Polycystic Liver Disease (PLD)
About Polycystic Liver Disease (PLD)
Polycystic liver disease is a rare inherited disorder that creates multiple fluid-filled sacs on the liver and on the kidneys. The presence of these cysts makes the texture of the liver hard. The liver cysts can expand and create pressure on the organs surrounding the liver. A second, and more common form of PLD, in which an individual has cysts in both the liver and the kidneys, is called autosomal dominant polycystic kidney disease.
Why it is Problematic
Most individuals are unaware of the presence of cysts on their liver and will have few if any problems associated with the disease. More serious complications associated with the disease can be seen in about 10% of those affected with polycystic liver disease.
Causes and Risk Factors
Often polycystic liver disease will be asymptomatic. When symptoms are present they may include:
- Feeling full after eating very little food
- Shortness of breath
- Muscle wasting
- Leg/foot swelling (edema)
- Jaundice (especially around eyelids)
- Painful or inflamed bile ducts
- Spider angioma
- Bile duct obstruction
Because polycystic liver disease is often symptomless, people may learn of polycystic liver disease through a routine exam or diagnosis of another condition. Factors considered in diagnosing polycystic liver disease are:
A family history of PLD, in individuals under 40 years of age who have more than 1 cyst
A family history of PLD in individuals older than 40 years of age who have more than 3 cysts
No family history of PLD in individuals over 40 years of age who have more than 20 cysts.
Treatment and Prevention
Treatment for PLD will focus on slowing the growth of the cysts. This can be achieved in a number of ways:
Proton pump inhibitors. A theoretical benefit is that it diminishes secretin, a hormone that triggers cyclic AMP release. Cyclic AMP activates a liver cyst by filling it with fluid, causing it to grow, expand and enlarge.
Dietary changes and nutrition. Avoid caffeine and milk products. Eat a low or no sodium diet.
Lifestyle changes. Avoid hormones or contraceptives containing hormones
Cyst aspiration. If a cyst is infected or blocking a bile duct it may be drained. Aspiration (draining) offers only temporary relief because cysts will often refill.
Sclerotherapy- This is a process of filling the cyst with a hardening substance to prevent fluid from collecting, and is sometimes performed with aspiration. This course of treatment is used in conjunction with antibiotics for infection prevention.
Liver resection. Surgery is reserved for patients experiencing extreme symptoms or those who have not benefited from other forms of treatment. If the majority of cysts are present in one area of the liver, that area may be removed for relief. This may also be an option if only a few very large cysts are present. This treatment is unavailable if there are multiple small cysts located consistently throughout the surface of the liver. There are some complications associated with resection due to the massive size of the liver and distortion of usual anatomic landmarks.
Transplant. This treatment is rarely used and only in extreme cases.
There is nothing currently known to prevent PLD. Most people express no symptoms. Always consult your doctor with any questions or if you have symptoms of PLD.
Due to the role of the estrogen hormone in PLD, women are eight times more likely to develop PLD than men. Women often develop PLD at a relatively young age.