Caroli's Disease: Symptoms and Treatment

Caroli's disease is a very complex functional disorder of the liver, diagnosing and treating which causes serious difficulties for doctors.

What is the disease?

Caroli's disease is a rare congenital pathology of the liver caused by the expansion of the bile ducts located inside this organ. It is characterized by the fact that the normal movement of bile is disrupted, its stasis and the attachment of the infectious process. Often this disease occurs in men and it begins with childhood or adolescence. The course of the pathological process directly depends on the number and extent of damage to the ducts inside the liver.

Forms of the disease

To date, there is no separate classification of Caroli's disease in medicine. However, despite this, there are two different forms of this disease. The first form is characterized by the violation of the patency of the bile ducts, followed by the formation of stones.

The second form is associated with congenital liver fibrosis. In this case, an insignificant expansion of the ducts is observed, and the formation of stones is excluded, although it can sometimes occur at later stages of the course of the disease.

The dilated bile ducts can be connected with the cystic regions of the liver and at the same time, septa and wall thickening are visualized in the cyst lumen.

Causes

Caroli's disease refers to congenital diseases arising due to autosomal recessive inheritance. If there are close relatives with a similar pathology, then after the birth of the child immediately need to conduct a comprehensive examination, since there is a high probability of the transmission of the disease by inheritance.

In some cases, other pathological disorders of the liver also join this disease, which significantly complicates the treatment.

Main symptoms

Symptoms of Caroli's disease can manifest themselves at absolutely any age in children and young people. The main signs of the disease include:

• abdominal pain;
• fever;
• Slight icterus of the skin;
• Enlargement of the liver during palpation.

Expressed clinical manifestations of the disease are often associated with the course of the inflammatory process. Mostly, Caroli's disease is diagnosed in male children. When exacerbation there is a sharp increase in the level of bilirubin and leukocytes in the blood.

Conducting diagnostics

Despite the fact that the symptoms of the disease are quite pronounced, however, it is also characteristic of many other liver disorders. Precisely to diagnose and determine can Caroli's disease on ultrasound and in the conduct of CT. Computed tomography is considered to be one of the most informative methods of examination, since it is possible to sufficiently consider all the available violations from the norm. In addition, endoscopic cholangiography may be required to make a diagnosis.

Functional tests of the liver for a long time do not change at all, however, with the progression of the disease and a prolonged inflammatory process in the biochemical analysis of the blood, all the signs of cholestasis can be detected.

Since patients are at risk for cholangiocarcinoma, serological screenings should also be performed for the presence of cancer markers.

Features of treatment

In the presence of Caroli's disease, clinical recommendations must be observed without fail, since this pathology has a progressive nature. Treatment involves the use of broad-spectrum antibiotics, as well as the introduction of ursodeoxycholic acid to prevent the formation of stones.

In addition, the treatment includes:

• Use of analgesics;
• Litholysis of stones;
• Drainage of the bile ducts.

With the addition of cholangitis or other complications of a purulent nature, therapy is no different from treating bacterial cholangitis. It is very important to eliminate the purulent contents and in this case the bile ducts are cleaned, and antibacterial drugs that help to remove the infection are used.

The operation is performed only in the case of frequent exacerbations or when the application of conservative methods is ineffective. The scope of surgical intervention can vary significantly. Only stones or bile ducts can be removed.

In the case of a very strong expansion of the bile ducts and stagnation of the bile, the surgeon can perform the removal of one lobe of the liver. In especially severe cases, with hepatic insufficiency or signs of degeneration into a malignant tumor, liver transplantation from a close relative can be recommended.

To obtain the best results, some doctors recommend that liver transplantation be performed even in the absence of severe symptoms in the initial stages of the disease. However, often the presence of infection is a contraindication for transplantation. The survival rate of patients after transplantation with congenital fibrosis, in whom the inflammation of the biliary tract was additionally diagnosed during transplantation, is low enough.

The prognosis of this disease is quite unfavorable, since relapses can occur over several years. However, very rarely it leads to the death of the patient.

Caroli Syndrome

Caroli's disease is often combined with congenital liver fibrosis, resulting in Caroli syndrome. Both these pathologies are formed as a result of almost identical violations of the formation of the bile ducts in the liver tissues at the level of embryonic development. The syndrome is inherited from a close relative and is manifested by pain in the abdomen, as well as bleeding from the esophageal dilated veins. Newborns may have a combination of the main symptoms of congenital liver fibrosis, Caroli's disease and polycystic kidney disease.

This disease belongs to the congenital bile of the bile ducts, but it is quite rare and mostly in people under the age of 30 years. In the first few years the pathology proceeds practically asymptomatically, until the expansion of the bile ducts provokes stagnation of the bile, which will create all conditions for the formation of stones and infection. If jaundice is observed along with the remaining signs, then this indicates the presence of cholangitis.

There is mainly left-sided lesion of the liver, but in some cases it can be bilateral.

In some cases, liver damage is so severe that it can affect other organs. So one of the complications can be considered the emergence of kidney failure.