Caroli’s Disease – What is it, Symptoms and Treatments!

Caroli’s Disease – What it is, Symptoms and Treatments  and much more is what you’ll learn from now on, so stay with us and find out all about Caroli’s Disease , a disease that is so dangerous. In addition,  Caroli’s Disease is a congenital disease  that affects the liver . It was first described in 1958, in Paris, by the gastroenterologist Jacques Caroli.

There are two subtypes of this pathology. The first, called Caroli’s Disease , is a rare disorder that leads to multifocal segmental dilation of the intrahepatic bile ducts, without any other associated conditions. In approximately 20% of cases, only one hepatic lobe is involved, most often the left one. Apparently, Caroli’s Disease is not hereditary.

There is another subtype, called Caroli Syndrome , which is more frequent than the previous one and is characterized by dilatation of the intrahepatic bile ducts, together with congenital hepatic fibrosis.

This is a genetic disorder, usually with an autosomal recessive pattern of transmission, and is usually associated with renal pathologies, especially with polycystic  kidney disease with autosomal recessive transmission.

What is Caroli’s Disease: Caroli  ‘s Syndrome and Disease  are rare congenital disorders of the bile ducts. Both are characterized by dilatation of the intrahepatic biliary tree. The most common variant is Caroli Syndrome and is characterized by large bile duct dilatation in association with congenital hepatic fibrosis.

Caroli’s disease is limited to ectasia or segmental dilation of the greater intrahepatic ducts. This form is less common than the syndrome. Caroli Syndrome can be found in association with autosomal  recessive polycystic kidney disease with medullary cancellous kidney and medullary cystic disease.

Symptoms of Caroli’s Disease:  Symptoms of Caroli’s Disease are:

  • Acute pain  due to inflammation of the bile ducts and presence of stones;
  • Jaundice;
  • fever ;
  •  Abdominal pain ;
  • Generalized burning;
  • Liver growth .

Caroli ‘s Disease can manifest at any time in life and can affect many family members, but it is inherited in a recessive manner, which means that both the father and the mother must carry the altered gene.

Treatments for Caroli’s Disease:  The basic treatment for Caroli’s Disease  is the early diagnosis and treatment of cholangitis attacks. In addition to the risk of each infection, over time amyloidosis and liver failure can develop. If there are signs of stone obstruction, the stone must be removed. One way to prevent the appearance of this stone is the continuous use of ursodeoxycholic acid.

Another major concern is the emergence of cancer  of the bile ducts (cholangiocarcinoma), as the risk of developing this type of cancer  is 100 times greater in those who have Caroli disease than in other people.

If dilations are limited to a small area of ​​the liver , surgery can be performed to remove this area, with low morbidity (complications) and very low mortality. Another good treatment option for Caroli Disease is liver  transplantation  if there is progression to severe disease , with liver failure, after severe cholangitis (if partial resection is not possible), or when cholangiocarcinoma is suspected.

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