Hepatic venous outflow obstruction: Three similar syndromes

Abstract

Our goal is to provide a detailed review of venoocclusive 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.

Hemopexin Prevents Endothelial Damage and Liver Congestion in a Mouse Model of Heme Overload

“…VOD is attributable to hepatic venous outflow obstruction and is characterized by hepatomegaly, marked hyperbilirubinemia, and at histological level, narrowing of central veins and sinusoids, sinusoidal congestion, and hepatocellular necrosis. 32 VOD is attributable to a nonthrombotic sinusoidal obstruction, which occurs as a result of sinusoidal endothelial cell injury. This disease is most frequently caused by hematopoietic stem cell transplantation and is also seen after solid organ transplantation.…”

Budd–Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging

“…This definition excludes drainage abnormalities of the hepatic venous system secondary to cardiac insufficiency, pericardium disease, and veinoocclusive disease of the liver [2]. BCS is commonly classified into primary or secondary BCS [6]. Primary BCS is the consequence of a venous obstruction that is due in most cases to thrombosis and less frequently to hepatic vein stenosis.…”

“…Primary BCS is the consequence of a venous obstruction that is due in most cases to thrombosis and less frequently to hepatic vein stenosis. Secondary BCS results from venous obstruction due to an external compression by a tumor or by an infectious process [4,[6][7][8]. The etiologies of primary BCS and location of causative lesions greatly vary among countries.…”

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

“…1 Prolonged venous obstruction in HVOTO causing perivenular hepatocyte necrosis with resultant bridging fibrosis between the adjacent central veins with subsequent development of cirrhosis of the liver is well established. 2 Hepatocellular carcinoma (HCC) is the most common primary hepatic malignancy and the third leading cause of cancer-related mortality worldwide. 3 More than 80-90% of cases of HCC have underlying cirrhosis.…”