Department of Radiology; Department of Gastroenterology
Digestive System Diseases | Hepatology | Radiology
AIM: To re-examine whether hepatic vein thrombosis (HVT) (classical Budd-Chiari syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) are the same disorder.
METHODS: A systematic review of observational studies conducted in adult subjects with primary BCS, hepatic vein outflow tract obstruction, membranous obstruction of the inferior vena cava (IVC), obliterative hepatocavopathy, or HVT during the period of January 2000 until February 2015 was conducted using the following databases: Cochrane Library, CINAHL, MEDLINE, PubMed and Scopus.
RESULTS: Of 1299 articles identified, 26 were included in this study. Classical BCS is more common in women with a pure hepatic vein obstruction (49%-74%). HVC-BCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins (14%-84%). Classical BCS presents with acute abdominal pain, ascites, and hepatomegaly. HVC-BCS presents with chronic abdominal pain and abdominal wall varices. Myeloproliferative neoplasms (MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F mutation in 2%-5%. Classical BCS responds well to medical management alone and 1(st) line management of HVC-BCS involves percutaneous recanalization, with few managed with medical management alone.
CONCLUSION: Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.
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Citation: World J Hepatol. 2016 Jun 8;8(16):691-702. doi: 10.4254/wjh.v8.i16.691. Link to article on publisher's site
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
Budd-Chiari, Hepatic vein outflow tract obstruction, Hepatic vein thrombosis, Membranous obstruction of the inferior vena cava, Obliterative hepatocavopathy
Shin, Naomi; Kim, Young H.; Xu, Hao; Shi, Hai-Bin; Zhang, Qing-Qiao; Colon Pons, Jean Paul; Kim, Ducksoo; Xu, Yi; Wu, Fei-Yun; Han, Samuel; Lee, Byung-Boong; and Li, Lin-Sun, "Redefining Budd-Chiari syndrome: A systematic review" (2016). Radiology Publications and Presentations. 162.
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This work is licensed under a Creative Commons Attribution-Noncommercial 4.0 License