PLD (polycystic liver disease) is an abnormal disease in which cysts (fluid-filled sacs) develop throughout the liver. A healthy liver is soft and homogeneous. The polycystic liver resembles a large bunch of grapes. Cysts also develop on their own in other areas of the liver. If the cysts become too large or enlarged, they can cause pain and health problems. However, most people with polycystic liver disease have no symptoms and lead a normal life.

Polycystic Liver Disease: What Causes It?

Although polycystic liver disease is inherited in most cases, it sometimes arises randomly without a genetic link. Women are more prone to this disease than men.

PLD occurs most frequently in patients with polycystic kidney disease (PCKD) and is more common in people older and with severe kidney disease.

Most people do not know they have PLD until they are adults, and those cysts are large enough to be detectable. Cysts range from a pinhead to more than four inches wide. Similarly, your liver may be normal or very stretched in size. Polycystic liver function is normal regardless of the number or size of cysts and the disease is usually curable.

Because it is often inherited, if you or someone in your family has PLD, other family members should be tested for it. Physicians can diagnose polycystic liver disease with imaging studies such as ultrasound, CT scan, or MRI.

Polycystic liver disease: what are the signs and symptoms?

Polycystic liver disease is in most cases characterized by a lack of symptoms. However, if the liver becomes swollen and heavy due to cystitis, the following symptoms may occur:

• Abdominal bloating or swelling
• Abdominal pain
• Looks crowded
• Shortness of breath

Only about one in ten people with PLD have complications as a result. In addition to significant abdominal discomfort, other problems can occur:

• Bleeding in the cyst is a common occurrence.
• Cyst infection is a condition in which the cyst becomes infected.
• Jaundice is caused by a blockage in the bile duct (yellowing of the skin and eyes).

What is the treatment for polycystic liver disease?

If you have no symptoms, treatment is usually not necessary. Uncomfortable medications can be used to reduce the mild pain associated with PLD. If the cysts cause significant discomfort or other problems, several treatment options are available. The severity of your discomfort, the location of the cyst, and any additional issues will determine which option is best for you. Treatment options include:

Cyst Aspiration: If a cyst is infected or obstructs the bile duct, your doctor may consider emptying it. Your doctor will use a needle or catheter to drain fluid from the cyst or cyst during cyst aspiration guided by ultrasonography or CT imaging. Cyst aspiration can, unfortunately, provide only temporary relief. Cysts are often filled with fluid. Sclerotherapy can be used to inject a hard substance such as alcohol into the cyst after aspiration and to prevent damage to the cyst wall as well as fluid retrieval. Antibiotics are given in case of infection.

Cyst Fenestration: If you have very large cysts on the surface of your liver, your doctor may also recommend cyst fenestration or de-roofing surgery to remove the cyst wall.

Liver dissection: If there are multiple cysts in one area of the liver, your doctor may surgically remove that part of the liver to reduce the discomfort and reduce the size of the liver. Alternatively, if you have some large cysts, your doctor can remove them medically. However, if you have thousands of small cysts that spread throughout your liver, liver resection is unlikely to be successful.

Liver transplant: Liver transplantation is possible in the most severe cases. This treatment is usually assigned to those who suffer from significant discomfort in the abdomen, those who have difficulty eating, and people whose general quality of life is declining. The polycystic liver disease requires the use of a liver transplant. Every year in the United States, the polycystic liver disease affects less than 100 people, requiring a liver transplant.

Is it possible to prevent polycystic liver disease?

If you have PLD, you can not do much to stop the liver cyst from growing. Researchers are now investigating whether intravenous or injection administration of lanreotide (Samutulin) and octreotide (Sandostatin) may reduce the number of liver cysts associated with PLD. So far the results have been mixed.

Do not be alarmed if you have recently suffered from polycystic liver disease. Most people with this disease, if they have certain symptoms, lead a normal life. Tell your doctor if you have pain and suffer from PLD. The doctor can advise you on the best course of action.