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Acquired multifactorial condition caused by postsinusoidal hepatic venous outflow obstruction and ascites. The cause of the obstruction may be related to hypercoagulability of the blood, infections, cancer, and other conditions, which may be acute, chronic, or asymptomatic. The leading cause (in up to 40% of cases), however, is “idiopathic.”

Chiari Syndrome; Venoocclusive Disease of the Liver.

More than 4000 cases have been described.

No genetic basis has been described. The etiology is multifactorial; a causative factor is frequently not identified. Risk factors include myeloproliferative disease, polycythemia rubra vera, thrombophilia, including factor V Leiden, paroxysmal nocturnal hemoglobinuria, lupus anticoagulant, protein C deficiency, antithrombin III deficiency, α1-antitrypsin deficiency, oral contraceptive medication, pregnancy, malignancy, systemic and local infection, autoimmune disease, trauma, sarcoidosis, and membranous webs.

The symptoms result from decreased hepatic venous outflow, which may be secondary to an obstruction of small hepatic venules (e.g., following hepatotoxin ingestion), major hepatic veins (caused by hypercoagulable states, malignancy, or other conditions), or the inferior vena cava between the hepatic veins and the right atrium (e.g., caused by a membranous web, thrombosis, progression of renal adenocarcinoma, or compression from outside). Obstruction to flow results in portal hypertension and hepatocellular damage. Cirrhosis and portal hypertension eventually develop. The two-year mortality rate in untreated disease is 20 to 40%.

The most important diagnostic tool is ultrasonography with the pathognomonic finding of absent hepatic vein images, intrahepatic collaterals enlargement of the caudate lobe of the liver, and lack of portal venous, hepatic, and inferior vena cava blood flow. Alternatively, hepatic venography or MRI may be used.

Depending on the level of the obstruction, 3 types of Budd-Chiari syndrome can be distinguished:

  • Type I: occlusion of the inferior vena cava with or without secondary hepatic vein occlusion.
  • Type II: occlusion of major hepatic veins.
  • Type III: obstruction of small centrilobular venules (also called veno-occlusive disease).

Very rare in infants and young children. It may present as either fulminant hepatic failure associated with pain, hepatomegaly, jaundice, ascites, and rapid deterioration of hepatic function with encephalopathy requiring acute intervention, or acute nonfulminant Budd-Chiari syndrome associated with pain, ascites, and hepatomegaly. Chronic Budd-Chiari syndrome with insidious onset of abdominal discomfort and ascites because of portal hypertension is also possible. Occlusion of the inferior vena cava may cause edema of the lower limbs and varicose veins of the legs and lower trunk. Patients may present with esophageal varices. Management of Budd-Chiari syndrome may include liver transplantation, particularly in fulminant forms, formation of cavoatrial or mesoatrial shunts, and use of thrombolytic agents. Transjugular intrahepatic parto-systemic shunts (TIRS) is becoming popular to decompress the portal venous system.

Preoperative investigation should be aimed at determining the extent of liver and other concomitant organ disease in addition to the effects of therapy (e.g., diuretics, anticoagulants, chemotherapy). In severe disease, multisystem involvement is seen. The operative risk ...

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