Redefining Budd-Chiari Syndrome: A Systematic Review

Overview

Journal World J Hepatol

Publisher Baishideng Publishing Group

Specialty Gastroenterology

Date 2016 Jun 22

PMID 27326316

Citations 16

Authors

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

Affiliations

Soon will be listed here.

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.

Citing Articles

Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: A Single-Centre Experience.

Joueidi F, Alhanaee A, Alsuhaibani H, Albenmousa A, Joueidi A, Elhassan A J Clin Med. 2024; 13(19).

PMID: 39407918 PMC: 11478255. DOI: 10.3390/jcm13195858.

Budd-Chiari syndrome in children: Challenges and outcome.

Samanta A, Sen Sarma M, Yadav R World J Hepatol. 2023; 15(11):1174-1187.

PMID: 38075006 PMC: 10698347. DOI: 10.4254/wjh.v15.i11.1174.

Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature.

Gaman M, Cozma M, Manan M, Srichawla B, Dhali A, Ali S World J Clin Oncol. 2023; 14(3):99-116.

PMID: 37009527 PMC: 10052333. DOI: 10.5306/wjco.v14.i3.99.

Study of Morphological Changes in Rat Liver Caused by Occlusion of Inferior Vena Cava.

Tanabayeva S, Almabayev Y, Kamyspaev M, Kulmanbetov R, Kopbayeva M, Akhmad N J Clin Exp Hepatol. 2022; 12(6):1451-1462.

PMID: 36340320 PMC: 9630039. DOI: 10.1016/j.jceh.2022.06.001.

Percutaneous transluminal angioplasty for symptomatic hepatic vein-type Budd-Chiari syndrome: feasibility and long-term outcomes.

Elkilany A, Alwarraky M, Denecke T, Geisel D Sci Rep. 2022; 12(1):14095.

PMID: 35982064 PMC: 9388522. DOI: 10.1038/s41598-022-16818-8.

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