hepatic / Q000379:methods; D016896:Treatment Outcome