Biliary Atresia

• • 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