Hepatic venous outflow obstruction: three similar syndromes

Abstract

Our goal is to provide a detailed review of veno-occlusive disease (VOD), Budd-Chiari syndrome (BCS), and congestive hepatopathy (CH), all of which results in hepatic venous outflow obstruction. This is the first article in which all three syndromes have been reviewed, enabling the reader to compare the characteristics of these disorders. The histological findings in VOD, BCS, and CH are almost identical: sinusoidal congestion and cell necrosis mostly in perivenular areas of hepatic acini which eventually leads to bridging fibrosis between adjacent central veins. Tender hepatomegaly with jaundice and ascites is common to all three conditions. However, the clinical presentation depends mostly on the extent and rapidity of the outflow obstruction. Although the etiology and treatment are completely different in VOD, BCS, and CH; the similarities in clinical manifestations and liver histology may suggest a common mechanism of hepatic injury and adaptation in response to increased sinusoidal pressure.

Figure 1

Site of venous obstruction in veno-occlusive disease, Budd-Chiari syndrome, and congestive hepatopathy.

Figure 2

Histological examination demonstrates partial central vein occlusion in a patient with veno-occlusive disease.

Figure 3

Histological examination shows sinusoidal dilatation and congestion.

Figure 4

Abdominal ultrasonography reveals thick and obliterated middle hepatic veins.

Figure 5

Computed tomography showing central contrast enhancement in the liver.

Figure 6

Magnetic resonance imaging of the liver with contrasting agent shows that inferior vena cava is compressed by hypertrophy of caudate lobe and gross lobulation of the liver.

Figure 7

Almost completely obstructed IVC and shows collateral drainage through azygous system.

Figure 8

Ultrasound of the liver shows gross dilatation in the inferior vena cava and hepatic V.