Excerpt from Budd-Chiari Syndrome

Synonyms, Key Words, and Related Terms: Budd-Chiari syndrome, hepatic vein occlusion, hepatic vein obstruction, hepatic obstruction, liver obstruction, liver disease, hepatic disease, thrombotic hepatic vein obstruction, nonthrombotic hepatic vein obstruction, non-thrombotic hepatic vein obstruction, obliterating hepatic vein endophlebitis, hepatomegaly, ascites, thrombotic diathesis, congestive hepatopathy, hepatic congestion, liver congestion, membranous webs, acute liver disease, subacute liver disease, fulminant liver disease, liver failure

Please click here to view the full topic text: Budd-Chiari Syndrome

Background

Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction to hepatic venous outflow. Budd described it in 1845, and Chiari added the first pathologic description of a liver with "obliterating endophlebitis of the hepatic veins" in 1899. Hepatomegaly, ascites, and abdominal pain characterize Budd-Chiari syndrome.

The syndrome most often occurs in patients with underlying thrombotic diathesis, including myeloproliferative disorders, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria, pregnancy, tumors, chronic inflammatory diseases, clotting disorders, and infections.

Pathophysiology

Obstruction of intrahepatic veins leads to congestive hepatopathy. This results from obstruction of large- or small-caliber veins, which leads to hepatic congestion as blood flows into, but not out of, the liver. Hepatocellular injury results from microvascular ischemia due to congestion. Portal hypertension and liver insufficiency result.

Frequency

United States

Budd-Chiari syndrome is rare, but the exact frequency is unknown.

International

Internationally, Budd-Chiari syndrome is also rare, but the exact frequency is unknown. Membranous webs are a common cause of Budd-Chiari syndrome in Asian countries.

Mortality/Morbidity

Budd-Chiari syndrome is a potentially fatal disorder, if untreated.

Race

The syndrome occurs in persons of all races.

Sex

The syndrome is equally present in both sexes. Emergent presentation is more common in women than in men.

Age

Age at presentation is usually the third or fourth decade of life, although the condition may also occur in children or elderly persons.

Please click here to view the full topic text: Budd-Chiari Syndrome

We subscribe to the
HONcode principles of the
Health On the Net Foundation

Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER