Overview

During the digestive process to break down the food we eat, your body’s liver (organ located on the right side of the belly) produces bile (digestive fluid) to help digest food. A choledochal cyst (sac filled with air or fluid) blocks the bile ducts (tubes that go from the liver to the small intestines) from carrying bile from the liver to the small intestine. This condition can lead to bile backing up in the liver, liver problems and pancreatitis (inflammation of the pancreas).

Choledochal cysts can develop in the bile duct inside the liver (intrahepatic) or outside the liver (extrahepatic). There are four main types of choledochal cysts:

• Type 1. This type accounts for almost 90% of all choledochal cysts and is located outside the liver.
• Type 2. This type is an abnormal pouch or sac opening on the outside of the bile duct.
• Type 3. This type is a cyst inside the wall of the duodenum (first part of the small intestine, located next to the stomach).
• Type 4. This type causes cysts both inside and outside the liver.

A choledochal cyst is a congenital (present at birth) condition. Children born with this condition have a higher risk of bile duct (digestive fluid tube) cancer later in life. However, proper surgical treatment of a choledochal cyst during childhood decreases this risk.

Symptoms of Choledochal Cyst

Signs and symptoms of this condition may include:

• Fever
• Jaundice (a yellow tint in the eye whites and skin)
• Mass in the abdomen felt through the skin
• Nausea
• Pain in the upper right area of the abdomen
• Vomiting

When to see a doctor

Contact your child’s pediatrician (doctor who specializes in treating children) right away if severe abdominal pain prevents them from being able to stand up straight, or if they have vomiting and nausea.

Causes of Choledochal Cyst

The exact cause is unknown, but choledochal cysts are thought to arise during development in the mother’s womb due to an issue in the formation of the bile ducts.

Who’s at risk

The following factors increase a baby’s risk of being born with a choledochal cyst:

• Female babies are more at risk than male babies
• East Asian ancestry, particularly Japanese, can increase the risk