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Budd-Chiari Syndrome

(Budd-Chiari's syndrome)

by Nicholas T. Orfanidis, MD

Budd-Chiari syndrome is caused by blood clots that completely or partially block blood flow from the liver. The blockage may occur anywhere from the small and large veins that carry blood from the liver (hepatic veins) to the inferior vena cava.

  • Some people have no symptoms, but others experience fatigue, abdominal pain, nausea, and jaundice.

  • Fluid may accumulate in the abdomen, the spleen may enlarge, and sometimes severe bleeding occurs in the esophagus.

  • Doppler ultrasonography can detect narrowed or blocked veins.

  • Drugs may be used to dissolve or decrease the size of the clot, or a connection may be made between veins to allow blood to bypass the liver.

Budd-Chiari syndrome usually occurs when a clot narrows or blocks the hepatic veins, which carry blood out of the liver.

Because blood flow out of the liver is impeded, blood backs up in the liver, causing it to enlarge. The spleen may also enlarge. This backup of blood (congestion) causes blood pressure in the portal vein (which carries blood to the liver from the intestines) to increase. This increased pressure, called portal hypertension (see Portal Hypertension), can result in dilated, twisted (varicose) veins in the esophagus (esophageal varices). Portal hypertension, plus the engorged and damaged liver, leads to fluid accumulating in the abdomen (called ascites—see Ascites). The kidneys contribute to ascites by causing salt and water to be retained.

The clot may extend to also block the inferior vena cava (the large vein that carries blood from the lower parts of the body, including the liver, to the heart). Varicose veins in the abdomen near the skin’s surface may develop and become visible.

Eventually, severe scarring of the liver (cirrhosis) occurs.

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