Hepatic Venoocclusive Disease

David Cheng, MD
Faye C Laing, MD

Presentation

Imaging Findings

Abdominal ultrasound demonstrates a small amount of ascities, thickening of the gallbladder wall (arrow), and a right pleural effusion. Splenomegaly (not shown) was also present.

Color Doppler demonstrates reversed (hepatofugal) flow within the portal vein.

The hepatic veins (arrows) are small. Pulse wave Doppler of selected branches demonstrate a normal flow pattern. The main hepatic veins and inferior vena cava (IVC) (not shown) are patent and free of thrombus.

Diagnosis

Discussion

VOD occurs in over half of BMT patients, and although approximately half of all cases resolve, the mortality rate can be over 90% in severe cases. Risk factors in BMT patients include preexisting liver disease or liver function test abnormalities and underlying infection or antibiotic use during conditioning chemotherapy. Large studies have shown no difference between autologous and allogeneic transplantation although the use of mismatched or unrelated donor marrow may increase the incidence of VOD.

The major clinical manifestations are jaundice, painful hepatomegaly, weight gain, and ascities occurring before day 20 of BMT. Liver abnormalities occuring later may be due to graft versus host disease and not VOD. In severe cases, multiorgan failure with pulmonary infiltrates, pleural effusions, congestive heart failure, and renal failure may occur. Good correlation has been found between clinical diagnoses of VOD and pathologic results.

Many nonspecific sonographic findings have been described in patients with VOD. These include ascities, gallbladder wall thickening, and hepatosplenomegaly. Doppler ultrasound may be helpful in some cases. The demonstration of normal portal venous flow prior to transplantation with flow reversal within 3 weeks after BMT is a highly suggestive but insensitive marker for VOD.

Prospective trials have reported conflicting data regarding the utility of measuring the hepatic artery resistive index (RI). While an early study by Herbetko et al found a significantly higher hepatic artery index (0.81 vs 0.69) in patients with VOD as opposed to patients with graft versus host disease, hepatitis, or no disease, a later study by Teefey et al found no significant difference. Teefey et al has also shown that decreased portal venous flow is not significantly different between BMT patients with and without VOD. Longitudinal data obtained in these patients show that increases in the hepatic artery RI or decreases in portal venous flow during transplantation also do not correlate with clinical VOD. All patients studied, however, demonstrate normal hepatic venous flow.

Conflicting data exist regarding the efficacy of heparin prophylaxis for VOD. Other agents which have been evaluated are prostaglandin E1, a platelet inhibitor and vasodilator, and ursodeoxycholic acid, which protects hepatocytes from damage by cholestasis. Recombinant tissue plasminogen activator and prostaglandin E1 are undergoing evaluation for treatment of VOD.

References

2. Herbetko J, Grigg AP, Buckley AR, Phillips GL. Venoocclusive liver disease after bone marrow transplantation: findings at duplex sonography. AJR 1992; 158: 1001-1005.

3. Teefey SA, Brink JA, Borson RA, Middleton WD. Diagnosis of venoocclusive disease of the liver after bone marrow transplantation: value of duplex sonography. AJR 1995; 164 : 1397-1401.

4. Hommeyer SC, Teefey SA, Jacobson AF, Higano CS, Bianco JA, Colacurcio CJ, McDonald GB. Venocclusive disease of the liver: prospective study of ultrasound evaluation. Radiology 1992; 184: 683-686.

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