Imaging and interventions in Budd-Chiari syndrome

Abstract

Budd-Chiari syndrome (BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension. Clinically, two forms of disease (acute and chronic) are recognized. Mostly the patients present with ascites, hepatomegaly, and portal hypertension. In acute disease the liver is enlarged with thrombosed hepatic veins (HV) and ascites, whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava (IVC), or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC. Due to advances in radiological interventional techniques and hardware, there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation. The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.

Comparison of 3.0T MRI with 3D LAVA sequence and digital subtraction angiography for the assessment of accessory hepatic veins in Budd-Chiari syndrome

“…The angle between the axis of the IVC and AHV could provide a baseline value that determines the approach for interventional therapy. In the case of an obtuse angle, AHV angioplasty can be performed via the femoral approach, while for an acute angle, the transjugular approach might be used . Furthermore, the opening position of the AHV into the IVC is required to prevent the IVC stent from blocking the AHV outflow .…”

Budd-Chiari Syndrome

“…If restenosis occurs after stenting, it is usually due to inadequate anticoagulant therapy that has to be maintained for at least 6 months after the intervention (Mukund and Gamanagatti, 2011). In general, 5-year survival in patients treated by endovascular recanalization is 86% in patients with intermediate disease severity and 77% with severe BCS (Eapen et al, 2006).…”

“…If this is not successful or feasible, percutaneous transhepatic approach or access through the femoral vein is another option. In acute thrombosis, thrombolytic therapy alone has good results as well (Mukund and Gamanagatti, 2011).…”

“…This includes focal liver lesions (hepatocellular carcinoma, abscess, cyst, …), renal or adrenal adenocarcinoma, blunt abdominal trauma, and rarely primary sarcomas of IVC or myxoma of the right atrium (Mukund and Gamanagatti, 2011).…”

Incidence and risk factors of hepatocellular carcinoma in patients with hepatic venous outflow tract obstruction

“…28 Based on the imaging findings, HVOTO patients were categorised into: (i) obstruction of a single vein (IVC or HV) or (ii) combined obstruction of IVC and HV (single or multiple). The obstruction of the vein was either focal/membranous (<3 cm) or long segment if ≥3 cm).…”