KidsMD Health Topics

Choledochal Cysts

  • A choledochal cyst is a rare congenital swelling of the hepatic or bile duct of your child’s liver.

    • This is the tract that transports bile produced by the cells to the gallbladder and duodenum (the first part of the small intestine).

    • These cysts can be intrahepatic, meaning that they occur in the part of the duct located inside of the liver.

    • They can also be extrahepatic, meaning part of the bile duct that is located outside the liver.

    Pioneering treatment

    Boston Children's Hospital's Robotic Surgery Research and Training Program pioneered or perfected many of the minimally invasive surgical procedures in use today. Minimally invasive surgery (MIS) is surgery done through small incisions using miniaturized surgical tools and cameras or telescopes. MIS usually results in less pain, less scarring and a quicker recovery time for our patients.

    One form of MIS used to remove choledochal cysts is called laparoscopic surgery, during which surgeons use small instruments guided by a small telescope. The instruments are manipulated by the surgeon who controls their movements, while watching them on a video screen. This form of MIS has been used widely in adults and more recently in children.

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    Boston Children's Hospital
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    Boston MA 02115

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  • What does the liver do?

    The liver is located in the upper right-hand portion of your child’s abdominal cavity, beneath the diaphragm and on top of the stomach, right kidney and intestines.

    The liver consists of two main lobes, both of which are made up of thousands of lobules.

    These lobules are connected to small ducts that connect with larger ducts to ultimately form the hepatic duct. The hepatic duct transports bile produced by the liver cells to the gallbladder and duodenum, which helps to break down fats, preparing them for further digestion and absorption.

    All of the blood leaving the stomach and intestines passes through the liver. The liver processes this blood and breaks down the nutrients and drugs in the blood into forms that are easier to use for the rest of the body.

    What are the most common varieties of these cysts?

    There are five basic types and they’re classified by where they appear.

    • Type 1 — A cystic dilation of the extrahepatic bililary duct. This is the most common type, making up about half of all choledochal cysts.
    • Type 2 — An abnormal pouch or sac opening from the duct.
    • Type 3 —A cyst located within the duodenal wall.
    • Type 4 — Swellings of both the intrahepatic and extrahepatic biliary tracts.
    • Type 5 —Multiple intrahepatic cysts. This type of clustering of cysts is also known as Caroli's disease and is the least common type.

    How common are choledochal cysts?

    • Choledochal cysts occur in between 1 and 100,000 and 1 in 150,000 people in Western countries but is much more common in Japan.
    • They are three to eight times more common in females than males.

    What causes choledochal cysts?
    The cause of these cysts is unknown, but researchers believe that their formation is due to an abnormal connection between the pancreatic and hepatic ducts.

    This abnormal connection leads to a reflux of pancreatic juice into the biliary tract, which could be responsible for cyst formation.

    What are the symptoms of choledochal cysts?

    The following symptoms are usually experienced by an older child whose earlier diagnosis of this congenital anomaly may have been missed.

  • How are choledochal cysts diagnosed?

    Choledochal cysts can be diagnosed prenatally on an ultrasound.

    After your baby is born, you or your child's doctor may notice a right upper quadrant mass, with or without jaundice.

    Your child will likely undergo a combination of the following tests to verify the diagnosis:

    • Computerized tomography scan (CT or CAT scan) — A CT scan shows detailed images of any part of your child’s biliary system.
      • CT scans are more detailed than general x-rays.
    • Cholangiography — This is a radiographic visualization of your child’s bile ducts after ingestion or injection of a substance that illuminates the ducts.
  • What's the treatment for choledochal cysts?

    If your child's doctor suspects the presence of a cyst, a pediatric surgeon will perform exploratory surgery to check your child out.

    If there are any cysts in your child's duct system, the pediatric surgeon can remove them. This could involve removing a lobe of the liver if the cyst is intrahepatic, or part of the extrahepatic duct, followed by duct reconstruction using a piece of intestine.

    Without surgery, there is an ongoing risk of other problems:

    • biliary obstruction
    • infection of the ducts
    • jaundice
    • cirrhosis

    Another long-term concern is for malignant degeneration. Choledochal cysts are inflammatory in nature, which makes them at risk for becoming cancerous if left untreated.

    Your child's surgeon may decide only to remove the cyst lining, to protect the underlying structures. Once the cyst or cysts are surgically removed, your child's biliary duct requires reconstruction.

    This could involve surgical creation of a passage uniting the common bile duct and the small intestine using a piece of small intestine.

  • Less pain, faster recovery

    A surgeon may be able to remove your child’s choledochal cysts with a minimally invasive surgery (MIS) called a laparoscopy. With this technique, small instruments guided by a small telescope are passed through the body.

    The overall safety of MIS techniques appears to be equivalent to open surgery in both children and adults. As with all surgery, the experience of the surgeon and the center where it’s being performed is very important to the outcome of the operation.

    What are the advantages of minimally invasive surgical techniques, such as laparoscopy?

    • very small scars, which are difficult to see after several months
    • less pain after surgery
    • usually a shorter hospital stay

    In many cases children, can go home in one to two days instead of three to four, and can return to their normal activities more quickly.

    What are the disadvantages of these surgical techniques?

    There are many procedures in which MIS is not appropriate for. Technical limitations of MIS equipment, such as lack of dexterity of the tools, make it unsuitable for many pediatric procedures.

    More surgeons are being trained in these methods and it’s becoming a routine part of training in many centers. In rare cases, the operation will not be able to be finished laparoscopically, due to unusual complications from the procedure.

    In these cases, the operation is nearly always be finished by traditional methods of "open" surgery with an incision.

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