What are cystic diseases of the liver?
Diagram of cyst
arising from the liver
De-roofing of liver cyst
(also called fenestration)
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,
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
Cysts in the liver separate from the biliary tree (polycystic
What is a Cyst?
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.
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
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
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.
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.
occasionally benign or malignant tumours (cancers) present as
cysts. Also some of the above conditions are rarely complicated
by a form of cancer.
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