Radiological Interventions in Pediatric Budd-Chiari Syndrome: Current Trends and Review of Literature

儿童布加氏综合征的放射介入治疗:当前趋势及文献综述

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Abstract

Pediatric Budd-Chiari syndrome (BCS) is a rare but serious vascular disorder of the liver characterized by obstruction of the hepatic venous outflow leading to portal hypertension and liver dysfunction. Radiological endovascular interventions have revolutionized its management by providing minimally invasive options to restore venous patency and improve clinical outcomes. Early interventions are critical to prevent irreversible hepatic damage. Comparative studies highlight that endovascular therapies have high technical and clinical success with low complication rates. This review consolidates current evidence on the role of hepatic vein and inferior vena cava angioplasty, stenting, mechanical thromboaspiration, transjugular intrahepatic portosystemic shunt (TIPS), and direct intrahepatic portosystemic shunt (DIPS) in children with BCS. Doppler ultrasonography (US) remains the primary diagnostic modality, accurately localizing venous obstructions and guiding interventions. Post-procedural anticoagulation and surveillance with Doppler US are essential for long-term optimization. Novel techniques like 2D shear wave elastography enable non-invasive assessment of liver and splenic stiffness, reflecting fibrosis regression and hemodynamic improvement over time, and are being increasingly used for response assessment. This review underscores the evolving role of radiological endovascular techniques as first-line management for pediatric BCS, drawing upon established techniques and recent advancements to optimize patient outcomes.

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