Our team cares for each child with expertise and compassion, working closely with surgeons and other specialists to find answers, ease symptoms, and support lifelong health.
The biliary tract is part of a group of organs that help the body digest food. This system includes the liver (which makes bile), the gallbladder (which stores bile), and the bile ducts (small tubes that carry bile to the small intestine). Bile is a digestive fluid that helps the body break down fats to be absorbed into the intestines.
Sometimes, babies are born with problems in this system. These are called congenital anomalies of the biliary tract. At MedStar Health, our experienced pediatric gastroenterologists and GI specialists are skilled in diagnosing and managing these complex conditions. They understand how these anomalies can affect bile flow and cause symptoms at birth, during childhood, or even later in life.
What are congenital biliary anomalies?
A congenital anomaly is a condition that develops before birth. In the biliary tract, these conditions affect how the bile ducts, liver, or gallbladder are formed. They can lead to problems with how bile flows through the body, which may cause digestive symptoms or liver damage over time.
Some patients have cystic anomalies, which involve fluid-filled sacs or cysts. Others have structural differences, which change the shape or connection of the bile ducts or nearby organs.
Examples of congenital biliary tract conditions we diagnose and treat
- Biliary atresia: A serious condition and the most known congenital biliary abnormality where the bile ducts are missing or blocked, often diagnosed in infants
- Choledochal (biliary) cysts: Sacs filled with fluid that grow in the bile ducts and can interfere with bile flow
- Alagille syndrome: A rare genetic condition that affects the liver, heart, and other parts of the body, often causing narrow or missing bile ducts
- Progressive familial intrahepatic cholestasis (PFIC): A group of rare inherited disorders that prevent bile from flowing out of liver cells, often causing liver disease in children
- Aberrant bile ducts: Extra or misplaced bile ducts that may interfere with normal bile flow
- Abnormal pancreaticobiliary junction: When the bile duct and pancreatic duct join in an unusual way, increasing the risk of inflammation or infection
Many congenital biliary anomalies are rare, and some patients don’t know they have one until symptoms appear later in life. In infants and children, early diagnosis and treatment can help prevent serious complications.
Additionally, genetic conditions such as cystic fibrosis can affect bile flow and liver health in infants, potentially requiring treatment.
What are the symptoms of congenital anomalies of the biliary tract?
Symptoms of congenital biliary anomalies can vary depending on the type and severity of the condition. Some patients may have no symptoms for years, while others, especially infants and children, may show signs early.
Common symptoms include:
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Abdominal pain or discomfort
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Jaundice, which is a yellowing of the skin and the whites of the eyes
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Fever, which can be a sign of an infection in the bile ducts (called cholangitis)
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Vomiting
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Poor growth or weight gain problems in infants and children (often referred to as failure to thrive)
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Swelling or tenderness in the belly
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Pancreatitis (inflammation of the pancreas), which can cause severe belly pain and nausea
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Itching sensation (pruritus)
If you or your child experience any of these symptoms, it’s important to see a doctor for evaluation and treatment.
How are congenital anomalies of the biliary tract diagnosed?
If your doctor suspects a congenital biliary anomaly, they will start by reviewing you or your child’s symptoms and medical history. This helps them understand your health and any signs pointing to a bile duct or liver problem.
Your doctor may begin with lab tests, including:
- Liver enzyme tests to check how well your liver is working
- Bile acid tests to assess bile flow and liver function
- Genetic testing if a hereditary condition like Alagille syndrome or Progressive Familial Intrahepatic Cholestasis is suspected
To get a closer look, your doctor may order imaging tests such as:
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Ultrasound: A painless test that uses sound waves to create pictures of the liver, gallbladder, and bile ducts
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Magnetic Resonance Imaging (MRI): Uses magnets and radio waves to create detailed images of the biliary system
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Computed Tomography (CT) scan: A special type of X-ray that gives detailed cross-sectional images of the abdomen
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Cholangiography: A test that shows the bile ducts clearly, often done with an injection of a special dye to help see blockages or cysts. One advanced type is Endoscopic Retrograde Cholangiopancreatography (ERCP), which uses a thin tube passed through the mouth to inject dye and take X-rays.
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Percutaneous liver biopsy: A small sample of liver tissue may be taken to look for signs of disease
These tests help doctors find abnormalities, blockages, cysts, or infections in the biliary tract and understand the underlying cause.
After diagnosis, your care team will discuss the best treatment options based on you or your child’s specific condition.
How are congenital anomalies of the biliary tract treated?
Treatment for congenital biliary anomalies depends on the type and severity of the condition. Your care team will create a personalized plan to help improve you or your child’s symptoms and protect your liver and digestive health.
For many cystic anomalies, surgery is often needed to remove the cyst and restore normal bile flow. This helps prevent infections, blockages, and other complications.
Structural anomalies may require surgery to repair or improve the shape and function of the bile ducts or liver.
In some cases, ongoing medical care is needed to manage symptoms and reduce risks such as infections, gallstones, or liver damage. This may include:
- Medications to reduce inflammation or infection
- Nutritional support to maintain growth and health
- Regular monitoring with imaging and blood tests
For patients with severe liver damage, a liver transplant may be necessary.
Your dedicated team at MedStar Health’s Pancreatic and Biliary Disease Program will work closely with you and your family to guide you through diagnosis, treatment, and follow-up care with compassion and expertise.
Why choose us
At MedStar Health, we know that congenital biliary anomalies can be complex and challenging for patients and families. Our expert team provides compassionate, personalized care with a focus on the whole patient.
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Multidisciplinary care: Our team includes pediatric gastroenterologists, surgeons, interventional radiologists, and other specialists who collaborate closely to create the best treatment plan for you or your child.
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Expert pediatric surgery: Our exceptionally skilled surgeons have extensive experience safely operating on patients of all ages, from newborns to adolescents, with excellent outcomes.
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World-class transplant program: Our transplant surgeons are internationally recognized experts who perform a high volume of liver and small bowel transplants, using innovative techniques that set global standards.
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Comprehensive, lifelong support: We coordinate seamlessly with our adult transplant program and specialists to ensure smooth, continuous care as patients transition into adulthood.
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Advanced technology and techniques: We use the latest imaging and minimally invasive procedures to provide accurate diagnoses and effective treatments with less discomfort.
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Patient- and family-centered care: We listen carefully to your concerns and partner with you to develop a care plan that fits your needs, lifestyle, and long-term health goals.
Our providers
Expert gastroenterology care
Getting the care you need starts with seeing one of our gastroenterologists.
Frequently asked questions
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Is a bile duct birth defect serious?
Some congenital anomalies are mild, while others can cause pain, infections, or liver damage if not treated. Early diagnosis and care can help prevent long-term problems.
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Can these conditions be found before birth?
Most biliary anomalies are not detected during pregnancy. Symptoms usually appear in infancy, childhood, or adulthood, depending on the type and severity.
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Will my child need surgery?
Many biliary anomalies are treated with surgery, especially if cysts are present or if there’s a risk of complications. Your child’s doctor will explain the best treatment options based on their specific diagnosis.