Caroli’s disease is a rare genetic disorder that causes the bile ducts inside the liver to dilate, leading to frequent infections, blockages, and impaired kidney function. There is no cure, but symptoms can be managed with medical and surgical interventions. Diagnosis is made through imaging studies and treatment involves managing symptoms. Caroli’s disease can be confused with Caroli syndrome, which includes liver function problems and may require liver transplantation.
As an inherited biliary disease, Caroli’s disease can cause a number of unpleasant symptoms in those who develop the condition. Sufferers may experience frequent infections, blockage of the bile ducts, and impaired kidney function due to the disease, which causes the bile ducts located inside the liver to dilate. There is no cure for the disease, but the symptoms caused by the condition can be managed with 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 releases it into the gastrointestinal tract, consists of a system of ducts. In the normal biliary system, the small ducts in the liver combine and form larger ducts that eventually connect to the gallbladder and small intestine. With Caroli disease, the ducts within the liver, which are typically quite small in diameter, increase in size.
Symptoms of Caroli’s disease develop from dilated bile ducts located in the liver. Having enlarged ducts can lead to bile fluid stagnation, putting patients at greater risk for biliary tract or liver infection. Fluid stagnation could eventually lead to obstruction of bile flow; this can cause increased levels of bilirubin in the blood, resulting in jaundice. Many patients also experience abnormalities in the function of their kidneys because they develop kidney cysts, which compress the kidneys and inhibit their ability to filter blood adequately.
A diagnosis of Caroli’s disease is typically made based on imaging studies. Radiographic techniques such as ultrasound imaging, endoscopic retrograde cholangiopancreatography (ERCP), computed tomography (CT), and magnetic resonance angiography (MRA) can be used to evaluate whether the disease is present. A variety of laboratory abnormalities, such as elevated blood bilirubin levels or elevated liver function studies, could also support this diagnosis.
Treatment of Caroli disease typically relies on managing the symptoms caused by the birth defects. Liver or bile duct infections can be treated with antibiotics. Many doctors give patients with this disease medications that help dissolve different components of the bile, allowing it to flow more freely. Sometimes an ERCP can be done as a therapeutic measure to remove biliary tract stones that have formed due to slow bile flow.
Caroli disease can often be confused with Caroli syndrome, which is a related but distinct congenital anomaly. Patients with Caroli syndrome have the same clinical features as those with Caroli disease, but also have problems with liver function. As a result, these patients often develop cirrhosis of the liver and require liver transplantation to survive.
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