Choledochal Cysts

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Medical Author: Jay W. Marks, MD
Medical Editor: Melissa Conrad Stöppler, MD

• What is a choledochal cyst?
• What are the symptoms and complications of choledochal cysts?
• How are choledochal cysts diagnosed?
• How are choledochal cysts treated?

What is a choledochal cyst?

Bile that is produced in the liver flows through increasingly larger channels (ducts) within the liver and finally into even larger ducts that leave the liver, pass through the substance of the pancreas, and then empty into the duodenum.

A choledochal cyst is a cyst (hollow outpouching) of the bile ducts. Choledochal cysts have been classified into several different types depending on where they are located and whether they can be seen as separate structures from the ducts (diverticulum-like); or whether they can be seen as a localized dilation (enlargement) of the ducts. Choledochal cysts are rare, occurring in less than 1% of individuals. The cause of choledochal cysts is unknown, but they are congenital, that is, present from birth, and, therefore, represent developmental abnormalities of the bile ducts in the fetus.

What are the symptoms and complications of choledochal cysts?

In infants, choledochal cysts usually lead to obstruction of the bile ducts and retention of bile. This leads to jaundice and an enlarged liver. If the obstruction is not relieved, permanent damage may occur to the liver - scarring and cirrhosis - with the signs of portal hypertension (obstruction to the flow of blood through the liver) and ascites (fluid accumulation in the abdomen). There is an increased risk of cancer in the wall of the cyst.

In older individuals, choledochal cysts are more likely to cause abdominal pain and intermittent episodes of jaundice and occasionally cholangitis (inflammation within the bile ducts caused by the spread of bacteria from the intestine into the bile ducts). Pancreatitis also may occur. The cause of these complications may be related to either abnormal flow of bile within the ducts or the presence of gallstones.

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