State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review

Abstract

Background Budd-Chiari syndrome (BCS) is an eponym that includes a group of conditions characterized by partial or complete hepatic venous tract outflow obstruction, and the site of obstruction may involve one or more hepatic veins, inferior vena cava, or the right atrium. The classification of BCS is based on etiology, site of obstruction, and duration. Its etiology is very heterogeneous; in particular, hepatic vein thrombosis is the most common type of obstruction and myeloproliferative disorder, the most common thrombophilic disorder, in the West. In Asian countries, the type of obstruction, thrombophilic disorders, clinical features, and treatment strategies vary widely from region to region. Although the cause can be identified in 90% of patients with the help of gene mutation testing, BCS remains under-recognized in many countries. A higher prevalence of acute cases has been reported in the West than in the East. This global and regional heterogeneity raises several challenges regarding the evaluation, management strategy, and individualized approach of BCS. This study aimed to conduct a systematic review of BCS to elucidate treatment strategy options. Methods PubMed, Embase, Cochrane Library, and Google Scholar databases were searched systematically. Results Sixty-nine pertinent articles were retrieved and included in the present study. Conclusions Further research on the following three topics would help define individualized treatment strategies. The first is a better understanding of the molecular pathways underlying the thrombophilic conditions implicated in the pathogenesis of BCS. The second is the role of the genotype and gene mutations in the determination of coagulation status of patients with BCS. The third is the definition of clear criteria and development of a common prognostic index to risk stratify the patients at presentation and consequently detect candidates for invasive therapies.

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

“…Patients with fulminant disease have a hyperacute onset of disease pathology (≤ 2 month), which is manifested as acute hepatic failure with ascites, hyperbilirubinemia, tender hepatomegaly, and renal failure secondary to renal outflow compromise resulting from hepatic vein obstruction[ 8 , 53 ]. Particularly, the development of hepatic encephalopathy within 2 months of onset of jaundice is regarded as fulminant disease[ 54 ]. Fulminant disease requires acute obstruction of all three hepatic veins and so its recorded incidence is quite low [ 55 ].…”

“…Fulminant disease requires acute obstruction of all three hepatic veins and so its recorded incidence is quite low [ 55 ]. Acute BCS has a short duration of onset which is usually within a month while the onset of subacute ranges from one to six months[ 54 ]. Interestingly, there is data to suggest geographical variation in the incidence of various types of BCS based on onset of pathology.…”

“…Interestingly, there is data to suggest geographical variation in the incidence of various types of BCS based on onset of pathology. In the eastern geographic regions, chronic presentations are more prevalent with onset ranging from 6 months to 30 years[ 54 , 56 ]. In the western geographic region, acute presentation is encountered relatively more frequently[ 56 ].…”

Síndrome de Budd-Chiari: abordaje y manejo

“…Clínicamente, este síndrome tiene una presentación muy variada, que puede ir desde una forma muy severa o ser asintomática (6)(7)(8). Dicha variación depende de la severidad de la obstrucción y vasos afectados; por ejemplo, una evolución fulminante ocurre cuando hay presencia de una obstrucción a nivel de las tres venas hepáticas; afortunadamente, esta presentación es poco frecuente (6). Los signos y síntomas más frecuentemente documentados son hepatomegalia, ascitis, ictericia, dolor abdominal y dilatación venosa a nivel de pared abdominal superficial (6-9).…”

“…Se puede observar un aumento en las transaminasas alcanzando niveles 5 a 10 veces superiores al límite normal con niveles variables de bilirrubina (7). En caso de presentar ascitis y obtener una muestra del líquido ascítico, es posible obtener altas concentraciones de proteínas en dicha muestra (6,8).…”