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Choledochal cyst

Q:What Is Choledochal cyst?

Choledochal Cyst is a rare congenital (present at birth) defect where the bile ducts (tubes that carry bile from liver to intestines) are dilated and wider than usual. These cysts may be intra-hepatic (bile ducts inside the liver) or extra-hepatic (outside the liver).

Q:What are the types of Choledochal cyst?

Choledochal cysts are described into 5 types depending on their location in the bile duct and their shape. Type I cysts are sac like and are the most common type, representing 80-90% of choledochal cysts. Type II cysts appear as an outpouchings from the wall of the bile duct, sometimes joined to the common bile duct by a narrow stalk. Type III choledochal cysts, also called choledochocele arise from the lower part of bile duct into the duodenum (part of intestine). Type IV consist of multiple cysts involving the bile ducts. Type V choledochal cyst, also called Caroli disease, consists of multiple cysts limited to intra-hepatic bile ducts.

Q:How common is the problem? Who is more likely to be affected?

Choledochal cysts occur in about 1 in 100,000 people in western countries, but is more common in Japan, where it occurs in about 1 per 1000 population. They are more often found in females.

Q:Why does it happen? How does it progress?

The cause of these cysts is unknown, but it is believed that due to abnormal connection between the pancreatic and bile ducts, leading to reflux of pancreatic juice into the biliary tract, choledochal cysts form.

If left untreated, there is a risk of repeated infection in the cyst and bile duct cancer in them.

Q:What are the signs and symptoms of Choledochal cyst ?

The classic symptoms of choledochal cysts are abdominal lump, pain and jaundice. Some children may not show symptoms for years but about two thirds of them have symptoms before 10 years of age. In Infants, symptoms are usually jaundice, acholic (white / clay colored) stools and a lump in the right upper abdomen. Older children have intermittent attacks of pain in the abdomen due to biliary obstruction or bouts of pancreatitis (swelling of the pancreas).

Q:Which tests and examinations are required to diagnose / evaluate this disease?

Q:What is the best treatment for choledochal cyst?

The best way of treating choledochal cysts is to surgically remove completely when possible. Once removed, the bile ducts may need reconstruction. Mostly however, after excision of the cyst, they are directly connected to the intestine (Hepatico-jejunostomy) to allow direct drainage of bile into the intestine. In few cases, part of the liver may have to be removed for removing the cysts (hepatectomy).

Q:What is the expected prognosis (Outlook) after various treatments?

Long term outcome of choledochal cyst is good, if treated at optimum timing. The outcome is poor for patients who develop bile duct cancer in the cysts.