From accumulated modern experience we conclude that biliary atresia is basically a hepatitis with a component of sclerosing cholangitis of the extrahepatic ducts. This condition leads to irreversible hepatic cirrhosis within two or three months of onset. Diagnosis is made from light-colored stools, percutaneous liver biopsy showing intracanalicular bile plugs and proliferation of the end ductules, a steady or rising bilirubin level, and a rose bengal scan over a three-day period showing retention of dye in the liver. Kasai, in Japan, anastomosed the end of a double Roux-en-Y loop to a shallow dissection in the porta hepatis of the liver where the intrahepatic ducts should be. If done before the patients are 3 months old, the procedure results in a 40% one-year survival without jaundice. We have duplicated these results in the United States.
Bill AH, Brennom WS, Huseby TL. Biliary Atresia: New Concepts of Pathology, Diagnosis, and Management. Arch Surg. 1974;109(3):367–369. doi:10.1001/archsurg.1974.01360030019005
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