Choledochal Cyst

What is choledochal cyst?

Choledochal (pronounced coal-ee-DOCK-uhl) cyst is a problem with the tubes (ducts) that carry bile from your child’s liver to their gallbladder and intestine. The tubes get bigger than normal (dilate) or pouches form on the tubes. This keeps bile from flowing well, which can cause liver problems.

Bile is a liquid that helps the body break down (digest) fats. Normally, bile flows through tiny bile ducts within the liver into larger ducts. Then bile leaves the liver through 2 main ducts. Outside the liver, these ducts join to form the common bile duct.

The common bile duct connects the liver to the gallbladder and the gallbladder to the small intestine. The gallbladder stores bile until the body needs it to digest food. Food triggers the gallbladder to contract and squeeze bile out into the intestine.

Cysts can block the flow of bile. When bile doesn’t flow well, the bile ducts can get swollen and irritated (inflamed) or infected. This is called cholangitis. Over time, it can cause scars in the liver (cirrhosis). Then the liver cannot work well. Children with choledochal cysts may also get an inflamed pancreas (pancreatitis).

Choledochal cyst in children

Choledochal cyst is present at birth (congenital). It happens while a baby is forming, before they are born.

  • What causes choledochal cyst?

    Doctors aren’t sure why cysts form. One reason may have to do with the pancreas. Normally, a duct from the pancreas joins with the common bile duct right before it goes into the small intestine. Muscle in the wall of the intestine keeps pancreas juices from flowing up into the bile duct. In some babies, the duct from the pancreas joins with the common bile duct higher up, so the muscle cannot keep the pancreas juices out of the bile duct. Instead, the juices flow up the common bile duct. They damage and weaken the wall of the duct as it forms.

  • Five types of cysts

    There are 5 main types of choledochal cysts. The type is based on where the cyst is in the ducts and what shape the cyst has.

    • In type I, the common bile duct dilates, or expands. It looks more like a small balloon than a tube. About 9 out of 10 cysts are type I.
    • In type II, a pouch (diverticulum) forms off of the common bile duct.
    • In type III, the bottom end of the duct dilates inside the small intestine. This is sometimes called choledochocele (pronounced kuh-LED uh-koe-SEAL).
    • In type IV, the bile ducts inside the liver and outside the liver dilate.
    • In type V, the large ducts inside the liver dilate and the ducts outside the liver are normal. This type is also called Caroli disease.
  • Are cysts hereditary?

    Girls are 3 to 4 times as likely as boys to have choledochal cysts. Babies of Eastern Asian descent (including Southeast Asian descent) are more likely to have cysts than babies from other family backgrounds.

    These factors suggest that genes passed from parents to babies (especially genes passed to girls) might be 1 cause of cysts. But having one baby with a cyst does not mean you will have another baby with the same problem. Other factors besides genes must play a part too.

  • Risks of choledochal cyst

    People who have had choledochal cysts have a higher chance of getting cancer of their bile ducts (cholangiocarcinoma). This mainly affects adults with cysts because they have had their cysts for many years. The chance of getting cancer is much lower if cysts are removed early in life

Choledochal Cyst at Seattle Children’s

  • The experts you need are here

    We have treated many babies as well as older children with choledochal cysts at Seattle Children’s. Our surgeons are experienced at performing the surgeries these children need to remove their cyst and create good bile flow.

    When you come to Seattle Children’s, you have a team of people to care for your child. Along with your child’s surgeon, you are connected with nurses, dietitians, child life specialists and others. We work together to meet all of your child’s health needs and help your family through this experience.

  • We understand the unique needs of children

    Since 1907, Seattle Children’s has been treating children only. Our team members are trained in their fields and in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. Our expertise in pediatrics truly makes a difference for our patients and families.

Symptoms of Choledochal Cyst

Some newborns with cysts have symptoms right away. Others have good bile flow at first, but they get symptoms soon after birth. Some children don’t have symptoms for a couple of years or longer. Some people live with a choledochal cyst for many years but don’t know about it because they don’t have symptoms until they are an adult.

Babies or children with a choledochal cyst may have these symptoms: 

  • Yellow color to the eyes and skin (jaundice)
  • Pain in the upper-right belly (upper-right-quadrant pain)
  • Soft mass that can be felt in the upper-right belly
  • Pale or clay-colored stools (feces)
  • Fever, if they have an infection (cholangitis)
  • If they have an inflamed pancreas (pancreatitis): belly pain that may come and go, nausea and vomiting

Diagnosing Choledochal Cyst

  • Prenatal ultrasound

    Sometimes doctors can see a choledochal cyst on an ultrasound before a baby is born (prenatal ultrasound). If this happens with your baby, doctors will plan the tests and treatment your baby will need after birth. It’s not always possible to see a cyst before birth.

