Budd-Chiari syndrome: Prognostic scores, special populations, and management challenges

Abstract

This editorial narrative review discussed Budd-Chiari syndrome (BCS), which represents a rare but critical vascular liver disease resulting in an obstruction of hepatic venous outflow. Despite having a unifying mechanism, the syndrome shows a large heterogeneity across presentation, cause, and disease trajectory, complicating diagnosis and management. Based on established prognostic scoring systems, the New Clichy Score, the BCS-transjugular intrahepatic portosystemic shunt Index, the Zeitoun Score, and the Pediatric End-stage Liver Disease score were examined. These scoring systems are used for risk stratification and therapeutic decision-making. Although these models deliver suitability information, their static parameters, narrow validation, and limited generalizability reduce their usefulness in diverse populations. Specific challenges are highlighted in pediatric patients, pregnant females, and individuals with myeloproliferative neoplasms for whom current tools often fall short. Moreover, there remains uncertainty regarding the durability of Pediatric End-stage Liver Disease score response and longer-term risks, such as hepatocellular carcinoma. There is a need to have a dynamic prognostic model that uses imaging and genetic factors in future studies. The article discussed enhancing recruitment to improve research. Overall, this article provided a contemporary, evidence-based approach for clinicians to aid in the evaluation and treatment of BCS.

Keywords: Budd-Chiari syndrome; Prognostic scoring system; Pediatric liver disease; Myeloproliferative neoplasms; Hepatocellular carcinoma; Transjugular intrahepatic portosystemic shunt; Advanced imaging techniques; Dynamic prognostic models

Core Tip: This narrative review provided a comprehensive update on Budd-Chiari syndrome and discussed the advantages and disadvantages of current prognostic scores. It also emphasized special conditions in children, pregnancy, and cases related to myeloproliferative neoplasms. This paper identified key research gaps and advocated for future work on personalized risk assessment, long-term outcome evaluation, and standardized response criteria to improve clinical decision-making in this heterogeneous disease.