Budd-Chiari Syndrome (BCS) is a thrombosis of the hepatic veins and / or obstruction of the sub-diaphragmatic segment of the inferior vena cava.
BCS is a rare pathology. Most of its cases are recorded in East Asia and South Africa, where his is dominant congenital (hereditary) form. In Europe it prevails secondary formwhich may occur in connection with:
• diseases of the hematopoietic system (polycythemia, myelodysplastic syndromes, etc.),
• certain malignant neoplasms,
• systemic connective tissue diseases (antiphospholipid syndrome, AS, systemic lupus, Sjögren’s syndrome, etc.),
• certain infections (aspergillosis, hepatic amoebiasis, syphilis, tuberculosis, echinococcosis),
• cirrhosis of the liver,
• celiac disease,
• pregnancy and puerperium,
• the use of certain medications (immunosuppressants, oral contraceptives),
• radiation therapy.
BCS occurs in acute, subacute and chronic forms. The first two are manifested by abdominal pain, rapidly progressive ascites, hepatomegaly, jaundice and renal failure. It is more common chronic formwhere these symptoms increase more slowly and are less intense.
The basis of BCS diagnostics is Doppler ultrasound. Treatment depends on the form of the disease and its cause. In acute forms, liver transplantation is most often required, in chronic forms – surgical creation of a collateral circulation for obstructed blood vessels. In addition, there is treatment of the underlying disease, an appropriate diet to reduce ascites and anticoagulants. The therapy is complicated and requires the cooperation of doctors of many different specialties.
Text: SzB
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