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Extrahepatic biliary atresia.

D Alagille

    Hepatology (Baltimore, Md.)
    |January 1, 1984
    PubMed
    Summary
    This summary is machine-generated.

    Extrahepatic biliary atresia, a bile duct disorder, affects 1 in 8,000 infants. Kasai

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    Area of Science:

    • Pediatric Surgery
    • Hepatology
    • Gastroenterology

    Background:

    • Extrahepatic biliary atresia is a congenital condition characterized by the partial or total absence of permeable bile ducts.
    • It leads to total and permanent cholestasis, with an incidence ranging from 1:8,000 to 1:10,000 live births.
    • Without surgical intervention, affected infants typically succumb to the condition between 1 and 2 years of age.

    Purpose of the Study:

    • To evaluate the long-term outcomes of surgical intervention for extrahepatic biliary atresia.
    • To assess the efficacy of Kasai's procedure in improving survival and quality of life.
    • To analyze the histological changes and complications in patients who underwent surgery.

    Main Methods:

    • The study involved 248 infants who underwent surgery for extrahepatic biliary atresia, with 121 operated on before 1977.

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  • Diagnostic methods included the 131I Rose Bengal test and needle liver biopsy for pre-surgical identification.
  • Long-term follow-up included assessment of survival, growth, development of portal hypertension, and histological examination of liver biopsies.

    • Main Results:

    • Out of 121 infants operated on before 1977, 44 (36.6%) survived for at least 5 years post-surgery.
    • Children who survived exhibited normal growth, despite experiencing episodes of cholangitis.
    • Progressive portal hypertension, splenomegaly, and esophageal varices were observed in 23 children; 4 required portal systemic shunts.
    • Liver biopsies revealed cirrhosis in all assessed patients, with absence of biliary ducts or ductules being a prominent histological finding.

    Conclusions:

    • Kasai's procedure, when performed by experienced groups, offers a significant survival benefit for infants with extrahepatic biliary atresia.
    • Despite the presence of cirrhosis and portal hypertension, long-term survivors can achieve a near-normal quality of life.
    • Histological findings of absent biliary ducts correlate with imaging studies and highlight the severity of the condition.