What Is Caroli Disease?

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  • Written By: Clara Kedrek
  • Edited By: Jessica Seminara
  • Last Modified Date: 11 August 2019
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As an inherited biliary tract disorder, Caroli disease can cause a number of unfortunate symptoms in those who develop this condition. Sufferers may experience frequent infections, blockage of the biliary tract, and impaired kidney function as a result of the disease, which causes dilation of the bile ducts located inside the liver. There is no cure for the disease, but symptoms caused by the condition can be managed by medical and surgical interventions.

Caroli disease is a rare inherited genetic disorder that causes the body’s biliary system to develop abnormally. The biliary system, which collects the bile produced by the liver and gets rid of it in the gastrointestinal tract, is comprised of a system of ducts. In the normal biliary system, small ducts in the liver combine and form larger ducts that eventually connect to the gallbladder and the small intestine. With Caroli disease, the ducts within the liver, which are typically rather small in diameter, increase in size.


Symptoms of Caroli disease develop from the dilated bile ducts located in the liver. Having enlarged ducts can lead to stagnation of the bile fluid, putting patients at increased risk for infection of the biliary tract or the liver. The stagnation of the fluid could eventually lead to obstruction of the biliary flow; this can cause increased bilirubin levels in the blood, leading to jaundice. Many patients also experience abnormalities in the function of their kidneys because they develop renal cysts, which compress the kidneys and inhibit their ability to properly filter the blood.

A diagnosis of Caroli disease is typically made on the basis of imaging studies. Radiographic techniques such as ultrasound imaging, endoscopic retrograde cholangiopancreatography (ERCP), computed tomography (CT), and magnetic resonance angiography (MRA) studies can be used to evaluate whether the disease is present. A variety of laboratory abnormalities, such as elevated bilirubin levels in the blood or elevated liver function studies, could also lend support for this diagnosis.

Treatment of Caroli disease typically relies on managing the symptoms caused by the congenital abnormalities. Infections of the liver or biliary tract can be treated with antibiotics. Many doctors give patients with this disease medications that help to dissolve different components of the bile, allowing it to flow more freely. Sometimes an ERCP can be performed as a therapeutic measure to remove biliary tract stones that have formed as a result of sluggish biliary flow.

Often Caroli disease can be confused with Caroli syndrome, which is a related but distinct congenital abnormality. Patients with Caroli syndrome have the same clinical characteristics of those suffering from Caroli disease, but additionally experience problems with the function of the liver. As a result, these patients often develop cirrhosis of the liver and require liver transplantation for survival.


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