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An uncommon condition, Budd-Chiari syndrome (BCS) syndrome is caused by blood clots that narrow completely or partially block the large veins that carry blood from the liver to the heart.
Budd-Chiari occurs in people of all races and, while it’s somewhat more common in women, both sexes can become afflicted. Age of onset for the disease is usually in the thirties and forties, but it does occur in people of all ages.
Budd-chiari syndrome signs and symptoms
Symptoms of Budd-Chiari vary depending on whether they appear suddenly, or develop more gradually. Most often, symptoms develop slowly over a period of weeks or months.
Generally, BCS is characterized by liver enlargement (hepatomegaly), an abnormal collection of fluid within the abdomen (ascites), and abdominal painng. These symptoms are a result of impeded blood flow from the liver, causing blood to accumulate in the liver and enlarging it. The spleen may also become enlarged, and sometimes severe bleeding occurs in the esophagus.
- Abdominal swelling
- Ankle / leg swelling
- Elevated liver enzymes
- Impaired brain functioning (hepatic encephalopathy: behavioral, neurological, and psychological changes associated with advanced liver disease)
- Lactic acidosis (too much lactic acid)
- Liver failure (resulting from cirrhosis)
- Prominence of collateral vein
- Severe liver cell damage
- Skin ulcers
- Vomiting blood
Some people, however, develop no symptoms whatsoever.
Budd-chiari syndrome causes and risk factors
Usually, the cause of Budd-Chiari syndrome is a condition that makes it more likely for blood to clot.
- Clotting disorders
- Excess red cells (polycythemia rubra vera)
- Premature breakdown of red blood cells (paroxysmal nocturnal hemoglobinuria)
- Sickle cell disease
- Amebic abscess
- Hydatid cysts
Chronic inflammatory diseases:
- Behçet disease
- Inflammatory bowel disease
- Mixed connective-tissue disease
- Sjögren syndrome
- Systemic lupus erythematosus
Inherited thrombotic diathesis:
- Antithrombin III deficiency
- Factor V Leiden deficiency
- Protein C deficiency
- Protein S deficiency
- Adrenal carcinoma
- Hepatocellular carcinoma (liver tumor)
- Leiomyosarcoma (malignant smooth muscle tumor)
- Renal cell carcinoma
- Right atrial myxoma (non-cancerous heart tumor)
- Wilms tumor (kidney cancer)
- Alpha1-antitrypsin deficiency
- Membranous webs
- Oral contraceptives
- Parasitic infections
- Pregnancy and postpartum
- Trauma and injury
Budd-Chiari syndrome can begin suddenly and rather severely during pregnancy because at this time a woman’s blood clots more readily.
In approximately one third of BCS patients, the underlying cause is unclear.1
Budd-chiari syndrome complications
The congestion of blood in the veins of the liver causes blood pressure in the portal vein (a large vein that carries blood from the stomach and intestines to the liver) to increase.
Known as portal hypertension, this increased pressure can result in varicose veins in the esophagus as well as in the abdomen near the skin’s surface. If BCS is left untreated, bleeding from varicose veins in the esophagus can result.
Eventually, severe scarring of the liver (cirrhosis) also occurs.
Diagnosing budd-chiari syndrome
BCS may be suspected when there is an abnormal amount of fluid in the abdomen, liver enlargement and/or failure, or cirrhosis when testing shows no obvious cause.
Abnormal results of evaluative liver function tests may also indicate BCS.
Liver enzyme levels and other organ markers (creatinine, electrolytes, lactic acid dehydrogenase/LDH, and urea) are measured when BCS is suspected.
A liver biopsy may be done to confirm a BCS diagnosis and to determine whether or not cirrhosis has developed.
- Doppler ultrasonography
- Magnetic resonance imaging of blood vessels (magnetic resonance angiography)
- Computed tomography (CT/catscan)
When surgery is indicated, venography is necessary. X-rays of the veins are taken after a radiopaque dye is injected into a vein in the groin.
Budd-chiari syndrome treatment and prognosis
Treatment depends on how severe the BCS is, and how quickly the illness has developed. A small percentage of patients can be treated through symptom management: sodium restriction and diuretics to control excessive abdominal fluid build-up, and anticoagulants such as heparin and warfarin to prevent the formation of blood clots.
Most people, however, require more complex medical intervention.
Surgical shunts – used to divert blood flow around the obstruction – are an option for milder forms of BCS. For best results, shunts must be placed relatively soon after diagnosis. Shunts sometimes become blocked, especially in those prone to forming blood clots. Shunts also increase the risk of brain function deterioration due to liver dysfunction.
Another option is the transjugular intrahepatic portosystemic shunt (TIPS). Similar to a surgical shunt, this intervention has a lower procedure-related mortality rate.
Life-saving liver transplantation may be necessary for patients with severe liver failure.
Most people need to take anticoagulants indefinitely to prevent new blockages from developing, enlarging, or recurring.
If left untreated, Budd-Chiari syndrome is potentially fatal. While life expectancy varies with partial vein blockages, if a completely blocked vein is left untreated, most people die of liver failure within three years.2
The underlying cause of BCS affects survival rate. Despite treatment, patients with malignant diseases of certain bone marrow cells, for example, may development acute leukemia.
Generally, close to two thirds of BCS patients survive for ten years.3 Budd-Chiari syndrome can be difficult to live with and manage, but much research and testing is being done to help people overcome it.