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Key Features

  • Extrahepatic neonatal cholestasis is characterized by

    • Complete and persistent cholestasis (acholic stools) in the first 3 months of life

    • Lack of patency of the extrahepatic biliary tree proved by intraoperative, percutaneous, or endoscopic cholangiography

    • Firm to hard hepatomegaly

    • Typical features on histologic examination of liver biopsy tissue

  • Cause only 2–5% of cases of extrahepatic neonatal cholestasis; incidence is higher in girls and patients of Asian descent

  • In most cases, the clinical manifestations, basic laboratory findings, and histopathologic features on liver biopsy are indistinguishable from those associated with biliary atresia

Clinical Features

  • Cystic lesions located in all or part of the extrahepatic biliary system

  • Recurrent episodes of right upper quadrant abdominal pain

  • Fever

  • Vomiting

  • Obstructive jaundice

  • Pancreatitis

  • Right abdominal mass

  • Infants and children with choledochal cysts are at increased risk for developing bacterial cholangitis


  • Ultrasonography or magnetic resonance imaging (MRI) reveals the presence of a cyst


  • Surgery is indicated for neonates once abnormalities in clotting factors have been corrected and bacterial cholangitis, if present, has been treated with intravenous antibiotics

  • Excision of the cyst and choledocho–Roux-en-Y jejunal anastomosis are recommended

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