Caroli syndrome is a rare and inherited disease that affects the liver, which received its name because it was the French doctor Jacques Caroli who discovered it in 1958. It is a disease characterized by the dilation of the channels that carry bile, causing pain due to inflammation those same channels. It can produce cysts and infection, in addition to being associated with congenital liver fibrosis, which is an even more serious form of the disease.
Symptoms of Caroli Syndrome
This syndrome can remain without manifesting any symptoms for more than 20 years, but when they start to appear, they can be:
- Pain in the right side of the abdomen; Fever; Generalized burning; Liver growth; Yellow skin and eyes.
The disease can manifest at any time in life and can affect several members of the family, but it is inherited recessively, which means that both the father and the mother must carry the altered gene for the child to be born with this syndrome, which is why it is very rare.
The diagnosis can be made by performing tests that show saccular dilations of the intrahepatic bile ducts, such as abdominal ultrasound, computed tomography, endoscopic retrograde cholangiopancreatography and percutaneous transchopaneous cholangiography.
Treatment for Caroli Syndrome
Treatment involves taking antibiotics, surgery to remove the cysts if the disease affects only one lobe of the liver, and liver transplantation may be necessary in some cases. Normally, a person needs to be followed by doctors for life after diagnosis.
To improve the person's quality of life, it is recommended to be followed by a nutritionist in order to adapt the diet, avoiding the consumption of foods that require a lot of energy from the liver, which are rich in toxins and rich in fat.
