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Diseases

Genetic and Rare Diseases Information Center (GARD)

Budd-Chiari syndrome


Other Names for this Disease
  • Membranous obstruction of the inferior vena cava
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

Budd-Chiari syndrome is a rare disorder characterized by obstruction of the veins of the liver that carry the blood flow from the liver.[1][2] When the blood flow out of the liver is impeded, blood backs up in the liver, causing it to enlarge (hepatomegaly). The spleen may also enlarge (splenomegaly). This backup of blood increases blood pressure in the portal vein, which carries blood to the liver from the intestines (portal hypertension), and result in  dilated, twisted veins in the esophagus (esophageal varices). Portal hypertension, leads to fluid accumulating in the abdomen (called ascites). The clot may extend to also block the inferior vena cava (the large vein that carries blood from the lower parts of the body to the heart). Varicose veins in the abdomen near the skin’s surface may develop and become visible. In some cases,  scarring of the liver (cirrhosis) occurs. Other symptoms may include fatigue, abdominal pain, nausea, jaundice and bleeding in the esophagus.[1][3] The severity of the disorder varies from case to case, depending on the site and number of affected veins.[1] It most often occurs in patients which have a disorder that makes blood more likely to clot, such as those who are pregnant or who have a tumor, a chronic inflammatory disease, a clotting disorder, an infection, or a myeloproliferative disorder. In about one third of the cases, the cause of Budd-Chiari syndrome is unknown. Drugs or surgical interventions may be used to dissolve or decrease the size of the obstruction (if it is a clot). In some cases liver transplantation is needed.[3][2] 

Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD).[2]
Last updated: 5/2/2016

References

  1. Budd Chiari Syndrome. National Organization for Rare Disorders (NORD). 2007; http://rarediseases.org/rare-diseases/budd-chiari-syndrome/.
  2. Roy PK, Choudhary A, Shojamanesh H, Bragg J, Dehadrai G & Bashir S. Budd-Chiari Syndrome. Medscape Reference. December 16, 2015; http://emedicine.medscape.com/article/184430-overview.
  3. Shaffer EA. Budd-Chiari Syndrome. Merck Online Medical Library. 2016; http://www.merck.com/mmhe/sec10/ch138/ch138d.html.
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Basic Information

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. 
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Budd-Chiari syndrome. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Membranous obstruction of the inferior vena cava
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.