Budd–Chiari syndrome
| Budd–Chiari syndrome | |
|---|---|
| Budd–Chiari syndrome secondary to cancer; note clot in the inferior vena cava and the metastasis in the liver | |
| Specialty | Hepatology |
| Complications | Liver failure |
| Treatment | Anticoagulant medication, Transjugular intrahepatic portosystemic shunt, Liver transplantation |
| Named after |
|
Budd–Chiari syndrome is a condition when an occlusion or obstruction in the hepatic veins prevent normal outflow of blood from the liver.
The symptoms are non-specific and vary widely, but it may present with the classical triad of abdominal pain, ascites, and liver enlargement. Untreated Budd-Chiari syndrome can result in liver failure.[1]
It is usually seen in younger adults, with the median age at diagnosis between 35 and 40 years, and it has a similar incidence in males and females.[2] It is a very rare condition, affecting one in a million adults.[3][2] The syndrome can be fulminant, acute, chronic, or asymptomatic. Subacute presentation is the most common form.
Patients with hypercoagulable disorders, polycythemia vera, and hepatocellular carcinoma are at a higher risk of having Budd-Chiari syndrome.[4]
- ^ "Budd-Chiari Syndrome - Hepatic and Biliary Disorders". Merck Manual Professional Edition. Retrieved 2025-02-14.
- ^ a b Cite error: The named reference
Garcia-Pagan NEJM 4/2023was invoked but never defined (see the help page). - ^ Rajani, Rupesh; Melin, Tor; Björnsson, Einar; Broomé, Ulrika; Sangfelt, Per; Danielsson, Åke; Gustavsson, Anders; Grip, Olof; Svensson, Hans; Lööf, Lars; Wallerstedt, Sven; Almer, Sven HC (February 2009). "Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18-year experience". Liver International. 29 (2): 253–259. doi:10.1111/j.1478-3231.2008.01838.x. PMID 18694401.
- ^ Miller, Frank H. (2010). "Case 295". The Teaching Files: Gastrointestinal. pp. 626–627. doi:10.1016/B978-1-4160-5944-8.00295-5. ISBN 978-1-4160-5944-8.
Budd-Chiari syndrome is characterized by intrahepatic venous or inferior vena cava obstruction. Budd-Chiari syndrome most commonly is due to hypercoagulable states, including pregnancy, systemic lupus erythematosus, polycythemia vera, and cancer, especially hepatocellular carcinoma.