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Ultrasound Characteristics of Budd-Chiari Syndrome: A Multicenter Analysis
Jimmy Daza1, Isabella Wiest2, Xin Wu Cui3
1Division of Hepatology, Department of Internal Medicine II, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.
Insights
Budd-Chiari syndrome (BCS) diagnosis is improved by multinational ultrasound data. Standardized protocols reveal key signs like hepatic vein thrombosis and collaterals, aiding earlier detection of this liver condition.
Area of Science:
- Hepatology
- Vascular Medicine
- Diagnostic Imaging
Background:
- Budd-Chiari syndrome (BCS) is a rare hepatic venous outflow obstruction leading to liver failure.
- It presents as primary (thrombotic) or secondary (external compression/invasion).
- Lack of cross-regional data hinders standardized diagnostic criteria for BCS.
Purpose of the Study:
- To establish reproducible ultrasound hallmarks for Budd-Chiari syndrome diagnosis.
- To explore geographic variations in BCS presentation.
- To advocate for harmonized ultrasound protocols in BCS diagnosis and classification.
Main Methods:
- Retrospective review of ultrasound examinations from 118 BCS patients (2010-2020) across China, Germany, India, and Italy.
- Utilized standardized B-mode, Doppler, and contrast-enhanced ultrasound.
- Documented venous patency, caudate lobe size, and collateral pathways.
Main Results:
- The cohort (mean age 46) predominantly had chronic BCS (74%).
- Primary thrombotic BCS (81%) was more common than secondary obstruction (6%).
- Ultrasound revealed hepatic vein thrombosis (70%), collaterals (83%), caudate hypertrophy (30%), and portal vein thrombosis (14%). Regional differences noted in IVC thrombosis and hepatic vein confluence obstruction.
Conclusions:
- Multinational ultrasound series identified reproducible BCS hallmarks: hepatic vein thrombosis, extensive collaterals, and caudate lobe enlargement.
- Geographic phenotypes of BCS were highlighted, with distinct patterns in Chinese versus European cohorts.
- Harmonized ultrasound protocols are crucial for earlier diagnosis, consistent classification, and improved outcomes in BCS.
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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