Caroli Disease – Types, Symptoms, Detection and Treatment

Caroli disease

is a rare congenital disorder where intrahepatic bile ducts (ducts that carry bile from the liver) are abnormally enlarged. The result would be infection, irritation and gallstone formation. This disease is associated with liver failure and poly cystic ( multiple cysts) kidney disease.

About 1 in 1,000,000 gets affected with this disease.


Types of Caroli Disease

This disease manifests itself in two ways:

  • The first or the simpler one is caused by dilation or widening of the liver ducts or by ectasia ( any dilation of a tubular structure).
  • The second or the more complex one is caused by portal hypertension ( increased pressure in the main portal vein which brings blood from the gastrointestinal tract and spleen and deposits in the liver) or congenital liver hepatic fibrosis (additional fibrous connective tissue being formed in the tubular structures of the duct).


Symptoms of abnormal hepatic widening of bile ducts

  • Fever.
  • Abdominal Pain.
  • Hepatomegaly or enlargement of liver.
  • Multiple cysts on kidneys.
  • Gall stones.
  • Inflammation or Cholangitis of bile duct.
  • Biliary abscess.
  • Kidney failure.
  • Liver Cirrhosis.
  • Cholangiocarcinoma ( Cancer in the tissues of the bile ducts). People having the discussed condition are 100% more prone to such cancers.
  • Infection and Septicemia.

Chances of the disease proving fatal are high, especially from complications of bile duct swelling and cancer and from sepsis.

Detection of the enlargement of bile ducts

  • Advanced imaging techniques like X-ray, CT Scan, MRI will show an enlarged intrahepatic duct. Dilation of the tubes of the ducts can also be seen with ultrasound.
  • In CT Scan, fluid filled structures resembling tubes and extending from the liver indicates the said disorder.

Treatment of Caroli Disease

  • The treatment depends on the current state, location and symptoms of the bile duct abnormality.
    Commonly, the disease is limited to the left lobe of the liver. When the disease has affected only one lobe, a surgical resection of the liver or a hepatectomy can relieve symptoms and reduce chances of cancer.
  • A lobectomy or surgical removal of a lobe of the liver can also bring relief and keep malignancy away.
  • Antibiotics can bring down the swelling of the hepatic ducts.
  • In extreme cases, internal biliary bypass procedure and liver transplantations may become necessary.

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