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Review
.2006 Jul 26:1:28.
doi: 10.1186/1750-1172-1-28.

Biliary atresia

Affiliations
Review

Biliary atresia

Christophe Chardot. Orphanet J Rare Dis..

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.

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Figures

Figure 1
Figure 1
Acholic white stools.
Figure 2
Figure 2
Operative view of complete extrahepatic biliary atresia.
Figure 3
Figure 3
Hepatoporto-enterostomy (Kasai procedure) [68]*. * Valayer J, Chardot C: Atrésie des voies biliaires. Encycl Méd Chir (Elsevier SAS, Paris, All rights reserved), Techniques chirurgicales – appareil digestif (40–890); 2002: p. 12. Reproduction authorized by Elsevier Masson SAS.
Figure 4
Figure 4
Hepatoporto-cholecystostomy [68]*. * Valayer J, Chardot C: Atrésie des voies biliaires. Encycl Méd Chir (Elsevier SAS, Paris, All rights reserved), Techniques chirurgicales – appareil digestif (40–890); 2002: p. 12. Reproduction authorized by Elsevier Masson SAS.
Figure 5
Figure 5
Survival with native liver of 271 infants who underwent Kasai operation for biliary atresia between 1968 and 1983 at Bicêtre hospital (Paris) [75]*. * Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O: Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology 2005, 41: p. 367. Reproduction authorized by John Wiley and Sons, Inc.
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