Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content ++ 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 ++ Diagnosis ++ Ultrasonography or magnetic resonance imaging (MRI) reveals the presence of a cyst ++ Treatment ++ 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 GET ACCESS TO THIS RESOURCE Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options