Quick summary
- Caroli disease is very rare.
- It is usually found in young adults.
- People with Caroli syndrome also have a serious condition called congenital hepatic fibrosis.
- Symptoms can come and go in episodes.
- Treatment is usually surgery.
- If the condition is not treated, it can increase your risk of bile duct cancer.
What causes Caroli disease?
Caroli disease is part of a group of conditions called fibrocystic liver conditions. They are caused by a problem with the way your liver starts to grow before you are born. Doctors call this problem a “ductal plate malformation”. You might have more than one of these conditions, you can find out more about the others here.
Caroli disease sometimes runs in families (is inherited). But most people with Caroli disease won’t have anyone else in their family who has it. It can also be linked to another inherited condition called polycystic kidney disease.
Caroli disease causes swellings in the bile ducts inside your liver (intrahepatic bile ducts).
Bile is a liquid made in your liver. It helps you to digest fatty foods. Your bile ducts normally carry bile from your liver to your intestines (your guts).
Swellings in your bile ducts can stop bile from getting out of your liver. If the bile builds up in the swelling it can form tiny stones. Usually, these stones don’t cause a problem. But occasionally they will block your bile duct and cause symptoms.
What are the symptoms of Caroli disease?
The symptoms of Caroli disease can come and go. They can be a bit different each time. The most common symptoms of Caroli disease are:
- Jaundice (a yellow colour to your eyes or skin)
- Pain under your ribs on your right-hand side
- Fever (a high temperature)
- Vomiting
People with Caroli syndrome might also have symptoms of congenital hepatic fibrosis.
Tests for Caroli disease
The symptoms of Caroli disease are very similar to a lot of other liver conditions. These other conditions are much more common. So, you will have tests to rule them out before doctors can be sure that you have Caroli disease.
Your first tests will probably be a blood test and an ultrasound scan. These will help doctors to narrow down what is causing your symptoms. After this you might have a special type of scan called an MRCP.
You can find out more about all these tests in the drop-down boxes below:
Ultrasound scan
An ultrasound scan is quick and should not hurt. A wand will be put on your skin over your liver. The wand will use sound waves to get pictures of your liver and bile ducts. An ultrasound scan will help doctors to find out if there are cysts in your bile ducts. You can find out more about ultrasound scans here.
MRCP scan
MRCP stands for Magnetic Resonance CholangioPancreatography
It is a type of MRI scan. An MRI scan uses magnets and radio waves to build up a picture of the inside of your body. It does not use x-rays and is very good at looking at the soft parts of your body, such as your liver.
Before your MRI you will have a little tube (catheter) put into a vein on the back of your hand. You will lie on a bed that moves into the scanner. The scanner is a tube. It can be very loud, so you will be asked to wear headphones.
The MRI scanner will take some pictures. Then some dye will be injected into the tube in your hand. You will then have more pictures taken. The dye will help doctors to get a really good look at your bile ducts and any cysts that might be in them.
Treatment for Caroli disease
Because Caroli disease is very rare, you should ask to be referred to a specialist with experience of treating it.
Caroli disease can sometimes be treated with medicines to help with symptoms. But the only way to cure it is with surgery. This will not be suitable for everyone. The right treatment options for you will depend on a lot of things. Including how much of your liver is affected, and any other conditions you have.
You can read the key information about some of the treatments you might be offered in the drop down boxes below. These might not all be right for you. Ask your doctor which treatments they recommend and why. You should also ask about the risks of the treatment and if there are any other options.
You do not have to decide right away. It might help to take notes or record your appointment on your phone. Then you can think about what you have been told and write down any questions you have.
If you also have congenital hepatic fibrosis, you may need treatment for that as well. You can find out more about this here.
Ursodeoxycholic acid for gall stones
If your bile ducts are damaged by Caroli disease you can develop gallstones. These gallstones can then block your bile ducts. To stop this, you might be prescribed a medicine called ursodeoxycholic acid. This can help stop the stones from forming . But it does not always work.
Antibiotics for an infection
If a stone gets stuck in your bile ducts you can get an infection. An infection will cause a high temperature (fever) and symptoms like a bad cold or flu. If you have Caroli disease and you start to have these symptoms, you should see a doctor as soon as possible.
The infection can be treated with antibiotics. A mild infection can be treated with medicine you take at home. In more serious cases you might need to go to hospital to have antibiotics through a drip.
Surgery
Sometimes the damaged part of your liver can be removed, doctors call this a resection. Your liver has an amazing ability to re-grow. So, if the rest of your liver is healthy it should be able to get back to its normal size after a few months.
This is a big operation. You will have a general anaesthetic so that you are asleep and do not feel anything during the operation. You will probably have to stay in hospital for a few days afterwards. This is to make sure that you are recovering and have not got an infection.
If your surgery removes all your damaged bile ducts, then you should not get symptoms again.
Transplant
If your liver is too damaged for a resection you might need a liver transplant.
A liver transplant is not suitable for everyone. If your doctors think you might need a transplant you will be referred to a specialist centre. The specialists will do more tests to see if a transplant is the right option for you.
You can find a lot more information about liver transplants here.
Living with Caroli disease
Caroli disease is a rare but serious condition. If you have just been diagnosed it is normal to feel worried about what this might mean for you. Especially as there is not much information about it. But many people can be successfully treated.
If you have Caroli disease you should be treated by a specialist. If you receive benefits, you might be able to get help with travel costs. You can find out more on the NHS website.
Food, drink, and supplements
If you also have congenital hepatic fibrosis, you should not drink any alcohol.
But unless you are currently very unwell, there are no foods you have to avoid. You should try to eat a healthy balanced diet . This will help to prevent any other damage to your liver.
If your symptoms are making it difficult to eat, or you are losing a lot of weight, you should talk to your doctor. They can refer you to a registered NHS dietician You can also read our page on coping with eating difficulties .
There is no evidence that any supplements will help with Caroli disease. You should always talk to your doctor if you have a liver condition and would like to try any type of supplement or complementary medicine. Some supplements can cause liver damage. People with congenital hepatic fibrosis will be at more risk of this.
Support for you
The British Liver Trust is here to support people with any kind of liver condition. We know that it can be particularly difficult to be diagnosed with a rare condition. If you are in the UK you can speak to one of our specialist liver nurses by calling our helpline on 0800 652 7330.
You can find out about the other ways we can support you here.
Published on 10/08/2023
Review date 10/08/2026