Caroli
Disease is a rare inherited disorder characterized by
abnormal widening (dilatation) of the ducts that carry
bile from the liver (intrahepatic bile ducts).
It is of two types:
In most
cases, the isolated or simple form Caroli Disease,
affected individuals experience recurrent episodes of
inflammation of the bile ducts (cholangitis) and unusual
accumulation of pus (abscesses) on the liver
The second
form of Caroli Disease is associated with abnormal
formation of bands of fibrous tissue in the portal area of
the liver (congenital hepatic fibrosis). The portal area
of the liver is a groove (fissure) where the portal vein
and the hepatic artery enter the liver. The portal vein is
the blood vessel that carries blood from the stomach,
intestine, and spleen to the liver and the hepatic artery
is the blood vessel that carries blood from the aorta.
This form of Caroli Disease is also often associated with
high blood pressure of the portal vein (portal
hypertension), polycystic kidney disease, and, in severe
cases, liver failure.
Signs and Symptoms:
Recurrent
episodes of fever, chills and abdominal pain due to
cholengitis
This disease
mostly affect before the age of 30 years (80%).
Occasionally
the patients also experience multiple liver abscesses.
Caroli
disease is more prevalent in males then in females.
Diagnosis:
Ultrasound
CT scan
after biliary contrast
This disease
mostly affect before the age of 30 years (80%).
Occasionally
the patients also experience multiple liver abscesses.
Treatment:
Adequate
biliary drainage is the primary approach in the management
of caroli's disease.
Percutaneous
transhepatic cholangiogram (PTC) is effective in draining
the cysts and avoid recurrent episodes of cholangitis,
especially if patients comply with periodic flushing and
changing of drainage catheters.
Treatment of
Caroli Disease also involves surgical resection of the
liver cysts and/or removal of stones in the bile ducts.
