Endoscopic Retrograde Cholangiopancreatography Study in Alagille''s Syndrome: First Report

Endoscopic retrograde cholangiopancreatography revealed the association between intra- and extra-hepatic biliary tree hypoplasia in an 8-year-old boy affected by Alagille's syndrome. The patient, in whom chronic cholestasis had been present from birth, presented characteristic facies, retarded growth, hepatomegaly, and splenomegaly. Chromosome studies were normal. HIDA Tc 99m appeared late in the gallbladder and emptied into the bowel after 3 h. Endoscopic retrograde cholangiopancreatography showed marked and diffuse narrowing of the extrahepatic biliary ducts and uniform narrowing of the intrahepatic ducts with reduced arborization. Menghini needle biopsy revealed no interlobular ducts in the portal tracts. The last two techniques confirmed the clinical picture and made surgical diagnosis unnecessary. This is, as far as we know, the first time endoscopic retrograde cholangiopancreatography has been used in the diagnosis of Alagille's syndrome.