- People with polycystic liver disease have more than 10 cysts in their liver.
- Some cases are linked to polycystic kidney disease.
- Many people with polycystic liver disease will have no symptoms and will not need any treatment.
- Women are more likely to have symptoms and need treatment.
- In rare cases the cysts can cause the liver to get very big and push on other parts of your body.
- Tests for polycystic liver disease include ultrasound, MRI and CT scans.
- Severe polycystic liver disease is treated with surgery to drain the cysts or remove part of the liver.
- In very rare cases a liver transplant might be needed.
Polycystic liver 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 a “ductal plate malformation”.
Polycystic liver disease can run in the family. It can be passed on from parents to their children even if the parents have no symptoms themselves.
Some cases are linked to a condition called polycystic kidney disease. If you have polycystic kidney disease you should be screened to see if you might also have cysts in your liver. If this finds liver cysts you can ask to see a liver specialist as well as your kidney doctors.
Most liver cysts will stay very small. But over time some of them can grow big enough to cause a problem.
The female hormone oestrogen could be involved in polycystic liver disease. Most people who develop severe polycystic liver disease are women. The condition can stabilise after the menopause, when oestrogen levels go down.
But even with a lot of cysts, your liver will carry on working normally. The liver cells around the cysts are not damaged.
Most people with polycystic liver disease will have no symptoms.
If the number or size of your cysts gets very big this can cause symptoms including:
- Difficulty bending and moving about
- Tummy pain
- Back pain
- A swollen tummy
- Feeling full very quickly or all the time
- Feeling short of breath
In rare cases, polycystic liver disease can cause your liver to become very large. This is called “hepatomegaly”. When this happens your other organs can get pushed out of the way and your tummy can get very swollen. This is more common in women before menopause.
Most cases of polycystic liver disease are found by accident when you have a scan for something else. You might not need any more tests if this scan gives enough information for a clear diagnosis, and you do not have any symptoms.
In other cases you will have more tests to find out more about your condition or to rule out other types of cyst.
The first test you will have will be an ultrasound scan. You might also have a liver blood test to rule out other liver conditions. Depending on the results of the ultrasound your doctor might also want to do an MRI or CT scan.
You can find out more about these tests in the drop down boxes below.
An ultrasound scan can build up a picture of your liver. The picture can help doctors to see the number and size of your cysts and where they are. It is a simple test and should not hurt. Some gel will be put on your tummy and a wand will use sound waves to look at your liver. You can find out more about ultrasound scans here.
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.
An MRI scan usually takes about half an hour. The scanner is a tube. You will lie on a bed that moves into the tube. The scanner can be very loud, so you will be asked to wear headphones.
A CT scan will take a series of X-rays and use a computer to put them together to make a detailed picture.
A CT scan takes about half an hour. The scanner looks like a ring doughnut. You will lie on a bed that will move in and out of the hole in the middle of the ring.
Most people with polycystic liver disease will not need any treatment. If the cysts are not causing you any problems then the safest option is to leave them alone.
If you start to have symptoms then you might need treatment. You should be referred to a specialist to discuss the different options.
Even though polycystic liver disease runs in families, everyone is different. So you may need different treatment to a close family member with the same condition. Or one family member might need a lot of treatment while another never has any.
If you do need treatment there are a range of different options. The best treatment for you will depend on:
- The size and number of cysts
- Where the cysts are
- If they are pressing on other parts of your body
- If they are infected
- If you have had treatment for liver cysts before
- Your overall liver health
Some treatments will not be available at every hospital. You can ask your doctor which treatments are best for you. If the best option is not available at your hospital you can ask to be referred to a different hospital.
Types of treatment
These are the main types of treatment for polycystic liver disease. You can find more detailed information in the drop-down boxes below.
- Stopping the contraceptive pill or other oestrogen treatments
Oestrogen is thought to be involved in polycystic liver disease. So if you are taking any medication containing oestrogen you will be asked to stop. You can find out more about this here.
If a cyst becomes infected you will need antibiotics to get rid of the infection.
- Draining cysts
If a small number of cysts get very big they might need to be drained. There are different ways to do this. It might be called Cyst aspiration and sclerotherapy, PAIR, de-roofing, or cyst fenestration.
- Surgery (resection)
In rare cases, a section of the liver containing lots of cysts can be removed.
A liver transplant can replace your liver with a healthy liver from a donor. It is very rare for someone with polycystic liver disease to have a transplant.
More information on treatments for polycystic liver disease
It is rare for liver cysts to become infected.
If this happens you will probably have symptoms of a fever (high temperature). And feel generally unwell. If you think you might have an infected liver cyst you should see a doctor as soon as possible.
Infected cysts need to be treated quickly with antibiotics.
Antibiotics for an infected liver cyst are usually given in a drip. You may have to take antibiotic tablets or medicine for a few weeks afterwards. This is to be sure that the infection has gone.
The type of antibiotic you are given will depend on your symptoms and test results.
Doctors might also suggest draining the cyst.
There is some evidence that a type of medicine called a somatostatin analogue can slow down the growth of cysts. But it can also have side effects and it is not currently a standard treatment for polycystic liver disease.
You can ask your doctor about somatostatin analogue medicines and if they might be suitable for you. You may need to be referred to someone with experience of using them or to a clinical trial.
