Systematic Reviews
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Jun 8, 2016; 8(16): 691-702
Published online Jun 8, 2016. doi: 10.4254/wjh.v8.i16.691
Redefining Budd-Chiari syndrome: A systematic review
Naomi Shin, Young H Kim, Hao Xu, Hai-Bin Shi, Qing-Qiao Zhang, Jean Paul Colon Pons, Ducksoo Kim, Yi Xu, Fei-Yun Wu, Samuel Han, Byung-Boong Lee, Lin-Sun Li
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States
Hao Xu, Qing-Qiao Zhang, Department of Interventional Radiology, the Affiliated Hospital of Xuzhou Medical College, Xuzhou 221006, Jiangsu Province, China
Hai-Bin Shi, Yi Xu, Fei-Yun Wu, Lin-Sun Li, Department of Radiology, the First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, Jiangsu Province, China
Jean Paul Colon Pons, Ducksoo Kim, Department of Radiology, Boston University School of Medicine, Boston, MA 02118, United States
Samuel Han, Department of Gastroenterology, University of Massachusetts Medical School, Worcester, MA 01655, United States
Byung-Boong Lee, Department of Surgery, George Washington University School of Medicine, Washington, DC 20037, United States
Author contributions: Shin N, Kim YH, Xu H, Shi HB, Zhang QQ and Xu Y designed the research; Shin N, Colon Pons JP, Kim D, Xu Y, Wu FY, Lee BB and Li LS conducted the research; Shin N, Kim YH, Xu H and Han S wrote the paper.
Conflict-of-interest statement: The authors declare no conflicts of interest.
Data sharing statement: Technical appendix, statistical code, and dataset available from the corresponding author at
Open-Access: 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:
Correspondence to: Young H Kim, MD, PhD, Associate Professor, Department of Radiology, University of Massachusetts Medical School, 55 Lake Avenue North, Worcester, MA 01655, United States.
Telephone: +1-508-3342087 Fax: +1-508-8564910
Received: January 13, 2016
Peer-review started: January 15, 2016
First decision: March 23, 2016
Revised: April 8, 2016
Accepted: May 17, 2016
Article in press: May 27, 2016
Published online: June 8, 2016

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 1st 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.

Keywords: Budd-Chiari, Hepatic vein outflow tract obstruction, Membranous obstruction of the inferior vena cava, Obliterative hepatocavopathy, Hepatic vein thrombosis

Core tip: With improved diagnostic methods, the terminology for Budd-Chiari syndrome (BCS) has expanded discordantly. This systematic review discusses recent population studies of BCS and proposes the delineation of two clinically unique syndromes.