Budd–Chiari Syndrome: Symptoms, Treatment

Overview

Budd-Chiari syndrome is a rare disease
caused by the obstruction of the hepatic veins that drain the liver. Due to the
obstruction of the hepatic veins, blood cannot flow out of the liver and causes
the liver to enlarge (hepatomegaly). Sometimes the spleen may also enlarge
(splenomegaly).

Budd-Chiari syndrome is so rare that it affects only one in a million people. It can be fulminant, acute, chronic, or asymptomatic. Budd-Chiari syndrome is more likely to occur in patients whose blood is prone to clot, such as pregnant women, or people who have a tumor, a chronic inflammatory disease, an infection, or a myeloproliferative disorder. Most cases occur in people at the ages of 20-40.

Besides, Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD).

Causes

The exact cause of about 70% of all cases
is unknown currently. Others may be caused by:

Symptoms

Depending on the site and number of
affected veins, symptoms and severity are various from case to case. Some
symptoms may include:

  • Ascites
  • Pain in the upper right part of the abdomen
  • Hepatomegaly
  • Splenomegaly
  • Nausea or vomiting
  • Dilated, twisted veins in the esophagus (esophageal varices): caused by portal hypertension
  • Cirrhosis
  • Fatigue
  • Jaundice
  • Bleeding in the esophagus
  • Weight loss for no reason
  • Edema of the legs
  • Visible abdominal varicose veins near the skin’s surface
  • Impaired brain function (confusion or even coma) due to liver failure

Diagnosis

If your doctor suspects you have Budd-Chiari syndrome, your doctor will ask your medical and family history, then he will check your symptoms and order some other tests, such as:

  • Liver function tests
  • Measuring blood pressure within the liver
  • Angiography
  • MRI
  • Ultrasound
  • CT scan
  • Doppler ultrasound (to detect narrowed or blocked veins)
  • A liver biopsy

Treatment

Budd-Chiari can be treated more
successfully if it is diagnosed early. Treatment options for Budd-Chiari
include medications and surgery.

Medications

  • Anticoagulant such as heparin
  • Thrombolytic treatment: use clot-busting drugs
  • Medications for liver disease, such as ascites
  • High doses of the corticosteroid drug: prednisone
  • Diuretics

Surgery

  • Angioplasty: dilate the blood vessels to relieve the high pressure
  • Stent placement: insert a slender rod into the vein to maintain blood flow
  • Venous shunt surgery
  • Liver transplant

Please consult your doctors for your treatment.



Keywords: Budd-Chiari syndrome; liver disease.

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