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There are several different types of bile duct disorders. Some babies are born with bile duct disorders, including:
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Biliary atresia.  Condition that results in inflammation and obstruction of the bile ducts, leading to jaundice and cirrhosis.
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Alagille syndrome. Condition characterized by progressive loss of the bile ducts inside the liver and narrowing of the bile ducts outside the liver.
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Choledochal cysts. Condition in which cysts form in the bile ducts, causing them to be abnormally large (dilated). It may also lead to pancreatitis.
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Caroli disease. Rare disorder characterized by abnormal widening (dilation) of the bile ducts that carry bile from the liver.
Other types of bile duct disorders include:
- Primary biliary cirrhosis. Chronic liver disease that causes progressive destruction of the bile ducts in the liver. In some patients it is associated with autoimmune diseases, such as scleroderma or rheumatoid arthritis.
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Primary sclerosing cholangitis. Disease in which the bile ducts inside and outside the liver become inflamed and scarred. This causes bile to accumulate in the liver and damage liver cells, and it often leads to cirrhosis. It may occur on its own, or it may occur with inflammatory bowel disease (chronic problems resulting in irritation and ulcers in the gastrointestinal tract) such as ulcerative colitis.
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Biliary stricture. Abnormal narrowing of the bile duct that transports bile from the liver to the small intestine. It is often caused by trauma to the bile ducts during surgery.

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Biliary dyskinesia. Condition in which the bile duct does not drain properly due to scarring or spasm of the sphincter of Oddi muscle, a small circular muscle located at the end of the bile duct and pancreatic duct. The sphincter of Oddi muscle’s function is to keep bile duct and pancreatic duct muscles closed.
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Gallstone pancreatitis. Inflammation of the pancreas as a result of a gallstone blocking the pancreatic duct. This results in blocking material that flows from the liver and pancreas.
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Bile duct cancer (cholangiocarcinoma). Cancer can develop in the bile ducts. |