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ISRN Surgery
Volume 2012 (2012), Article ID 132089, 15 pages
http://dx.doi.org/10.5402/2012/132089
Review Article

Biliary Atresia: 50 Years after the First Kasai

Division of Pediatric Surgery, Department of Pediatrics, University Hospital of Geneva, 1211 Geneva, Switzerland

Received 23 September 2012; Accepted 30 October 2012

Academic Editors: D. W. Blackhurst, J. C. Cendan, D. Laub, and D. E. Ziogas

Copyright © 2012 Barbara E. Wildhaber. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.