Biliary atresia: 50 years after the first kasai

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.

Figure 1

Acholic, discolored stool of a baby with biliary atresia.

Figure 2

(a) Complete biliary atresia with a shrunken, fibroticgall bladder (arrow) and biliary remnant (white star) in a 2-month-old baby. Note the already cirrhotic aspect of the liver. (b) Hilar region after removal of the biliary remnant (white star), surrounded by the hepatic artery's right branch (fine arrow) and the portal vein's left branch (large arrow). (c) Final aspect of the Kasai hepato-porto-enterostomy with the Roux-en-Y limb (black star) anastomosed to the hilar region.