What is polycystic liver disease?

Polycystic liver disease (PLD) is a condition in which 10 or more cysts grow inside the liver. The cysts are not a type of cancer. In children and young people, the cysts are usually very small.

In a small number of people the cysts grow and increase in number with age. This is why symptoms may only appear in adulthood.

Polycystic liver disease can run in the family (be inherited). But people can still have different experiences of it. Some people may become very unwell, but others will have no problems, even though they are related.

There are two forms of polycystic liver disease:

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How is polycystic liver disease diagnosed?

Polycystic liver disease is often found by accident when doctors are doing scans to look for something else.

Tests used to help with the diagnosis may include:

• abdominal ultrasound scan
• magnetic resonance imaging (MRI) scan
• blood tests
• genetic tests

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What are the symptoms of polycystic liver disease?

Most children and young people show no signs or symptoms. Even if your child has a lot of cysts in their liver, it will carry on working as normal. The liver cells around the cysts are not damaged.

Even in adulthood, most people have no symptoms from liver cysts. Symptoms only happen if the liver cysts grow larger over time.

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What are the possible complications of polycystic liver disease?

It is very unusual for complications to happen in children and young people. But in a small number, the liver cysts get larger as they age. This happens in around 1 in 20 people with PLD.

If the number, or size of cysts gets very big, it can cause symptoms. This is due to the space the cysts take up in the tummy (abdomen).

Symptoms may include:

• tummy (abdominal) pain
• tummy (abdominal) swelling
• back pain
• feeling short of breath (dyspnoea)
• feeling full early in a meal
• indigestion (dyspepsia)
• heartburn (gastro-oesophageal reflux)

In rare cases, the cysts get so big that the liver grows and pushes on other organs and blood vessels. This can lead to further complications including:

• enlarged liver (hepatomegaly)
• portal hypertension and ascites
• cyst infection
• cyst bleeding
• cyst rupture (bursting)
• yellowing of the whites of the eyes and skin (jaundice)

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How is polycystic liver disease treated?

Polycystic liver disease does not usually cause problems in children and young people. It is very rare for them to need treatment. This will only be needed if your child develops serious symptoms that cause concern.

Treatments in rare cases may include:

• antibiotics for cyst infection
• draining fluid from cysts (cyst aspiration)
• medication to slow down cyst growth
• surgery – procedures such as removing the wall from cysts (cyst fenestration), liver resection and liver transplant.

If your child is diagnosed with polycystic liver disease, they will be monitored over time. This is done to check for any changes in the size or number of cysts.

As they age, hormones may play a role in the number and size of cysts. In adulthood, women are more likely to have symptoms and need treatment. The medical team may recommend avoiding contraceptives that contain oestrogen. This is because oestrogen can increase the growth of liver cysts.

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