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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 theduodenum. 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.
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.
Papilla of Vater (the point at which the bile duct enters the small intestine, also known as the Ampulla of Vater). The most recent technique for identifying choledochal cysts is magnetic resonance imaging, also known as MRI.