Bile duct cysts are atypical enlargements that develop within your biliary system. They may disrupt the normal flow of bile from the gallbladder and liver to your small intestine.

Bile duct growths are also called “choledochal cysts,” and they can also develop outside of your liver.

Most bile duct cysts are detected during childhood before they can lead to long-term health problems involving the liver, pancreas, and gastrointestinal system at large. While considered rare, this is a serious condition that may pose health risks and may require surgical treatment to prevent complications.

Learn more about bile duct cysts, including the symptoms, types, and complications as well as the risk factors for this biliary condition.

There are five main types of bile duct cysts, with between 80% and 90% classified as type 1. Types are classified based on location and the severity of enlargement of the bile ducts. The different types of bile duct cysts are:

  • Type 1: occurs in the common bile duct (after the cystic duct from the gallbladder meets the common hepatic duct that carries bile from the liver)
  • Type 2: an isolated cyst that protrudes out of the common bile duct
  • Type 3: also known as a “choledochocele,” an enlargement of the space where the common bile duct meets with the pancreatic duct
  • Type 4: multiple cysts involving bile ducts in or outside of the liver
  • Type 5: also called “Caroli disease,” which involves cysts of the ducts that carry bile inside the liver (intrahepatic bile ducts)

Signs and symptoms of bile duct cysts may develop as a result of blockage of the bile ducts, called bile flow obstruction.


Possible symptoms of a bile duct cyst include:


Aside from symptoms you may be experiencing, a doctor may also detect other possible signs of bile duct cysts after a physical exam, such as:

This condition is thought to be attributed to certain genetic mutations.

For example, type 5 (Caroli disease) is thought to be caused by an inherited PKHD1 gene mutation, which also causes autosomal recessive polycystic kidney disease (ARPKD). Bile duct cysts and ARPKD commonly occur together in such cases.

Some researchers also believe that bile duct cysts are acquired, though the causes are unknown.

A bile duct cyst is considered a congenital condition, which means it’s present at birth. The condition is also sometimes called “choledochal cyst disease.”

Bile duct cysts are most commonly diagnosed during childhood. However, this biliary system condition isn’t entirely exclusive to children. It’s estimated that up to 20% of cases are detected in adulthood.

Advanced types of bile duct cysts, such as those seen in Caroli disease, are even rarer and affect 1 in 10,000 people at birth. According to current research, all genders are affected equally, but Caroli disease is more prevalent in people of Asian descent.

Potential complications of bile duct cysts include:

There’s also a possibility that bile duct cysts may become cancerous (malignant). Long-term inflammation and recurring infections may contribute to the risk of malignant bile duct cysts.

While considered rare, up to 2% of people may experience biliary tree malignancy, a type of cancer that may occur after surgical removal of bile duct cysts.

If you or a loved one is experiencing possible symptoms of a bile duct cyst, it’s important to see a doctor who can help diagnose the underlying issue and offer treatment options if needed.

You may also want to speak with a doctor if you have symptoms and have a family history of bile duct cysts or ARPKD.

Aside from an initial physical exam, bile duct cysts are diagnosed with imaging tests. These tests include a CT scan or an ultrasound, which can help provide images of the bile ducts and allow a doctor to detect signs of unusual dilation.

The only way to treat bile duct cysts is to remove them. There are no known home remedies that will treat this biliary system disease.

Speak with a doctor about the following information regarding bile duct cyst treatment.

Medical and surgical treatments for bile duct cysts

Treatment for bile duct cysts consists of surgery to remove these atypically dilated structures. Surgery involves the complete removal of the cyst followed by biliary tree reconstruction.

Such measures are considered far more effective than previous surgeries that only focused on draining the cyst, leaving the risk of the cyst returning or becoming malignant.

In some cases, antibiotics may be provided for infected bile duct cysts (cholangitis). However, treating the infection doesn’t cure these cysts.

A diagnosis of one or more bile duct cysts can raise a lot of questions regarding treatment and complications. Consider discussing the following information with a doctor:

When does a bile duct cyst need surgery?

Surgery is the only effective way to treat bile duct cysts. Surgical removal improves short-term problems with bile backup in the gastrointestinal system and also decreases your risk of long-term complications such as bile duct cancer.

Is a bile duct cyst a medical emergency?

A bile duct cyst can be a medical emergency, especially if you’re experiencing an acute infection. Acute cholangitis is considered life threatening and may lead to septic shock if left untreated. Always call 911 or your local emergency services in the case of an immediate emergency.

Bile duct cysts are areas of dilation within the biliary system that connects the liver, gallbladder, and small intestine. The cysts are primarily a congenital condition diagnosed in children, though it’s possible to develop bile duct cysts as an adult.

While benign, it’s possible for these cysts to become cancerous at some point. Surgery is the only effective treatment, and removal can also help reduce your risk of future complications.