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Budd-Chiari syndrome is a disease characterized by a violation of the outflow of blood and venous congestion in the liver, due to obstruction of the veins of the organ. The disease can be primary and secondary. It is quite rare, it can have a subacute, acute and chronic course. The leading symptoms of the pathology are intense pain in the right hypochondrium, liver enlargement, vomiting and yellowing of the skin. The acute form is extremely dangerous for human life, as it can lead to coma and death.
The syndrome develops due to occlusion of the veins adjacent to the liver and responsible for its normal blood supply. The liver is an important organ for a person that performs a huge number of functions, ensuring the normal functioning of the body as a whole. In this regard, any failures in its blood supply immediately affect all life support systems, and cause general intoxication of the body.
The syndrome was named after the authors who gave the most complete description of this pathological condition. These are doctor Budd (England) and pathologist Chiari (Austria).
Gastroenterology considers Budd-Chiari syndrome as a rare pathology that occurs in one person in 100 of the population. Most often, this syndrome is diagnosed in women aged 000 to 30 years. In pediatric practice, the disease is rare, the average age of patients is 50 years.
Causes of Budd-Chiari Syndrome
The causes of Budd-Chiari syndrome are varied.
The statistics point to the following numbers:
Hematological disorders lead to the development of the disease in 18% of cases;
Malignant neoplasms contribute to venous congestion in the liver in 9% of cases;
The etiology of the development of the syndrome in 30% of cases (idiopathic disease) remains unclear.
The main cause of the pathology is considered to be congenital disorders in the development of the vessels of the liver and its structural units, as well as occlusion and obliteration of the liver veins.
In other cases, the following factors can provoke the development of pathology:
Injuries of the peritoneum and abdomen, as well as surgical interventions leading to stenosis of the hepatic veins.
Liver disease, cirrhosis is especially dangerous in this regard.
Peritonitis.
Pericarditis.
Malignant neoplasms.
Hemodynamic disorders.
vein thrombosis.
Taking certain medications.
Infections, including: syphilis, amoebiasis, tuberculosis, etc.
Pregnancy and the process of childbirth.
All these factors lead to the fact that the normal patency of the veins is disturbed, congestion is formed, which over time destroys the structural components of the liver. In addition, tissue necrosis of the organ occurs against the background of increased intrahepatic pressure. The liver tries to get more blood from small arteries, but if large veins are damaged, then other venous branches are unable to cope with the load placed on them. As a result, atrophy of the peripheral parts of the liver, an increase in its size. The vena cava is even more compressed by hypertrophied tissues of the organ, which ultimately leads to its complete obstruction.
Symptoms of Budd-Chiari Syndrome
Vessels of different calibers are capable of suffering from obstruction in this syndrome. The symptoms of Budd-Chiari syndrome, or rather, the degree of their severity, directly depends on how many veins that feed the liver are damaged. A hidden clinical picture is observed when one vein malfunctions. At the same time, the patient does not feel any disturbances in well-being, does not notice the pathological manifestations of the disease. However, if two or more veins are involved in the process, then the body begins to react quite violently to the violation.
Symptoms of the acute stage of the disease:
Sudden start.
The appearance of high-intensity pain in the right hypochondrium and in the abdomen.
The appearance of nausea, accompanied by vomiting.
The skin and sclera of the eyes moderately turn yellow.
The liver increases in size.
The legs are very swollen, the veins throughout the body swell, bulge out from under the skin. This indicates involvement in the pathological process of the vena cava.
If the mesenteric vessels are disrupted, the patient develops diarrhea, pain spreads to the entire abdominal cavity.
Renal failure with ascites and hydrothorax develops several days after the onset of the disease. The patient’s condition worsens every day, hematemesis develops, drug correction helps little. Vomiting blood most often indicates that there has been a rupture of the veins of the lower third of the esophagus.
The acute form of the disease most often ends in coma and death. But even if he survives, in the vast majority of cases, cirrhosis or hepatocellular cancer develops.
The chronic form of the disease manifests itself somewhat differently and is characterized by the following clinical picture:
The patient’s well-being is not strongly disturbed. Periodically, he experiences weakness and increased fatigue.
The liver is enlarged.
As the disease progresses, pain appears in the right hypochondrium, vomiting occurs.
The peak of the disease is cirrhosis of the liver, followed by the formation of liver failure.
According to statistics, the chronic form of the disease occurs in 80% of cases. In addition, in the medical literature there are descriptions of the fulminant form of the Budd-Chiari syndrome, when the symptoms increase rapidly and the disease progresses at a high rate. Jaundice and renal failure with ascites develop within a few days.
As for the subacute form of the disease, the patient develops ascites, blood clotting increases, and the spleen increases in size.
Diagnosis of the Budd-Chiari syndrome
Diagnosis of Budd-Chiari syndrome is carried out by a gastroenterologist. He can make an assumption based on the presence of ascites and hepatomegaly, while an increase in blood clotting is always observed.
In order to clarify his assumption, the doctor will send the patient for further examination:
A general blood test, which reveals an increase in ESR and a jump in leukocytes.
A coagulogram will indicate an increase in prothrombized time.
An increase in the activity of liver enzymes is noticeable in blood biochemistry.
Ultrasound of the liver allows you to visualize the expansion of the hepatic veins, an increase in the portal vein in size. It is also possible to detect a thrombus or vein stenosis, which interferes with the normal blood supply to the organ. Doppler ultrasound is not ruled out.
MRI, CT, radiography of the abdominal organs are instrumental diagnostic methods that allow to assess the size of the liver, the severity of diffuse and vascular changes, and also determine the cause of the development of the pathological condition.
Angiography and hepatobiopsy data are considered informative.
Treatment of Badda-Chiari syndrome
Treatment of the Budd-Chiari syndrome is possible only in a hospital setting. Therapeutic measures are primarily aimed at normalizing venous blood flow. In parallel, the symptoms of the disease are eliminated.
Medication
As for drug treatment, it can only bring temporary relief to the patient. Patients are prescribed diuretics, designed to remove excess fluid from the body. The intake of drugs aimed at improving metabolic processes in the liver is shown. Glucocorticosteroids can relieve severe pain. Each patient is recommended the introduction of antiplatelet agents and fibrinolytics, which contribute to the resorption of the thrombus, increase the rheological properties of the blood.
Surgery
The operation is aimed at normalizing the blood supply to the liver. However, if the patient has already developed liver failure, or if there is thrombosis of the hepatic veins, then the operation is contraindicated.
If such complications are not observed, then one of the following types of interventions is possible:
Anastomosis. At the same time, part of the vessels that have undergone pathological changes are replaced with artificial ones, which ensures the restoration of blood flow.
Perform shunting. With this type of surgical intervention, additional blood outflow pathways are created. So, the inferior vena cava is connected to the right atrium.
A liver transplant helps to improve the functioning of the body.
Expansion of the vena cava or its replacement with a prosthesis is performed with stenosis of the superior vena cava.
If the patient has resistant ascites and oliguria, then a lymphovenous anastomosis is indicated for him.
As for the prognosis, it is almost always unfavorable. It is influenced by the age of the patient, the presence of concomitant diseases, the presence of cirrhosis of the liver. If the patient does not receive the necessary treatment, then death occurs within 3 months to 3 years from the moment of manifestation of the disease. The syndrome is especially dangerous for its severe complications, including: severe liver failure, internal bleeding, hepatic encephalopathy. The fulminant form of the disease is very unfavorable for life. In the chronic form of the pathology, the life span of patients increases to 10 years, and with successful liver transplantation, from 10 years or more.