Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Presents within first few weeks of life• 67% of patients ultimately require liver transplantation +++ Symptoms and Signs + • Progressive jaundice in newborns 2-4 weeks old• Mild hepatomegaly• Failure to feed well• Growth failure +++ Laboratory Findings + • Hyperbilirubinemia• Elevated alkaline phosphatase• Elevated transaminases (occasionally) +++ Imaging Findings + • 99mTc-IDA scan: Normal uptake into liver but failure to empty through bile duct into duodenum (100% sensitive, 94% specific)• US: Diminutive or absence of gallbladder, absence of choledochocele + • Age of patient at time of diagnosis will affect eventual success of treatment +++ Rule Out + • Biliary hypoplasia or Alagille syndrome• Other causes of neonatal jaundice• α1-Antitrypsin deficiency + • History and physical• Liver function tests• α1-Antitrypsin level• 99mTc-IDA scan• US• Possible liver biopsy to evaluate for other nonoperative causes of neonatal jaundice +++ Surgery + • Portoenterostomy (Kasai)• Liver transplantation +++ Indications + • Portoenterostomy when proximal bile ducts of adequate caliber are located (150 µm diameter most ideal)• Liver transplantation if > 1 year of age and presence of liver failure or growth retardation following portoenterostomy +++ Medications + • Phenobarbital to facilitate hepatocyte processing of bile preoperatively and postoperatively• Ursodeoxycholic acid to facilitate bile flow postoperatively +++ Treatment Monitoring + • Bilirubin• Temperature• Growth rate +++ Complications + • Cholangitis (40-50%)• Progressive liver failure• Continued bile obstruction +++ Prognosis + • 66-75% with bile flow after portoenterostomy if done before 60 days of age (90-95% if 150 µm ducts found)• Bile flow unlikely if portenterostomy performed after 120 days of life• 30-50% 5-year survival after portoenterostomy (25-35% 10-year)• 66% ultimately require transplantation with ~ 80% 5-year survival +++ References ++Narkowicz MR: Biliary atresia: an update on our understanding of this disorder. Curr Opin Pediatr. 2001;13:435. Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth