What Is a Choledochal Cyst?
If the bile duct becomes sac-like due to a congenital defect, it is called a choledochal cyst.
Why Does a Choledochal Cyst Develop?
It is usually associated with an abnormal congenital connection between the bile duct and pancreatic duct, which can allow abnormal flow of pancreatic or biliary secretions.
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Who Is More Likely to Develop a Choledochal Cyst?
- Approximately 67% of cases occur in children younger than 10 years.
- It is more common in girls, particularly those between 2 and 16 years of age.
- It is reported to be about four times more common in females than males.
Symptoms of a Choledochal Cyst
Many people may have no symptoms. Possible symptoms include:
- Pain in the upper right side of the abdomen.
- Jaundice.
- Fever.
- A mass or lump in the upper abdomen.
How Is a Choledochal Cyst Diagnosed?
Tests may include:
- Abdominal ultrasound.
- Liver function tests.
- MRCP, which is particularly important for defining the bile duct anatomy.
- CT scan.
- ERCP in selected cases. For example, if the patient presents with biliary obstruction and cholangitis, then ERCP should not be conducted as this may cause septicemia.
- CA 19-9 when there is concern about cancer.
Types of Choledochal Cysts
Choledochal cysts are generally classified into five main types. In type I, the bile duct is abnormally dilated, producing a sac-like structure. (Type I is the most common type and accounts for approximately 80%–90% of cases.)
Other types involve different parts of the biliary system.
Complications of Choledochal Cysts
Possible complications include:
- Bile duct infection (cholangitis).
- Pancreatitis.
- Rupture of the cyst.
- Stone formation within the choledochal cyst (cystolithiasis).
- Secondary biliary cirrhosis caused by long-term bile duct obstruction.
- Bile duct cancer (cholangiocarcinoma). The risk of cancer lies at 10%–15%.
Treatment of Choledochal Cysts
Treatment may include:
- Complete excision (removal) of the choledochal cyst.
- Reconstruction of bile drainage by connecting the bile duct to the intestine (biliary reconstruction).
- If a stent was previously placed through ERCP, it should be removed and then the cyst should be excised completely.
- If a previous operation connected the cyst to the intestine but did not completely remove the cyst, additional surgery may be required to remove the remaining cyst because leaving it behind can increase the long-term risk of cancer.
Conclusion
- Choledochal cysts should be diagnosed and treated appropriately as early as possible.
- They can lead to bile duct infection, secondary biliary cirrhosis, and bile duct cancer.
- Timely specialist treatment is important.