<aside> 🟡 Biliary atresia, or obstruction of extrahepatic bile ducts, presents with jaundice, dark urine, and acholic stools in the first 2 months of life due to conjugated hyperbilirubinemia.  Biopsy reveals intrahepatic bile duct proliferation, portal tract edema, and fibrosis.

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• Biliary atresia
  • Jaundice – conjugated hyperbilirubinemia
    • Prolonged neonatal jaundice (> 2 weeks)
    • Onset 2 weeks after birth is possible.
    • Acholic stools, dark urine
    • Hepatomegaly
  • Direct cholestasis defined as direct billurubin more than 20% of total billurubin or direct billurbim more than 2 mg/dl

Biliary atresia

• Definition: obliteration or discontinuity of the extrahepatic biliary system, most commonly of the common bile duct

• Epidemiology

  • Approx. 1:10,000–15,000 live births in the US
  • ♀ > ♂

• Etiology

  • Exact etiology unknown, most likely an embryonal malformation or acquired lesion
  • Approx. 10–35% of cases involve additional anatomical malformations (e.g., situs inversus, asplenia, polysplenia, cardiac anomalies)

• Pathophysiology: discontinuity of the biliary system due to obliteration or fibrosis → obstruction of bile flow (cholestasis) → secondary biliary cirrhosis and portal hypertension

• Clinical features

  • Jaundice
    • Prolonged neonatal jaundice (> 2 weeks)
    • Onset 2 weeks after birth is possible.
  • Acholic stools, dark urine
  • Hepatomegaly

• Diagnosis

  • Laboratory analysis
    • Conjugated hyperbilirubinemia
    • ↑ Aminotransferases and alkaline phosphatase
    • ↑ GGT
  • Ultrasonography
    • Absence of the gallbladder
    • No dilatation of the biliary tree
  • Liver biopsy
    • Active inflammation with bile duct degeneration and fibrosis
    • Bile duct proliferation
    • Portal stromal edema
  • Hepatobiliary scintiscanning (HBSS): failed excretion of the tracer into the bowel
  • Intraoperative cholangiography to confirm diagnosis

  HIDA scan more superior than US differitate between hepatitis and obstruction in infants • However before doing HIDA doctors like to give phenobartione to stimulate bile secretion • HIDA scan in advanced live damage might not be reliable • Liver biopsy is the best to diagnose neonatal hepatitis • Intraopertaive cholangiogram is the best for billary atresia

• Treatment

  • Kasai procedure (hepatoportoenterostomy): a connection is created between the liver and the small intestine to allow for bile drainage.
  • In cases of liver cirrhosis: liver transplantation (primary reason for liver transplantation in children)

• Complications
  • If undetected
    • Early biliary liver cirrhosis (at approx. 9 weeks of age)
    • Children may die within the first 2 years of life
  • Postoperative
    • Cholangitis in 50% of cases
    • Portal hypertension in > 60% of cases