Caroli 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 disrupted, its stagnation and the attachment of an 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 number and degree of damage to the ducts inside the liver.
Disease forms
To date, there is no independent 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 fact that the patency of the bile ducts is disturbed, with the subsequent formation of stones.
The second form is associated with congenital liver fibrosis. In this case, a slight expansion of the ducts is observed, and the formation of stones is excluded, althoughmay occasionally occur in later stages of the disease.
Dilated bile ducts can connect with cystic areas of the liver, and at the same time, septa, thickening of the walls are visualized in the lumen of the cyst.
Causes of occurrence
Caroli disease refers to congenital diseases that occur due to autosomal recessive inheritance. If there are close relatives with a similar pathology, then after the birth of a child, a comprehensive examination should be carried out immediately, since there is a high probability of transmission of the disease by inheritance.
In some cases, other pathological disorders of the liver also join this disease, which greatly complicates the treatment.
Main symptoms
Symptoms of Caroli disease can appear at any age in children and young people. The main signs of the disease include:
- abdominal pain;
- fever;
- slight jaundice of the skin;
- enlargement of the liver on palpation.
Severe clinical manifestations of the disease are often associated with the course of the inflammatory process. Mostly Caroli's disease is diagnosed in male children. With an 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 othersliver disorders. It is possible to accurately diagnose and determine Caroli's disease on ultrasound and during CT. Computed tomography is considered one of the most informative methods of examination, since it is possible to examine well enough all the existing violations from the norm. In addition, endoscopic cholangiography may be required to make a diagnosis.
Functional tests of the liver do not change at all for a long time, however, with the progression of the disease and a prolonged inflammatory process, all signs of cholestasis can be detected in the biochemical blood test.
Since patients are at risk of developing cholangiocarcinoma, serological screenings for the presence of tumor markers should also be carried out.
Features of treatment
In the presence of Caroli disease, clinical recommendations must be followed without fail, since this pathology is progressive. Treatment includes broad-spectrum antibiotics and courses of ursodeoxycholic acid to prevent stone formation.
In addition, treatment includes:
- use of analgesics;
- stone litholysis;
- drainage of bile ducts.
When joining cholangitis or other complications of a purulent nature, the 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.ducts, and antibiotics are used to help clear up the infection.
The operation is performed only in case of frequent exacerbations or when conservative methods are ineffective. The extent of surgery can vary greatly. Only stones or bile ducts can be removed.
In case of very severe dilation of the bile ducts and stagnation of bile, the surgeon may 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.
For 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 transplant patients with congenital fibrosis, who were additionally diagnosed with biliary tract inflammation at transplant, is quite low.
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 disease is often combined with congenital liver fibrosis, resulting in Caroli syndrome. Both of these pathologies are formed as a result of almost identical disorders in the formation of the bile ducts inliver tissues 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. Newborns may present with a combination of the main symptoms of congenital liver fibrosis, Caroli disease, and polycystic kidney disease.
This disease refers to a congenital cyst 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 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 other signs, then this indicates the presence of cholangitis.
Mainly left-sided liver damage is noted, 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 occurrence of kidney failure.
