Caroli's disease is a very complex functional disorder of the liver, the diagnosis and treatment of which causes serious difficulties for doctors.
What is a 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 disturbed, its stagnation and the attachment of the infectious process occur. Often this disease occurs in men and it begins in childhood or adolescence. The course of the pathological process directly depends on the amount and degree of damage to the ducts inside the liver.
Forms of the disease
To date, there is no independent classification of Caroli disease in medicine. However, despite this, there are two different forms of this disease. The first form is characterized by the fact that the patency of the bile ducts is violated, followed by the formation of stones.
The second form is associated with congenital liver fibrosis. At the same time, a slight expansion of the ducts is observed, and the formation of stones is excluded, although it can sometimes be found at later stages of the course of the disease.
The enlarged bile ducts can connect with the cystic regions of the liver and at the same time in the lumen of the cyst partitions are visualized, thickening of the walls.
Causes of occurrence
Caroli's disease refers to congenital diseases arising from an autosomal recessive inheritance type. If there are close relatives with a similar pathology, then after the birth of a child, you should immediately conduct a comprehensive examination, since there is a high probability of transmission of the disease by inheritance.
In some cases, other pathological liver disorders also join this disease, which greatly complicates the treatment.
Main symptoms
Symptoms of Caroli's disease can occur at any age in children and young people. The main signs of the disease include:
- abdominal pain;
- fever;
- slight yellowness of the skin;
- enlarged liver on palpation.
Severe clinical manifestations of the disease are often associated with the course of the inflammatory process. Mostly diagnosed with Caroli disease in male children. With exacerbation, there is a sharp increase in the level of bilirubin and leukocytes in the blood.
Diagnostics
Despite the fact that the symptoms of the disease are quite pronounced, however, it is also characteristic of many other liver disorders. It is possible to accurately diagnose and determine Caroli’s disease by ultrasound and CT. Computed tomography is considered one of the most informative methods of examination, since you can quite well consider all the violations from the norm. In addition, endoscopic cholangiography may be required for diagnosis.
Functional liver tests 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 blood, all signs of cholestasis can be detected.
Since patients are at risk for cholangiocarcinoma, serological screening should also be performed for the presence of tumor markers.
Treatment features
In the presence of Caroli's disease, clinical recommendations must be followed without fail, since this pathology is progressive in nature. Treatment involves the use of broad-spectrum antibiotics, as well as the introduction of ursodeoxycholic acid to prevent stone formation.
In addition, the treatment includes:
- the 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 the treatment of bacterial cholangitis. It is very important to eliminate the purulent contents, and in this case, the bile ducts are cleansed , and antibacterial drugs are used to help remove the infection.
The operation is performed only in case of frequent exacerbations or in the case of ineffective use of conservative techniques. The volume 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 bile, the surgeon can remove one lobe of the liver. In especially severe cases, in the presence of liver failure or signs of degeneration into a malignant tumor, a liver transplant from a close relative may be recommended.
To get the best results, some doctors recommend liver transplantation 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, who were additionally diagnosed with biliary tract inflammation during transplantation, is quite low.
The prognosis of this disease is rather 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 fibrosis of the liver, resulting in Caroli's syndrome. Both of these pathologies are formed as a result of almost identical violations of the formation of bile ducts in the tissues of the liver at the level of embryonic development. The syndrome is inherited from a close relative and is manifested by abdominal pain, as well as bleeding from the dilated veins of the esophagus. In newborns, a combination of the main symptoms of congenital liver fibrosis, Caroli's disease and polycystic kidney disease can be observed.
This disease belongs to the congenital cyst of the bile ducts, however, it is quite rare and mainly in people under the age of 30 years. In the first few years, the pathology is almost asymptomatic, until the expansion of the bile ducts provokes stagnation of bile, which will create all the conditions for the formation of stones and infection. If jaundice is observed along with the remaining signs, then this indicates the presence of cholangitis.
Mostly left-sided liver damage is noted, but in some cases it can be bilateral.
In some cases, the liver damage is so severe that it can affect other organs. So one of the complications can be considered the occurrence of renal failure.