Choledochal Cyst
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
Diagnosis of Choledochal Cyst
In some cases, a prenatal (pregnancy) ultrasound can reveal your child’s choledochal cyst before birth. If the condition is not diagnosed before birth, a choledochal cyst may be found at any time during childhood. Typically, a choledochal cyst is found when a child visits their pediatrician (doctor who specializes in treating children) for abdominal pain. Your child’s doctor may use one or more of the following tests to make a diagnosis:
Computerized tomography (CT) scan. This test uses X-ray and specialized computers to create detailed images of your child’s gallbladder, bile ducts and surrounding structures.
Intraoperative cholangiography. This test uses ultrasound and X-ray to create detailed images of your child’s gallbladder, bile ducts and surrounding structures. The surgeon performs this imaging test at the time of your child’s surgery.
Ultrasound. This test helps your child’s doctor get a closer look at the gallbladder and determine if your child has a cyst and possibly dilated biliary tree.
Magnetic resonance cholangiopancreatography (MRCP). This test helps your child’s doctor closely check the tubes that are in the liver and that extend to the pancreas. This is a safe option for more detailed pictures.
Treatments for Choledochal Cyst
If your baby or child has this condition, they will need surgery to remove the cyst or cysts. Having the cysts removed at a young age can reduce your child’s risk of developing bile duct cancer in later life. For this reason, your child may either be monitored for a period of time with repeat imaging, or surgery may be scheduled soon after your child is diagnosed with this condition.
In most cases, your child’s surgeon will approach this operation with a minimally invasive technique called laparoscopic surgery. For the surgery, your child will be under general anesthesia (fully asleep). To begin, the surgeon will make tiny incisions (cuts) in your child’s abdomen. They then will insert a tiny camera into one hole and thin surgical tools into the others.
Your child’s surgeon will use a monitor to see inside your child’s abdomen using the surgical camera. The surgeon will remove the cyst or cysts with the surgical tool through one of the incisions. This may involve other surgical procedures as deemed necessary, such as a liver biopsy or a cholangiogram (contrast in the biliary tree). The surgeon will then make a connection between the liver structures and the intestines to allow for bile flow. After removal, the surgeon will close the incisions with stitches and bandages.
Your child will spend several hours in a recovery area. The care team will carefully monitor your child as they wake up from the anesthesia. An intravenous (IV) line will supply fluids through a vein to help keep your child hydrated and avoid dehydration. Once your child begins showing signs of progress (such as bowel function) and they are cleared by the surgical team, they will be able to drink clear liquids and, later, solid foods.
Your child will spend a minimum of three to five days in our hospital following the surgery. During this time, they may receive medicines to reduce the risk of infection and/or manage any pain. Your child will typically see their surgeon for a post-operative appointment and then again around six weeks after their procedure. After that, your child will see their gastroenterologist for routine lab work and monitoring.
Contact Us
If you suspect that your child may have Choledochal Cyst, make an appointment with an Orlando Health pediatrician today so your child can start on the road to improved health.
