UMMS Affiliation

Department of Radiology; Department of Gastroenterology; School of Medicine; Senior Scholars Program

Publication Date

6-8-2016

Document Type

Article

Disciplines

Digestive System Diseases | Hepatology | Radiology

Abstract

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.

Keywords

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

Rights and Permissions

Copyright ©The Author(s) 2016. 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

Journal/Book/Conference Title

World journal of hepatology

Comments

Naomi Shin participated in this study as a medical student in the Senior Scholars research program at the University of Massachusetts Medical School.

Related Resources

Link to Article in PubMed

PubMed ID

27326316

Creative Commons License

Creative Commons Attribution-Noncommercial 4.0 License
This work is licensed under a Creative Commons Attribution-Noncommercial 4.0 License

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