Ultrasound Characteristics of Budd-Chiari Syndrome: A Multicenter Analysis.

Abstract

Introduction: Budd-Chiari syndrome (BCS) is a rare liver disease defined as an obstruction of the hepatic venous outflow, potentially leading to severe complications like liver failure. BCS can be classified as primary (due to endoluminal venous lesions such as thrombosis) or secondary (due to external compression or invasion of the hepatic veins or inferior vena cava; IVC). While numerous case reports illustrate imaging findings in individual patients, systematic data processing across multiple regions is lacking and establishing consistent ultrasound diagnostic criteria could significantly improve early detection and patient outcomes.

Methods: This retrospective multicenter study (2010-2020) included 118 patients from China (n = 59), Germany (n = 26), India (n = 18), and Italy (n = 15). The study used standardized ultrasound protocols, including Doppler and contrast-enhanced ultrasound, to assess hepatic venous outflow and other key findings, such as the presence of hepatic-vein thrombosis, caudate lobe hypertrophy, and collateral circulation.

Results: The cohort comprised 118 patients (65 men, 53 women; mean age 46 years). Most cases had a chronic disease course (74%), and primary BCS (n = 96, 81%), related to thrombotic alterations, predominated over secondary BCS, related to compressive masses (n = 7, 6%). Key sonographic features observed included portal vein thrombosis (14%), hepatic-vein thrombosis (70%), caudate lobe enlargement (30%), and collateral venous circulation (83%). Among the geographical differences in symptoms, thrombosis of the IVC was common in Chinese patients (33%), while the confluence area and hepatic veins were more frequently involved in European patients.

Conclusions: This study provides the first multicenter analysis of ultrasound characteristics in BCS patients from multiple regions. The findings support ultrasound as an essential tool in the standardized diagnostic process for BCS, particularly for identifying key sonographic features such as hepatic-vein thrombosis and caudate lobe enlargement. These results underscore the importance of standardized ultrasound protocols in BCS to facilitate early diagnosis and improve patient outcomes.

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