Choledochal Cyst

What are the signs and symptoms of choledochal cysts?

Children with choledochal cysts have symptoms that stem from poor liver function or from pressure created by the cyst. Not all children with a choledochal cyst have symptoms and they can sometimes be found incidentally. If your child does have symptoms, abdominal pain is usually one of the first symptoms of choledochal cysts. Other signs of choledochal cysts can include:

• Abdominal pain in the right, upper belly
• Jaundice (yellowing of skin and eyes)
• An abdominal lump that you can see or feel
• Pale stools
• Nausea
• Vomiting
• Pancreatitis, which causes abdominal pain that comes and goes, as well as nausea and/or vomiting

What tests are used to diagnose choledochal cysts?

Your child’s doctor usually diagnoses choledochal cysts using ultrasound. In rare cases, they may be diagnosed before a baby is born by prenatal ultrasound. In some cases we may also use CT (computerized tomography) scans, MRCP (magnetic resonance cholangiopancreatography) and ERCP (endoscopic retrograde cholangiopancreatography) to diagnose these cysts.

What can I expect from choledochal cyst testing?

• An ultrasound is a fast, noninvasive imaging test that uses sound waves to create pictures of structures inside the body. An ultrasound technician places a probe against the skin to take pictures. Your child might have brief discomfort when the probe is placed on certain areas of the abdomen. The full scan takes 30 to 60 minutes.
• A CT scan uses X-rays and special computer software to create more detailed pictures inside the body. The scanner is a large donut-shaped machine. Your child lays lay down on a table that moves through the scanner as it takes pictures. CT scans take 10 to 15 minutes. Small children can be swaddled or distracted to help them keep still.
• An MRCP is a type of MRI that uses magnetic waves to take detailed images of a patient’s liver, gallbladder, bile ducts, pancreas and pancreatic ducts. An MRI can be louder and longer than a CT scan, so some small children may need sedation or anesthesia to complete this procedure.
• An ERCP is a procedure that examines the pancreas and bile ducts. A gastroenterologist places an endoscope (small, bendable tube with a light) into your child’s mouth, through the stomach, and through the bile ducts into the small intestine. The doctor injects dye through the endoscope to help create more detailed X-ray images of the bile ducts. ERCP is performed under anesthesia in an operating room.

How do we make a diagnosis for choledochal cysts?

Doctors use the information from imaging tests to diagnose choledochal cysts. Cysts are categorized by where they occur:

• Type 1 — a cyst of the bile duct outside of the liver (up to 90% of all choledochal cysts)
• Type 2 — an abnormal pouch or sac opening from the duct
• Type 3 — a cyst inside the wall of the duodenum (the first part of the small intestine)
• Type 4 — cysts of the bile ducts both inside the liver and outside the liver

How are choledochal cysts treated?

Choledochal cysts are usually removed with surgery. A pediatric surgeon removes the cyst and surrounding tissues and reconstructs the bile duct using a section of the small intestine.

Why choose Children’s Colorado for treatment of choledochal cysts?

At Children’s Colorado, our surgeons are trained in both liver transplant surgery and pediatric surgery, which sets our pediatric hepatobiliary surgery program apart from many other hospitals. We’ve also diagnosed and treated more children with choledochal cysts than any other hospital in the region.

Our multidisciplinary team is made of pediatric ultrasound technicians, nurses, surgeons, gastroenterologists, hepatologists and anesthesiologists who are experts in caring for kids. We understands children and their needs and create customized care plans that ensure the best treatment with less pain, shorter recovery times and fewer missed school days.

Helpful resources

Children’s Liver Disease Foundation provides a comprehensive online information hub and support services for children and young adults with liver disease and their families.