Initial imaging analysis of Budd-Chiari syndrome in Henan province of China: most cases have combined inferior vena cava and hepatic veins involvement

Abstract

Aim: To evaluate the type of venous involvement in Chinese Budd-Chiari syndrome (BCS) patients and the relative diagnostic accuracy of the different imaging modalities.

Methods: Using digital subtraction angiography (DSA) as a reference standard, color Doppler ultrasound (CDUS), computed tomography angiography (CTA), and magnetic resonance angiography (MRA) were performed on 338 patients with BCS. We analyzed the course of the main and any accessory hepatic veins (HVs) and the inferior vena cava (IVC) to assess the etiology of obstructed segments and diagnostic accuracy of CDUS, CTA and MRA.

Results: Among the 338 cases, there were 8 cases (2.4%) of isolated IVC membranous obstruction, 45 cases (13.3%) of isolated HV occlusion, and 285 cases (84.3%) with both IVC membranous obstruction and HV occlusion. Comparing with DSA, CDUS, CTA had a diagnostic accuracy of 89.3% and 80.2% in detecting BCS, and 83.4% of cases correctly correlated by MRA.

Conclusion: In Henan Province, most patients with BCS have complex lesions combining IVC and HV involvement. The combination of CDUS and CTA or MRI is useful for diagnosis of BCS and guiding therapy.

Figure 1. The preoperative color Doppler flow imaging.

CDUS shows a membranous obstruction of IVC wiht thin beam-like flow (Figure 1 a), and the right HV is completely obstructed (Figure 1 b); There is a obliteration after the confluence of left HVs without blood flows (Figure 1 c), the right HV has compensatory enlargement, with blood flow draining into IVC (Figure 1 d).

Figure 2. MRI axial and 3D reconstruction images for showing HVs and IVC.

The right HV is completely obstructed and the middle and left HVs are obstructed after their confluence (Figure 2 a). The IVC shows a membranous stenosis, and the right HV is compensatory enlarged (Figure 2 b).

Figure 3. The inferior venocavography through the femoral access.

The IVC is obstructed at the secondary porta of liver, and the adopted contrast agent is thin and beam-like (Figure 3 a), the right and rear HVs are remarkably dilated (Figure 3 b).

Figure 4. The CTA images of portal venous phase and DSA image during intervention.

The middle HV is obstructed, the right and left HVs are completely obstructed (Figure 4 a); The right, rear and inferior HVs are expanded (Figure 4 b), and the segmental IVC is obstructed (Figure 4 c); DSA image shows a segmental obstruction of the inferior caval vein, and intrahepatic collateral circulation for drainage of HV flows (Figure 4 d).