Dr AvinashTank, is a super-specialist (MCh) Laparoscopic Gastro-intestinal Surgeon,
Choledochal Cyst is a 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). Based upon there location and size, there are classified in 5 types.
As theses are congenital (since birth), so its believed that due to abnormal connection between the pancreatic and bile ducts, leading to reflux of pancreatic juice into the biliary tract. There is no known risk factor that might cause it.
In infants, choledochal cysts usually lead to obstruction of the bile ducts and retention of bile. This leads to jaundice and an enlarged liver. 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. In some person, they may have dilated bileduct on ultrasound, but it may be ignored at the time of treatment of gall-bladder stones. Dilated bileduct is leads to stasis of bile, thus invite stone formation in bile-duct. So these people are likely to suffer from complications like jaundice, Cholangitis, pancreatitis etc.
Blood test (LFT: Liver function test), USG are commonly used test for diagnosis. MRCP (MRI of liver) help to define the type of choledochal cyst.
If left untreated, there is a risk of repeated infection in the cyst and bile duct cancer in them. The incidence of cancer (bile duct, hepatic, or gallbladder in origin) in the choledochal cyst ranges from 2.5% to 26%, which is well above the rate of less than 1% for the general population. Many patients have biliary cancer at the time of initial presentation. In infants, 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).
The most-effective treatment is surgery. After excision of the cyst, they are directly connected to the intestine (Hepatico-jejunostomy) to allow direct flow of bile into the intestine. In few cases, part of the liver may have to be removed for removing the cysts (hepatectomy).
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