  • Exam and lab tests

    If your child’s cyst was not seen but your child has symptoms after they are born, your child’s doctor will ask for a detailed history of your child’s illness. The doctor will do a thorough exam.

    There is no blood test for choledochal cysts. But the doctor may do blood tests to check whether your child’s bile ducts are inflamed or infected and whether their liver is working well.

  • Body imaging

    The doctor will do an ultrasound of your child’s belly (abdomen) to see your child’s bile ducts and other organs in the area. Imaging studies like ultrasound help the doctor know whether there is a cyst, where it is and how it is shaped. These factors help your child’s doctor plan surgery to remove the cyst.

    Your child may also need other imaging studies, like these: 

    • Cholangiography: injecting dye (contrast) into your child’s bile ducts and then taking an X-ray . This may be done in 1 of 2 ways. One way is to inject the dye through your child’s skin and liver into their ducts (percutaneous transhepatic cholangiography, PTC). The other way is to inject the dye through a small tube placed into your child’s mouth and down their throat into their intestine (endoscopic retrograde cholangiopancreatography, ERCP).
    • Abdominal CT (computed tomography) scan.
    • Abdominal MRI (magnetic resonance imaging) scan.
    • Hepatobiliary iminodiacetic acid scan (HIDA scan). A HIDA scan allows doctors to see whether a special dye that collects in the liver can pass out of the liver through the bile ducts.

Treating Choledochal Cyst

The treatment for a choledochal cyst is surgery. The purpose is to remove the cyst and create good bile flow from the liver to the small intestine.

  • Surgery for type I cysts

    For children with type I cysts, the surgery is called Roux-en-Y hepaticojejunostomy (pronounced roo-enn-WHY hee-PAT-ih-koe-jeh-joo-NOS-tuh-mee). “Hepatic” has to do with the liver. “Jejunum” is the middle segment of the small intestine.

    At the time of surgery, we give your child medicine to make them sleep without pain (general anesthesia).

    Your child’s surgeon cuts the common bile duct on each side of the cyst and takes out the cyst.

    Next the surgeon cuts through the small intestine. The surgeon brings the lower part of the intestine up and sews it to the bile duct. This makes a new complete tube. Bile can now flow from your child’s liver into their intestine.

    Then the surgeon cuts a hole in the side of the new tube. The surgeon brings the upper part of the intestine down and sews it to this hole. Now food can move from your child’s stomach into their intestine.

  • Surgery for other types of cysts

    For children with other types of cysts, the surgery may be different. Your child’s surgeon will explain the type of surgery that’s best for your child and will describe the steps to you. In most cases, the purpose of surgery is the same — to take out any cyst that is outside the liver. (Surgery is different for type III cysts, or choledochocele.) If cysts are inside the liver and causing liver problems, your child might need one part (lobe) of their liver removed. This is not common.

  • Laparoscopic or open surgery?

    Some children can have their surgery through small cuts (“keyhole” incisions) in their belly (laparoscopic surgery, or minimally invasive procedure). The surgeon may do this directly or in some cases may do it using the da Vinci robot. For some children, surgeons need to make one longer incision, called an open approach, in order to reach the cyst and intestine. Your child’s surgeon will explain the method that is best for your child.

  • After surgery

    After surgery, your child will be in the recovery room for about an hour. Then they will go to a regular hospital room and eventually to home. Usually, children with choledochal cyst stay in the hospital 7 days. This depends on factors like when your child gets back to eating normally and when their pain is well controlled with medicines taken by mouth. Often children need a drain left in the side of their belly after surgery. In most cases, we will remove this before your child goes home.

    Your child will have a follow-up visit with the surgeon 2 to 3 weeks after the surgery to make sure their incision is healing well.

    After healing from surgery, your child will need to visit the doctor from time to time for regular check-ups and if they have any symptoms. It’s important to make sure their bile keeps flowing well.

  • Medicine for infection

    If your child has infected bile ducts (cholangitis), the doctor will give your child antibiotics to fight the infection. This may need to be done in the hospital.

Contact Us 

Contact Seattle Children’s Gastroenterology and Hepatology Department at 206-987-2521 for a referral, a second opinion or more information.

Providers, see how to refer a patient.