Abstract
Biliary atresia (BA) is a rare disease characterised by a biliary obstruction of unknown origin that presents in the neonatal period. It is the most frequent surgical cause of cholestatic jaundice in this age group. BA occurs in approximately 1/18,000 live births in Western Europe. In the world, the reported incidence varies from 5/100,000 to 32/100,000 live births, and is highest in Asia and the Pacific region. Females are affected slightly more often than males. The common histopathological picture is one of inflammatory damage to the intra- and extrahepatic bile ducts with sclerosis and narrowing or even obliteration of the biliary tree. Untreated, this condition leads to cirrhosis and death within the first years of life. BA is not known to be a hereditary condition. No primary medical treatment is relevant for the management of BA. Once BA suspected, surgical intervention (Kasai portoenterostomy) should be performed as soon as possible as operations performed early in life is more likely to be successful. Liver transplantation may be needed later if the Kasai operation fails to restore the biliary flow or if cirrhotic complications occur. At present, approximately 90% of BA patients survive and the majority have normal quality of life.
Highlights
Biliary atresia (BA) is the most frequent surgical cause of cholestatic jaundice in neonates
Biliary atresia (BA) is a rare disease characterised by a biliary obstruction of unknown origin that presents in the neonatal period [1]
The common histopathological picture is one of inflammatory damage to the intra- and extrahepatic bile ducts with sclerosis and narrowing or even obliteration of the biliary tree [2]. This condition leads to cirrhosis and death within the first years of life
Summary
BA is the most frequent surgical cause of cholestatic jaundice in neonates. The common histopathological picture is one of inflammatory damage to the intra- and extrahepatic bile ducts with sclerosis and narrowing or even obliteration of the biliary tree [2]. If the Kasai operation fails to restore the bile flow, biliary cirrhosis progresses and necessitates liver transplantation This is usually performed in the second year of life but may be necessary earlier (from 6 months of life) when there is a rapid aggravation of the liver disease. Some of them are related to characteristics of the disease (and cannot be altered): the prognosis of the Kasai operation is worse when BA is associated with a polysplenia syndrome [17,18,99]; when macroscopic obstructive lesions of extra-hepatic biliary remnant are diffuse (prognosis worsens from type 1 to type 4) [17,55,56,57]; when histological obliteration of the bile ducts (especially at porta hepatis) is more severe [100,101]; and when liver fibrosis is more extensive at time of the Kasai operation (102– 107). In France, a collaborative policy between centres was promoted and a national observatory of BA was created, in order to standardize therapeutic results nationwide and evaluate the results of this decentralized management
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