How do you treat polycystic liver disease?

How do you treat polycystic liver disease?

The only definitive treatment of PLD, used in only the most severe cases, is liver transplant [2, 8, 33]. Medication to slow down cyst growth and fluid secretion in the liver (somatostatin analogs, namely octreotide and lanreotide) is also useful in reducing liver volume [7, 10].

Does polycystic liver disease cause ascites?

Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly.

When to know if you have polycystic liver disease?

You may be diagnosed with polycystic liver disease if: You have a family member with PLD, are under the age of 40, and have more than one cyst. You have a family member with PLD, are older than 40, and have more than three cysts. You have no family members with PLD, are over the age of 40, and have more than 20 cysts.

Can a person with polycystic liver disease get a liver transplant?

Or, if you have a few large cysts, your doctor may be able to surgically remove those. However, if you have thousands of small cysts spread all over the liver, liver resection will probably not work. Liver transplantation: In the most severe cases, a liver transplant may be an option.

How is genetic testing used to treat polycystic liver disease?

The images are used for diagnosis and monitoring of cysts growth. Molecular genetic testing is available to look for mutations in the SEC63, LRP5 and PRKCSH genes and may be particularly helpful in individuals that inherited the disease from one of their parents.

Can a cyst rupture in polycystic liver disease?

Infrequently, large liver cysts may rupture, causing severe abdominal pain. (Van Aerts et al. 2017) Even with the presence of many cysts, the liver of individuals with polycystic liver disease functions normally. Changes (mutations) in three genes, PRKCSH, LRP5 and SEC63, are linked to polycystic liver disease [15, 16].

You may be diagnosed with polycystic liver disease if: You have a family member with PLD, are under the age of 40, and have more than one cyst. You have a family member with PLD, are older than 40, and have more than three cysts. You have no family members with PLD, are over the age of 40, and have more than 20 cysts.

What are the treatment options for polycystic liver disease?

If symptoms are causing you significant discomfort or are affecting your liver function, there are several treatment options available. These can include: Cyst fenestration – Also known as de-roofing, this surgical process removes the wall of the cyst. This treatment works best on large cysts located on the surface of the liver.

Can a family member have polycystic liver disease?

Because it’s most often inherited, if you or someone in your immediate family has PLD, other family members should be tested for it. Doctors can diagnose polycystic liver disease with imaging studies, such as ultrasound, CT scan, or MRI. What Are the Symptoms of Polycystic Liver Disease?

Are there any differential diagnoses for polycystic kidney disease?

Differential diagnoses include multiple liver cysts, found in association with autosomal dominant polycystic kidney disease (ADPKD; see this term), but PCLD is genetically distinct from ADPKD with liver cysts. Simple liver cysts are also a differential diagnosis.