Polycystic liver disease: symptoms, tests and treatment

A liver cyst is a fluid-filled sac in the liver. Polycystic liver disease (PLD) is the term used when there are many of them, defined by Liver UK as 10 or more. It belongs to a group called fibrocystic liver conditions, which come from a problem in the way the liver begins to develop before birth, termed a ductal plate malformation.

The condition can be inherited and can be passed from parents to children even when the parents have no symptoms. Liver UK stresses that everyone has a different experience: some people become very unwell, while others never have any problems, even within the same family.

Liver UK adds that most liver cysts stay very small, but over time some can grow large enough to cause a problem. Kidney Research UK says cystic liver disease is a frequent finding alongside autosomal dominant polycystic kidney disease (ADPKD), and that cysts tend to develop in the kidneys and liver in late childhood or early adulthood.

Most people have no symptoms. When the number or size of cysts becomes very large, Liver UK lists the following. In rare cases the liver can become very large (hepatomegaly), pushing other organs out of the way and causing a very swollen tummy, which is more common in women before the menopause.

• Difficulty bending and moving about.

• Tummy pain and back pain.

• A swollen tummy.

• Feeling full very quickly, or all the time.

• Feeling short of breath.

Liver UK says that it is rare for a liver cyst to become infected, but if it does you will probably have a fever and feel generally unwell, and you should see a doctor as soon as possible because infected cysts need to be treated quickly with antibiotics. In practice, a high temperature with new tummy pain in someone known to have liver cysts should prompt a call to NHS 111 or an urgent GP appointment. Severe pain, vomiting blood or any sudden collapse is a 999 situation.

Liver UK says that most cases are found by accident during a scan for another reason. If that scan gives enough information and you have no symptoms, you may not need more tests. Otherwise, the first test is an ultrasound scan, which shows the number, size and position of cysts, often with a liver blood test to rule out other liver conditions. An MRI or CT scan may be added depending on the result.

If you have polycystic kidney disease, Liver UK says you should be screened for liver cysts and, if cysts are found, you can ask to see a liver specialist as well as your kidney doctors.

Liver UK says that most people will not need treatment. If symptoms develop, you should be referred to a specialist. The choice depends on the size, number and position of cysts, whether they press on other parts of the body or are infected, previous treatment and overall liver health.

The main options described are stopping the contraceptive pill or other oestrogen treatments, because oestrogen is thought to be involved; antibiotics for an infected cyst; draining cysts by aspiration and sclerotherapy (a needle through the skin removes the fluid and a liquid causes scarring so it does not refill); keyhole surgery to remove the roof of the cyst (de-roofing) for very severe symptoms; liver resection when cysts are concentrated in one part and the rest of the liver is healthy; and, very rarely, liver transplant.

It also mentions that somatostatin analogue medicines may slow cyst growth in some people, but they can have side effects and are not currently a standard treatment, so they may be available mainly through specialist centres or clinical trials. Some treatments are not available at every hospital, and you can ask to be referred elsewhere.

Being told you have liver cysts without further explanation can be worrying. Liver UK stresses that PLD causes no problems for most people, and that if you have no symptoms the safest option is to leave the cysts alone. It also says that the condition can stabilise after the menopause when oestrogen levels fall.

In the UK most care is provided by the NHS, with liver (hepatology) or kidney (nephrology) specialists depending on which organ is more affected. Private care follows the same general pathway for those who choose it. If you have inherited polycystic kidney disease, relatives may be offered screening, which a genetics service can explain.