Caroli’s Disease

 

  • – Congenial non-obstructive gross dilatation of the segmental intrahepatic bile ducts
  • – Associated diseases
  •                   • Medullary sponge kidney
  •                   • Congenital hepatic fibrosis
  • – Bile duct ectasia may diffuse/localized
  • – Both autosomal recessive and dominant inheritance
  • – F=M equally affected
  • – Usually symptomatic in early adulthood
  • – >80% become symptomatic <30 years

                   Symptoms:

  • – Cholangitis
  • – Abscess                ↑→Due to bile stagnation
  • – Septicemia
  • – Gall stone disease Cholangiocarcinoma (<10%)

                   Treatment:

  • – Treatment of cholangitis if present
  • – ERCP
  •              • To remove sludge/stone from the accessible part of biliary tree
  • – Cyst drainage by endoscopic/percutaneous route
  • – Liver transplantation for diffuse disease

          Figure: Caroli’s disease


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