Sclerosing cholangitis is a condition where inflammation of the bile ducts inside and outside the liver eventually leads to scarring and narrowing of the ducts. When this happens the ducts are unable to carry bile (liquid that breaks down fat) away from the liver and to the small intestine. Bile builds up in the liver, causing cirrhosis or liver failure.
The cause of sclerosing cholangitis is unknown, though the condition is associated with Crohn's disease and ulcerative colitis. Genetic factors may also be responsible. Sclerosing cholangitis is rare in children and occurs more often in males than females.
Symptoms of sclerosing cholangitis include:
- Enlarged liver
- Enlarged spleen
- General discomfort, uneasiness or ill feeling
- Indigestion
- Itching
- Jaundice (yellowing of the skin and whites of the eyes)
- Loss of appetite
Diagnosis of Sclerosing Cholangitis
If your child experiences symptoms of sclerosing cholangitis, a pediatric gastroenterologist can perform the following exams and tests to help make a diagnosis:
- Magnetic resonance imaging (MRI). Your child's doctor can perform a magnetic resonance cholangiopancreatography (MRCP) to obtain pictures of the bile ducts inside and outside the liver.
- Ultrasound. Your child's doctor may perform an abdominal ultrasound to view the liver and surrounding ducts.
- Blood test. The physician may order a chemistry panel to determine how the liver is functioning.
- Endoscopic retrograde cholangiopancreatography. An endoscopic retrograde cholangiopancreatography (ERCP) procedure can give gastroenterologists advanced views of hard-to-reach areas of the small intestine and the bile and pancreatic ducts.
- Liver biopsy. Your child’s doctor may perform a liver biopsy to obtain a tissue sample from the liver. A close examination of these cells and tissues can provide more information about what is causing symptoms.
Treatments
Treatments
Treatments for sclerosing cholangitis include:
- Antibiotics. Antibiotics are used to cure infections caused by narrowed or blocked bile ducts.
- Biliary intervention. A biliary intervention is an interventional radiology procedure that is used to drain bile and open up narrowed or blocked bile ducts.
- Medicine. Ursodiol is a bile acid that increases bile flow. This medicine is also used to dissolve gallstones.
- Liver transplant. If your child has advanced sclerosing cholangitis, he or she may need a liver transplant.
Key Points to Remember
Key Points to Remember
- Sclerosing cholangitis is inflammation of the bile ducts inside and outside the liver.
- The inflammation leads to scarring and narrowing of the ducts which blocks bile flow.
- Cirrhosis and liver failure can result.
- Treatments include antibiotics and opening up blocked bile ducts.
Support Services & Resources
Support Services & Resources
Visit the websites below to find support groups and services and learn more about sclerosing cholangitis.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
This website offers information about sclerosing cholangitis, including its symptoms, causes and treatments.
This National Institutes of Health website provides information for patients and families living with sclerosing cholangitis.
Locations
Locations
Locations
In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.
Riley Pediatric Gastroenterology, Hepatology & Nutrition
575 Riley Hospital Dr
Indianapolis, IN 46202