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Veno-occlusive liver disease: causes, symptoms, diagnosis, treatment
Last reviewed: 05.07.2025
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Veno-occlusive disease of the liver (sinusoidal occlusion syndrome) is caused by obstruction of the terminal hepatic venules and sinusoids of the liver, rather than the hepatic veins or inferior vena cava.
Causes of veno-occlusive disease of the liver
Venous congestion causes ischemic necrosis, which can lead to cirrhosis and portal hypertension. Major causes include radiation, graft-versus-host disease after bone marrow (or hematopoietic cell) transplantation, pyrrolizidine alkaloids from Crotalaria and Senecio plants (eg, medicinal teas), and other hepatotoxins (eg, nitrosodimethylamine, aflatoxin, azathioprine, some antineoplastic drugs).
Symptoms of veno-occlusive liver disease
Initial symptoms of veno-occlusive disease include sudden jaundice, ascites, and hepatomegaly—the liver is enlarged, tender, and smooth. In bone marrow recipients, the disease develops within the first 2 weeks after transplantation. Some cases recover spontaneously within a few weeks (patients with mild cases may respond to increased immunosuppression); in others, patients die of fulminant liver failure. The remaining patients develop recurrent ascites, portal hypertension, and eventually cirrhosis.
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Diagnosis of veno-occlusive disease of the liver
The diagnosis is suggested by the development of typical features, especially in bone marrow transplant recipients. Liver function tests, ultrasound, and PT/INR are necessary. Classic abnormalities include elevated aminotransferases, conjugated bilirubin, and PT/INR in severe cases. Ultrasound demonstrates retrograde flow in the portal vein. In patients with typical clinical, laboratory, and ultrasound features, especially after bone marrow transplantation, no further investigation is necessary. However, if the diagnosis is unclear, liver biopsy or determination of the pressure difference between the hepatic veins and the portal vein is necessary. A pressure difference of more than 10 mmHg confirms veno-occlusive disease.
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Treatment of veno-occlusive liver disease
Treatment of hepatic veno-occlusive disease includes elimination of the etiologic factor, symptomatic supportive care, and transjugular intrahepatic stenting in the case of portal hypertension. The last resort is liver transplantation. The use of ursodeoxycholic acid may be useful in preventing graft-versus-host disease after bone marrow transplantation.