Budd-Chiari Syndrome

Budd-Chiari Syndrome: Overview

Budd-Chiari syndrome is a condition in which veins that drain the liver are narrowed or blocked, preventing blood from flowing out of the liver.

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Blockage may be partial or complete, and may occur in small or large veins.

Incidence; Causes and Development

Only about 1 in a million people are affected.

There are many possible causes of Budd-Chiari syndrome, mainly related to underlying conditions that increase the tendency of the blood to clot.  In more than 80% of cases the cause can be identified.  Causes include:

Signs and Symptoms

Not all Budd-Chiari syndrome patients experience symptoms, which usually develop gradually over weeks or months.  Blockages occur suddenly in about 20% of cases.  Symptoms include:

Diagnosis and Tests

A patient presenting with abdominal pain, ascites, and liver enlargement will often be suspected of having Budd-Chiari syndrome.  Blood tests will often be abnormal, indicating elevated liver enzymes and liver disease.  Doctors may initially mistake Budd-Chiari syndrome for cirrhosis.

Physical examination may find that the liver is enlarged; further testing may include:

  • Liver function tests
  • Ultrasound (to show liver enlargement, an irregular pattern of veins in the liver, or other abnormalities)
  • Doppler ultrasound detects narrowed or blocked veins
  • CT scan
  • MRI
  • Liver biopsy, to confirm diagnosis and see if cirrhosis is present
  • Measuring blood pressure within the liver

Findings may include liver cell death and severe lactic acidosis.

Treatment and Prevention

In some cases, treatment for Budd-Chiari syndrome is as simple as breaking up blood clots using anti-clotting medication, as well as diuretics and restricting sodium intake.  Most patients, however, require surgery to unblock the veins inside the liver.  A shunt may be inserted, to bypass a clotted vein.  Angioplasty may be used to clear or widen a vein.  For those with liver disease, a liver transplant is usually the best option.

Prior to any surgery, a venography is required.  This involves X-rays of the veins, taken after a dye that is visible in X-rays is injected.

A low-salt diet, along with diuretics, helps to prevent fluid from accumulating in the abdomen.

After treatment, most patients continue taking anticoagulants indefinitely in order to prevent recurrence.

Prognosis; Complications; Seek medical attention if...

Prognosis depends on patient age, the severity of the blockage, the underlying cause of the blockage, time to diagnosis, and the treatment.  Untreated, most patients die of liver failure within 3 years.  If treated, two-thirds of patients survive for more than 10 years after diagnosis.

Budd-Chiari syndrome, if left untreated, will lead liver damage (cirrhosis) ranging from mild to severe, as well as possibly encephalopathy.

Vomiting blood is a medical emergency.

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Budd-Chiari Syndrome:

Signs, symptoms & indicators of Budd-Chiari Syndrome:

Symptoms - Abdomen

Moderate/severe/significant right hypochondriac pain or mild right hypochondriac discomfort

Pain in the upper right abdomen, along with ascites and liver enlargement, is one of the main signs of Budd-Chiari syndrome.

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