Cystic Diseases

What are cystic diseases of the liver?

One of the liver’s important functions is producing and excreting bile. This yellow-green fluid flows into the intestine through the bile ducts. The bile ducts in the liver are like the branches of a tree, that come together just below the stomach. A side branch leads to an organ storing bile, the gallbladder.

Gallbladder disease is a common type of illness involving the biliary tree. Less common is cystic disease of the biliary tree. This can take several forms:

Cysts in the main trunk (choledochal cysts)
Cysts in the small branches within the liver (called Caroli's syndrome)
Cysts in the liver separate from the biliary tree (polycystic liver disease)

What is a Cyst?

A cyst is an abnormal fluid-filled balloon-like structure (a sac). It can occur in any part of the body. Liver cysts are mostly congenital (from birth) or parasitic.

Choledochal Cysts

The main trunk of the biliary tree (the common bile duct) is structurally abnormal, probably from the time of birth. Eventually (usually by age two or three but sometimes not until adolescence or adulthood) the duct forms a cyst which may prevent the bile from reaching the intestine. Bile backs up into the liver and the patient becomes jaundiced (yellow). Occasionally this accumulation of bile becomes infected, causing abdominal pain and fever.

In some patients the cyst can be felt by the doctor examining the abdomen. In most patients the diagnosis can be confirmed by using ultrasound or by injecting a radioactive substance which gives an image of the abnormal duct (nuclear medicine). In most cases surgical treatment permanently corrects the disease. Rarely infection recurs in the newly constructed biliary tree. If the condition is not correctly diagnosed the blockage of bile may result in scarring of the liver (fibrosis), leading to cirrhosis.

Caroli's syndrome

Caroli's syndrome is another rare congenital disease. In this syndrome, the small branches of the biliary tree in the liver are abnormal. Small cysts alternate with narrowed segments of the bile ducts. These abnormalities may be present throughout the liver, or limited to only a small area. If the bile becomes infected, the patient develops fever, abdominal pain and rarely jaundice. This complication may first appear in childhood or may not occur until middle age.

Caroli’s syndrome is diagnosed by imaging the bile ducts and also by injecting dye directly into the biliary tree. This may be done by inserting a needle through the skin into the liver (percutaneous transhepatic cholangiography, or PTC) or by using a tube to pass dye through the intestine up into the bile duct (endoscopic retrograde cholangiography, or ERCP). A liver biopsy may also be taken.

Congenital Hepatic Fibrosis

In patients with this condition, there is abnormal growth of fibrous (scar) tissue around the small branches of the bile ducts in the liver. As a result, the liver becomes enlarged and hard, and blood can no longer flow freely through the liver. The spleen, situated under the rib cage to the left of the liver, becomes enlarged because of back pressure. This causes swelling of the veins along the gullet (oesophageal varices).

These may burst and cause bleeding into the stomach and bowels. People with this condition are usually discovered in childhood, either because of a large liver or because of bleeding. However in a few the diagnosis is not made until adult life. The diagnosis is by liver biopsy and x-rays of blood vessels. There is no specific treatment but many people require re-routing of blood from the intestines (shunt operation) to prevent more intestinal bleeding.

What is polycystic liver disease?

In some patients, large cysts develop from the biliary tree within the liver but do not obstruct the ducts. In severe cases, the liver looks like a sponge. These cysts may cause pain, but do not affect liver function. In most patients, the kidneys are similarly affected with cysts, which may cause high blood pressure and kidney failure. The tendency to form cysts is probably present at birth, but usually the cysts do not enlarge and give problems until adulthood. This condition may be detected using imaging methods such as ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) scan and x-rays of the kidney (intravenous pyelogram). Polycystic disease is inherited and once it has been detected in one member of a family, all the patient's relatives should be tested for it.

Simple Liver Cysts

Some cysts are commonly seen in the liver on ultrasound examination. These seldom cause symptoms. Occasionally if the cysts are large they may cause pain and some require surgical drainage. This condition is not inherited and is unrelated to polycystic liver disease in which many more cysts are present.

Hydatid Cysts

These are parasitic cysts from the dog tapeworm. They are not very common in the UK but may be contracted abroad where there has been contact with sheep dogs and other animals. They may respond to drug therapy (such as albendazole) but some may require surgical treatment.

Very occasionally benign or malignant tumours (cancers) present as cysts. Also some of the above conditions are rarely complicated by a form of cancer.

Treatment

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