Choledochal cysts are a congenital issue (meaning present from birth) with the tube-like duct that transports bile from the liver to the small intestine and gallbladder. Bile is a fluid produced by the liver that helps the body digest food.
Choledochal cysts can lead to swelling of the bile duct, which can cause bile to back up into the liver. Caused by blockage of the main bile duct from the pancreas to the intestine, this backflow of bile into the liver can lead to issues with liver function or inflammation of the pancreas (A.K.A. pancreatitis).
Choledochal cysts are fairly rare, occurring in one in every 100,000-150,000 births in the U.S. Girls are four times more likely to have choledochal cysts than boys. Though usually diagnosed in children, about 20% of choledochal cysts are diagnosed in adults.
If left untreated, choledochal cysts can increase the likelihood of developing bile duct cancer (AKA cholangiocarcinoma) in adulthood. In rare cases, choledochal cysts can be congenital, meaning they can be passed down from parent to child.
Choledochal cysts are classified based on their location within the ducts and their shape. There are five main choledochal cyst types, including:
Type I involves the dilation or expansion of the common bile duct, so that it resembles a small balloon rather than a tube. This type accounts for approximately 90% of all cysts.
Type II manifests as a pouchlike space (AKA a diverticulum) that forms off the common bile duct.
Type III (A.K.A. a “choledochocele”) involves the dilation of the lower end of the duct within the small intestine.
Type IV involves the dilation of both the bile ducts inside and outside the liver.
Type V involves dilation of the large ducts inside the liver, while the ducts outside the liver remain normal. This type is also referred to as Caroli Disease.
The biliary system is a complex and sometimes delicate structure. Treating issues involving the bile ducts requires a treatment center with the tools, techniques, advanced imaging and experience to repair issues without causing more damage.
In Orange County, that center is The Hoag Digestive Health Institute. Hoag features the most comprehensive and experienced hepatobiliary program in Orange County, offering advanced, hyper-accurate diagnosis and next-generation treatment options for serious conditions of the bile ducts, pancreas, liver and gallbladder.
There’s a reason why U.S. News and World Report has named Hoag to their Best Hospitals list in the field of Gastroenterology and GI Surgery. Better and more personalized care. Advanced imaging to pinpoint problems and drive personalized treatment. A program that sees you as more than just your condition.
Has someone you love been diagnosed with a choledochal cysts? Don’t wait. When everything is on the line, trust Hoag Digestive Health Institute to help you heal.
The symptoms of choledochal cysts may appear at birth, or may not become noticeable for several years. Common signs of choledochal cysts are different for every patient, but common bile duct cyst symptoms in newborns and older children may include:
A noticeable swelling in the upper-right abdomen that can be seen and felt through the skin.
Pain in the right upper abdomen
Jaundice, which is a yellowing of the skin and the whites of the eyes
Unexplained nausea and vomiting
Fever that may be caused by a cholangitis infection. Learn more about cholangitis
Pale or clay-colored stools, which may indicate that bile isn’t reaching the intestines
Abdominal pain that can come and go, which may be related to an inflammation of the pancreas called pancreatitis. Learn more about pancreatitis.
Cholangiocarcinoma, which is a form of cancer that can arise in the bile ducts in adulthood if choledochal cysts aren’t treated
Many researchers believe choledochal cysts form due to a defect at the junction of the bile duct and pancreatic duct that is present from birth. Though a very small number of cases are passed down from parent to child, the reason these defects form in most cases is unknown.
Choledochal cysts can sometimes be detected before a child is born by being seen in an ultrasound scan. After birth, the child’s caregiver or physician may notice a mass in the upper right abdomen that’s visible through the skin, which can be a sign of a choledochal cyst.
Doctors may use a combination of tests and imaging to confirm the diagnosis. These may include:
Abdominal ultrasound
Computerized tomography scan (CT or CAT scan)
Magnetic resonance cholangiopancreatography (MRCP), which is a diagnostic procedure that utilizes X-rays and a technique known as upper gastrointestinal endoscopy to diagnose and potentially treat issues associated with the bile ducts
Endoscopic retrograde cholangiopancreatography (ERCP), which utilizes powerful magnetic fields, radio waves and advanced computing to evaluate the bile ducts without the use of ionizing radiation
At the Hoag Digestive Health institute, we’re committed to giving every patient the understanding, care and treatment options we’d want for ourselves or a family member facing a serious illness.
Conditions involving the bile ducts, liver, gallbladder and pancreas are often hard to diagnose without a deep, nuanced understanding of each individual system and how they work together. That’s why you need a fully-integrated and experienced liver team with the tools and techniques to spot subtle signs, and the next-generation technology to make an accurate diagnosis fast.
At Hoag, some of the advanced imaging techniques we use to diagnose hepatobiliary conditions include:
Endoscopic retrograde cholangiopancreatography (ERCP), which is a diagnostic procedure that utilizes X-rays and a technique known as upper gastrointestinal endoscopy to diagnose and potentially treat issues associated with the bile ducts, including in cases of bile duct injury.
Magnetic Resonance Cholangiopancreatography (MRCP), which utilizes powerful magnetic fields, radio waves and advanced computing to evaluate the bile ducts, gallbladder, liver, pancreas and pancreatic duct for disease, without the use of ionizing radiation
Hepatobiliary Iminodiacetic Acid (HIDA) scanning, which is an advanced imaging technique which utilizes an injected radioactive tracer to diagnosis issues with the bile ducts, liver and gallbladder
FibroScan®, (AKA transient elastography), which is the first FDA-approved device in the U.S. that provides a painless, non-invasive method of testing for liver scarring without the side effects and complications of a needle biopsy.
Attenuation Imaging (ATI) for Fat Quantification, which is an imaging technique used for quantifying fat deposition in the liver in real time
Magnetic Resonance Elastography (MRE), which is used to detect stiffening of the liver caused by scarring and inflammation. This technology combines MRI imaging with low-frequency vibrations to create a visual map called an elastogram that shows the degree of stiffness of body tissues.
Magnetic Resonance Multi-Echo Dixon Vibe Liver Iron Quantification, which is a 3D imaging technique used to simultaneously assess fatty liver disease and abnormal iron levels in patients with chronic liver disease.
Magnetic Resonance Proton Density Fat Fraction (MRI-PDFF), which is an emerging imaging technique that accurately measures the amount of fat in liver tissue by correcting factors that can degrade or skew magnetic resonance signal intensity
The primary treatment for choledochal cysts is surgery to remove the cyst, allowing normal bile flow between the liver and small intestine. Minimally invasive laparoscopic surgery is often used to remove choledochal cysts, performed with small instruments and cameras passed through incisions in the body.
Without surgery, children with choledochal cysts face the risk of ongoing issues, including blockage and infection of the bile duct (AKA cholangitis), pancreatitis, jaundice and cirrhosis. If left untreated into adulthood, choledochal cysts can increase a person’s risk of developing bile duct cancer.
Choledochal cysts are believed to form due to defects in the biliary system that are present from birth. As such, there’s currently no known way to prevent a child from being born with the issues that lead to choledochal cysts. However, the condition is very rare.