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Budd-Chiara syndrome is a rare disease of the main organ of detoxification - the liver. The reason for the development of Budd-Chiara syndrome is a violation of the venous outflow from the liver, mainly at the level of the hepatic veins and the inferior vena cava.
Symptoms characteristic of Budd-Chiara syndrome include:
A similar clinical picture can appear when the hepatic veins are compressed by regenerating nodes of a cirrhotic liver, as well as various volumetric formations in the region of the mouth of the hepatic veins.
It should be noted that the Budd-Chiara syndrome can manifest itself from accidentally discovered asymptomatic thrombosis to liver failure, which progresses rapidly and leads to death.
Budd-Chiara syndrome as a cause of acute liver failure (APN) is very rare. It accounts for 0.9–1.5% of all cases. The occurrence of OPN is the result of obstruction of the venous outflow of the liver due to thrombosis of the hepatic veins or the inferior vena cava. In many patients with Bad-Chiara syndrome, symptoms appear acutely for weeks due to partial occlusion of one or more hepatic veins, when diffuse intrahepatic ischemia with massive necrosis occurs. As a result of OPN, there is not enough time left for the development of collaterals. Thus, Budd-Chiara syndrome has a high lethality and a rapid clinical course that requires urgent treatment.
Our Center for Pancreatic Surgery and Transplantation of the Obereg Clinic has accumulated significant practical experience in identifying clinical symptoms, etiology, diagnosis and treatment of Budd-Chiara syndrome. In order to receive high-quality medical care, it is important to contact a specialized specialist in our clinic "Oberig", which is headed by a leading Ukrainian oncologist, transplant doctor Oleg Gennadyevich Kotenko - doctor of medical sciences, professor, laureate of the State Prize of Ukraine in the field of science and technology (2008 ).
Budd-Chiara syndrome is classified by etiology: primary and secondary; according to the clinical course: fulminant (hepatic encephalopathy develops within 8 weeks after the development of jaundice), acute (lasts less than 1 month, develops ascites, liver necrosis without the formation of venous collaterals), subacute (hidden onset (1–6 months), ascites, minimal necrosis of the liver, there are collaterals of the portal and hepatic veins) and chronic (lasts more than 6 months, characteristic complications of cirrhosis of the liver). The main symptoms of Budd-Chiara syndrome are a significant increase in the liver (hepatomegaly), an increase in the abdomen due to the accumulation of fluid (ascites), and abdominal pain.
Primary Budd-Chiara syndrome occurs with obstruction due to a predominantly venous process (thrombosis or phlebitis), while secondary Budd-Chiara syndrome occurs with compression or invasion of the hepatic veins and/or the inferior vena cava by a lesion of an external vein (for example, a malignant pustule).
Types of Budd-Chiara syndrome according to the level of obstruction:
I type: obstruction of the inferior vena cava with or without secondary occlusion of the hepatic vein;
II type: obstruction of the main hepatic veins;
Type III: obstruction of small centrilobular venules.
Among the most common causes of Budd-Chiara syndrome are hypercoagulation conditions:
congenital - deficiency of antithrombin III, deficiency of protein C or S, mutation of factor Leiden;
acquired - myeloproliferative diseases, antiphospholipid syndrome, tumors, taking contraceptives.
Budd-Chiara syndrome usually occurs when a blood clot narrows or blocks the hepatic veins that carry blood from the liver. Since the outflow of blood from the liver is difficult, blood accumulates in the liver, which leads to its increase. The spleen may also enlarge. This reserve flow of blood (stasis) causes an increase in blood pressure in the portal vein (brings blood to the liver from the intestines). This increased pressure, called portal hypertension, can lead to dilated, twisted varicose veins in the esophagus (esophageal varices). As a result of portal hypertension, fluid accumulates in the abdominal cavity (ascites). A blood clot can also block the inferior vena cava (carries blood from the lower parts of the body, including the liver, to the heart). As a result, the function of the kidneys is impaired, which contributes to the increase in ascites due to water retention and scarring of the liver (cirrhosis).
Rarely, the cause of Budd-Chiara syndrome is tumor invasion (hepatocellular carcinoma, kidney or adrenal gland cancer), as well as abdominal trauma, aspergillosis.
The prognosis for life with Budd-Chiara syndrome is generally positive, provided timely diagnosis and the provision of qualified assistance in specialized centers for surgery of liver diseases. The level of survival depends on the etiology and the presence of risk factors, the degree of biochemical disturbances (levels of sodium and creatinine in blood plasma), as well as the choice of treatment method. Without therapy, 90% of patients die within 3 years, mainly due to complications of cirrhosis of the liver, increasing liver failure, and hepatic encephalopathy.
The Center for Pancreatic Surgery and Transplantation of the "Oberig" Clinic specializes in providing assistance to patients with complex liver pathology, and the excellent and coordinated work of the team of specialists led by the leading Ukrainian transplant surgeon Oleg Gennadyevich Kotenko, in particular, to patients with Budd-Chiara syndrome.
To book a consultation, call: 044 521 30 03
Or sign up on the website via the link - https://my.oberig.ua/ru/appointment/
Contact us, we operate on the most difficult cases and we can fight the disease together!
