| disease | Budd-Chiari Syndrome |
| alias | Hepatic Veno-Occlusive Syndrome, Budd-Chiari Syndrome |
Budd-Chiari Syndrome is a syndrome primarily characterized by impaired hepatic blood outflow due to partial or complete obstruction of the hepatic veins or inferior vena cava, also known as hepatic vein occlusion syndrome. This syndrome was first reported by Budd in 1846 and Chiari in 1889. The main clinical features include abdominal pain, hepatosplenomegaly, ascites, and lower limb edema. This condition is relatively rare and can occur at any age, but is most common in individuals aged 20 to 40.
bubble_chart Etiology
The causes of this condition include thrombosis of the hepatic veins and inferior vena cava, compression by lesions of adjacent organs, and intrinsic pathologies of the hepatic veins and inferior vena cava, such as congenital developmental abnormalities and obliterative phlebitis.
bubble_chart Pathological Changes
Thrombosis can be observed in the hepatic veins and inferior vena cava, often with occlusion in the acute phase. In the chronic phase, the hepatic veins show inflammatory changes, with thickening of the venous wall, thrombus organization, and recanalization of the lumen. Hepatosplenomegaly is present. Microscopically, dilation of the central veins of the hepatic lobules is seen, along with congestion, hemorrhage, and dilation of the hepatic sinusoids, as well as central hepatocyte atrophy and necrosis. In the advanced stage, fibrosis occurs in the central area of the lobules, accompanied by hepatic tissue regeneration and cirrhosis.
bubble_chart Clinical Manifestations
This condition often follows a chronic course, with clinical manifestations mainly including two aspects:
1. Hepatic venous outflow obstruction manifestations, often featuring progressive hepatosplenomegaly, esophageal and abdominal wall varices, ascites, liver area pain, abdominal pain, etc., with jaundice being rare. In the acute phase of venous thrombosis, varying degrees of fever and liver area pain may occur, even leading to shock and death. In the advanced stage, manifestations of portal hypertension and cirrhosis may appear.
2. Inferior vena cava outflow obstruction manifestations, such as lower limb edema, cyanosis, superficial varicose veins, pigmentation or ulcers, and a heavy, numb sensation in the lower limbs. The blood flow direction in the varicose veins is consistently upward.bubble_chart Auxiliary Examination
1. The degree of liver function injury is mild, with possible delayed indocyanine green excretion, elevated serum alkaline phosphatase and transaminase, and decreased albumin.
2. B-ultrasound and CT scans can reveal the location, extent, and severity of hepatic vein and inferior vena cava stenosis or occlusion, as well as morphological changes in the liver.
3. Inferior vena cava angiography and selective hepatic vein angiography can accurately determine the location, extent, nature, collateral circulation, and presence of external compression of the obstructive lesion.
4. Liver biopsy shows congestion and sinusoid dilation around the central vein of the lobule, with advanced stage presenting cirrhotic changes.
The main diagnostic criteria are hepatosplenomegaly, ascites, and the characteristic extensive abdominal wall varices. This syndrome has three important features:
1. The patients are mostly younger in age.
2. The characteristic of the varices is that they are located on the lateral thorax and abdominal wall, and the blood flow direction below the umbilicus is also from bottom to top.
3. Although it resembles cirrhosis, there is no significant liver function injury.
Hepatic vein and inferior vena cava angiography are the main methods for definitive diagnosis.
bubble_chart Treatment Measures
The treatment should primarily focus on disease cause therapy and symptomatic treatment. For thrombus formation, anticoagulants may be tried. For simple venous stenosis and incomplete occlusion, a metal elastic stent can be placed. For cases of complete venous occlusion, complex disease causes, or those unsuitable for venous catheter therapy, surgical treatment can be adopted. The goal is to relieve lower limb and abdominal congestion as well as portal hypertension. Surgical methods include: portocaval shunt, membrane fragmentation, membrane resection, splenopneumopexy, and occluded venous incision repair.
It should be differentiated from cryptogenic cirrhosis, posthepatitic cirrhosis, portal vein thrombosis, and constrictive pericarditis.
