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  • • 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.

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