Budd–Chiari syndrome is a very rare condition, affecting one in a million adults .The condition is caused by occlusion of the hepatic veins that drain the liver. It presents with the classical triad of abdominal pain, ascites, and liver enlargement. The formation of a blood clot within the hepatic veins can lead to Budd–Chiari syndrome. The syndrome can be fulminant, acute, chronic, or asymptomatic.
The cause can be found in more than 80% of patients
1. Primary Budd–Chiari syndrome (75%): thrombosis of the hepatic vein
Hepatic vein thrombosis is associated with the following in decreasing order of frequency:
• Polycythaemia Rubra Vera
• Postpartum state
• Use of oral contraceptives
• Paroxysmal nocturnal hemoglobinuria
• Hepatocellular carcinoma
• Lupus anticoagulants
2. Secondary Budd–Chiari syndrome (25%):
• compression of the hepatic vein by an outside structure (e.g. a tumor)
• Budd–Chiari syndrome is also seen in tuberculosis, congenital venous webs and occasionally in inferior vena caval stenosis.
• Budd–Chiari syndrome can also be the first symptom of such a tendency. Examples of genetic tendencies include protein C deficiency, protein S deficiency, the Factor V Leiden mutation, hereditary anti-thrombin deficiency and prothrombin mutation G20210A. An important non-genetic risk factor is the use of estrogen-containing (combined) forms of hormonal contraception.
• Other risk factors include the antiphospholipid syndrome, aspergillosis, Behçet’s disease, dacarbazine, pregnancy, and trauma.
Following signs and symptoms may found:
• severe upper abdominal pain,
• yellow discoloration of the skin and whites of the eyes,
• liver enlargement, enlargement of the spleen,
• fluid accumulation within the peritoneal cavity,
• elevated liver enzymes, and eventually encephalopathy.
• The fulminant syndrome presents early with encephalopathy and ascites.
• Liver cell death and severe lactic acidosis may be present as well
• A system of venous collaterals may form around the occlusion which may be seen on imaging as a “spider’s web”. cirrhosis and show the signs of liver failure.
The treatment of Budd-Chiari syndrome varies, depending on the cause of the blockage. Medical treatments may include:
• Blood-thinning (anticoagulation) medications
• Clot-busting drugs (thrombolytic treatment)
• Treatment for the liver disease, including ascites
Surgical treatments may also be considered and include.
• Angioplasty and stent placement
• Trans jugular intrahepatic portosystemic shunt (TIPS)
• Venous shunt surgery
By – Nursing Tutor – Ms. Anjali Mall
Department of Nursing
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Uttaranchal (P.G.) College Of Bio-Medical Sciences & Hospital
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