Also called PAIR. This stands for Percutaneous Aspiration, Instillation and Reaspiration.
Aspiration sclerotherapy might be used if a cyst has got very big and is causing symptoms.
You will be given a local anaesthetic and a sedative so that you are sleepy and do not feel anything during the procedure. In some cases you might have a general anaesthetic. Talk to your doctor about which option will be best for you.
During the procedure a needle will be put through your skin and into the cyst. It will gently suck out the cyst fluid (aspiration). A liquid will then be injected into the cyst. This will cause scarring, pulling the sides of the cyst together to stop it filling up again (sclerotherapy). The sclerotherapy liquid will be given a few minutes to work and then sucked back out again (reaspiration).
De-roofing is usually only done if you have very severe symptoms. And if aspiration sclerotherapy is not suitable, or has not worked well.
Cyst de-roofing is usually done by key-hole surgery. You might also hear it called laparoscopic surgery or minimally invasive surgery.
You will have a general anaesthetic, so you will be asleep during the operation.
The surgeon will make several small holes in your abdomen (tummy). They will put a tool called a laparoscope through one of these holes. A laparoscope is a thin bendy tube with a camera and a light at the end of it. Some gas will be put into your tummy to make more space for the surgery.
The surgeon will use long thin tools to remove the “roof” of your cyst. This is the part of the cyst that is on the surface of your liver. They might also stitch the walls of the cyst together to stop it from coming back.
The medical team will then let the gas out of your tummy and stitch up the small holes.
After the operation
You may need to stay in hospital after the operation. Usually just for one or two days.
You will be encouraged to start moving about as soon as you can. This will help you to recover quicker.
You will be given long socks to wear. These will help prevent clots in your veins. You might also be given injections to prevent clots.
It is normal to feel bloated after the operation because of the gas used. It is also normal to feel some pain or discomfort. Ask for more painkillers if you need them. It is better for your recovery to get pain under control than to put up with it.
An operation to remove part of your liver is called a liver resection.
This surgery is only considered if all of these things apply:
- You have severe symptoms of polycystic liver disease.
- You have lots of cysts in just one part of your liver.
- The rest of your liver is healthy.
Your liver has an amazing ability to repair itself and even to re-grow a section that has been removed. But it can only do this if the rest of your liver is healthy.
A liver resection is a big operation. It is a good idea to ask questions before making a decision. It might help to write a list of questions to ask your doctor. They should be able to tell you about the risks. And practical things like how long you will be in hospital.
It is very rare for someone with polycystic liver disease to need a liver transplant. This is usually only done if your cysts are causing severe problems and no other treatment options will work.
If your doctor thinks you might need a transplant you will be referred to a specialist transplant centre. The specialist centre will do more tests to find out if a transplant is the right option for you. You can find out more about liver transplants, from assessment to recovery, here.
Polycystic liver disease is often found by accident. You might be told you have the condition but not given any more information. This can be worrying and confusing.
Polycystic liver disease causes no problems for most people. If you have no symptoms, the safest option is to leave the cysts alone.
Medicines with oestrogen
If you have polycystic liver disease you will probably be told to stop taking any medicines containing oestrogen. This will include:
- All types of the combined contraceptive pill
- All types of HRT (hormone replacement therapy for menopause symptoms)
- Some types of feminising hormones for gender dysphoria
Stopping these medicines can be difficult. Talk to your doctor about other options and also about the impact of stopping. This will be different for everyone. In most cases stopping the hormone treatment will be the best option, but this is not always the case. For example, if your liver cysts are small and not causing any problems, but you are experiencing severe menopause symptoms.
Severe polycystic liver disease
Very rarely polycystic liver disease can become a serious condition that can make everyday life very hard. If your liver becomes very big it can make it difficult to move around and do everyday tasks. Some people feel very tired (fatigued) and struggle to enjoy things they normaly would. You might be told to avoid playing contact sports because of the risk that a hard hit could cause a cyst to burst.
You might also get indigestion and heart burn (acid reflux) because your liver is pressing on other parts of your digestive system. If this happens it can help to have several small meals a day rather than two or three bigger meals.
If you are a young woman with a very swollen tummy you can face thoughtless comments and questions from people who assume you are pregnant.
If you are struggling, it is important to look after your mental health. We have more information about this here, and links to organisations that can offer support.
It can also help to connect with other people going through a similar experience. We have an online forum for people with all kinds of liver disease.
You can also talk to our specialist liver nurses by calling out helpline on 0800 652 7330.
Diet and polycystic liver disease
There is no evidence that specific foods make polycystic liver disease better or worse. Your liver should still work well even with the condition. You can help your liver by trying to eat a healthy, balanced diet. You can find out more about this here.
If you have severe polycystic liver disease you might find it hard to eat. This can mean you are not getting all the nutrients and energy that you need. This might not be obvious from your weight. If your liver is very big and heavy your weight will not go down as you would expect. If you are struggling to eat enough you should talk to your doctor. You may need to be referred to a registered NHS dietician for help.
There are no supplements or alternative treatments that have been shown to help with polycystic liver disease. If you have a liver condition you should always take extra care and talk to your doctor before trying these. You can find out more about complementary and alternative treatments here.
Published on 10/08/2023
Review date 10/08/2026