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September 1974

Biliary Atresia: New Concepts of Pathology, Diagnosis, and Management

Author Affiliations

From the Childrens Orthopedic Hospital and Medical Center, Seattle (Dr. Bill), and the Childrens Hospital, St. Paul (Drs. Brennom and Huseby).

Arch Surg. 1974;109(3):367-369. doi:10.1001/archsurg.1974.01360030019005

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.