Redefining Budd-Chiari syndrome: A systematic review.
OBJECTIVE
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.


CONCLUSIONS
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.
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Naomi
N
Shin
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Young H
YH
Kim
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Hao
H
Xu
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Hai-Bin
HB
Shi
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Qing-Qiao
QQ
Zhang
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Jean Paul
JP
Colon Pons
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Ducksoo
D
Kim
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Yi
Y
Xu
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Fei-Yun
FY
Wu
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Samuel
S
Han
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Byung-Boong
BB
Lee
Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Lin-Sun
LS
Li
Budd-Chiari; Hepatic vein outflow tract obstruction; Hepatic vein thrombosis; Membranous obstruction of the inferior vena cava; Obliterative hepatocavopathy