What is a choledochal cyst?
The term choledochal cyst can be broken down into the following:
chole - relating to bile
dochal - containing or receiving
cyst - fluid collection
A choledochal cyst in a swelling / widening or dilatation of the bile ducts.
The condition usually affects the part of the bile ducts outside the liver (the common bile duct and the hepatic ducts) but sometimes it also affects those inside the liver (the intrahepatic ducts).
We don't know the exact cause of a choledochal cyst but there are a few theories.
One explanation is that it happens due to the bile duct not forming properly when the baby is developing in the womb in early pregnancy. This concept is supported by the fact that in some cases, the cyst is first seen during a routine ante-natal ultrasound scan of the baby in early pregnancy.
Another theory is that the junction of the bile duct with the pancreatic duct is not normal and forms an abnormally long 'common channel'. Currently there are arguments in support of both of these.
Is the condition rare?
Yes, it is rare. It affects about 1 in 50,000 children in the West but it occurs more frequently in Oriental and Asian races. Choledochal cysts are 3 times as common in girls as boys and rarely run in families.
What are the symptoms and signs of a choledochal cyst?
Some of these cysts are found before birth on the antenatal scan. Sometimes, a choledochal cyst is found when a child is having an abdominal ultrasound for an unconnected reason like abdomen pain and in a few cases the child has symptoms such as jaundice, a mass in the abdomen, pancreatitis or cholangitis. Most choledochal cysts are detected in childhood. A child with a choledochal cyst may have some or possible all of the following symptoms:
- Jaundice. This is due to blockage of bile drainage. This might be prolonged jaundice (lasting more than two weeks) in a newborn baby or intermittent jaundice (jaundice which comes and goes) in an older infant or child. Usually, the jaundice is associated with pale coloured stools and dark urine
- Intermittent abdominal pain (abdominal pain which comes and goes) which can be severe
- Cholangitis (an infection of the bile duct causing fever, jaundice, and sometimes shaking attacks called rigors)
- Peritonitis (a severe inflammation within the abdomen) if the cyst bursts or leaks
- A lump or swelling in the belly
- An inflammation (irritation, swelling and damage) in the pancreas
How is a diagnosis made?
The diagnosis is generally made by a combination of the following:
- A physical exam of the child
- Tests including blood tests and an ultrasound scan
What is the treatment for a choledochal cyst?
The treatment of choledochal cysts is surgery. This should be done in a centre which has experience of looking after children who have choledochal cysts and surgeons experienced with this kind of operation. The operation can be performed as key hole surgery (using a small opening through the skin) or as an open procedure (a larger opening in the skin). Both techniques achieve good results.
In the common variety, the cyst is completely removed. This means removing most of the bile ducts (tubes carrying bile) outside the liver together with the gallbladder. The hepatic ducts (tubes from the liver) coming out of the liver are then attached to a loop of the child's bowel so that the bile can drain into the bowel.
Removing the gallbladder has no important long-term effects that we know of. A liver biopsy may also be taken at the time of surgery to see if the liver is healthy or damaged.
In babies with a cyst discovered by ultrasound but who have no symptoms, there is debate about the best time to perform surgery. Children's surgeons who specialise in liver and bile duct surgery often recommend early surgery, generally at about six months of age.
Want to know more about choledochal cysts?
Download CLDF’s leaflet on choledochal cysts.You can also order a copy of the leaflet here.
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