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Polycystic liver disease (PLD) is a rare genetic disorder that causes normal liver tissue to be replaced by fluid-filled liver cysts. Liver cysts are actually pretty common, but their true prevalence is unknown, as they tend to be asymptomatic, says Dr. Danielle Brandman, Medical Director of the Center for Liver Disease and Transplantation at �鶹��ý�����.

“Most patients who have liver cysts actually don’t have PLD.”

Dr. Brandman explains that PLD-associated liver cysts are often associated with polycystic kidney disease (PKD). PLD also can occur in the absence of PKD. That’s called isolated PLD.

And just to be clear, while PKD can cause cysts to develop in the liver and elsewhere in the body, isolated PLD doesn’t behave in this manner. Cysts in patients with isolated PLD are generally confined to the liver. They’re usually found by accident on a scan ordered for some other reason. Surprisingly, even patients with massive liver cysts often have normal liver function, she says, unless they have another cause of chronic liver disease, such as alcohol- associated liver disease, metabolic dysfunction steatotic liver disease (formerly known as fatty liver), viral hepatitis or some other type of liver disorder.

Symptoms are the issue

“The main issue for some patients with PLD is the presence of symptoms,” she says. “These may include feeling ‘full,’ being unable to eat or having pain related to an enlarged, cystic liver.

“When I see a patient with PLD who doesn’t have an established diagnosis of PKD, I order an abdominal MRI (if not already done) to evaluate the patient for kidney cysts. If no kidney cysts are present, or there are an inadequate number of kidney cysts to qualify for a diagnosis of PKD, the PLD is assumed to be isolated PLD. Long-term outcomes are better overall in isolated PLD, largely due to the absence of chronic kidney disease,” she explains.

Medication

The PLD patients who may benefit most from taking medication are those with widespread cysts throughout the liver and a small volume of remaining liver tissue. These patients are the most symptomatic. Injectable medications called somatostatin analogs—octreotide and lanreotide, for example—can be used to reduce liver volume, thus easing the “full” feeling and allowing patients to eat normally again.*

*Somatostatin analogs have not yet been approved by the FDA for the treatment of PLD. They are being used on an experimental basis. For details about the status of these novel drugs, you can learn more �����Ի� .

Also consider enrolling in a of octreotide at �鶹��ý�����.

Surgical treatment

PLD affects people in different ways, even within the same family, says Dr. Benjamin Samstein, Chief of Liver Transplantation and Hepatobiliary Surgery, Professor of Surgery and Surgical Director of the Living Donor Liver Transplant Program at �鶹��ý�����. “We treat PLD according to these differences. For instance, we can remove or drain a large, dominant liver cyst surgically and shrink it by more than 90 percent. But in a patient who has numerous smaller cysts, we try to tease out other management options. If we drain them, they may come back quickly. How long will the treatment last? That’s one of the most important questions we ask ourselves when making decisions about surgical treatment.”

When is a liver transplant warranted?

If you get to a point where a large liver cyst takes over and you experience severe malnutrition and muscle wasting, or where the sheer size of the liver causes fluid to leak into the abdomen, a liver transplant may be warranted, Dr. Samstein says. “Very few get to that point, but a transplant is a fix for these problems, should they occur.” Patients with PLD who meet nationally-defined criteria for liver transplant may be eligible for special status on the liver transplant waiting list that facilitates timely access to transplant.

The main takeaway message

There may be no cure for PLD—at least not yet—but there’s a wealth of available treatments that can improve patients’ quality of life for years to come. Because this disease is rare, patients should be managed by hepatologists and surgeons who have expertise in PLD. You can find a doctor here.