Sinusoidal Obstruction Syndrome (Venoocclusive Disease)





KEY FACTS


Terminology





  • Hepatic venous outflow obstruction due to occlusion of terminal hepatic venules and sinusoids



  • Synonym: Hepatic sinusoidal obstruction syndrome



Imaging





  • Hepatosplenomegaly, ascites, gallbladder wall thickening



  • Narrowing of hepatic veins



  • Dilatation of main portal vein



  • Appearance or dilatation of paraumbilical vein



  • Color Doppler ultrasound




    • Elevated hepatic arterial velocity > 100 cm/s



    • Slow portal venous velocity (< 10 cm/s) or hepatofugal flow




Top Differential Diagnoses





  • Graft-vs.-host disease



  • Budd-Chiari syndrome



  • Portal vein thrombosis



  • Portal hypertension



  • Opportunistic infection



Pathology





  • Injury to hepatic venous endothelium



  • Progresses to deposition of fibrinogen + factor VIII within venule and sinusoidal walls



  • Progressive venular obstruction, centrilobular hemorrhagic necrosis



  • Sclerosis of venular wall and intense collagen deposition in sinusoids and venules



Clinical Issues





  • Occurs most frequently following hematopoietic cell transplantation




    • Responsible for 5-15% of deaths in population with VOD




  • Signs and symptoms of liver failure with painful hepatomegaly, jaundice, peripheral edema, unexplained weight gain



  • Clinical and laboratory features of VOD usually begin within 3 weeks of transplantation







Color Doppler US of the liver shows hepatofugal flow in the main portal vein in a patient with venoocclusive disease (VOD) after bone marrow transplant for AML. Note edematous appearance of the liver and hypertrophied hepatic artery .








On pulsed Doppler US in the same patient, peak systolic velocity measured at the common hepatic artery is elevated to 168 cm/s, confirming the high-flow state of the hepatic artery related to hepatic arterial buffer response to hepatofugal portal flow.








Grayscale USshows a markedly edematous and enlarged liver resulting in narrowed hepatic veins and small-caliber inferior vena cava in this patient with VOD. A small right pleural effusion is also evident.








Grayscale US shows diffuse gallbladder wall thickening and sludge in this patient with VOD. Gallbladder wall thickening in isolation is a nonspecific finding. However, in combination with other sonographic findings of VOD, it is supportive of this diagnosis.

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Nov 9, 2024 | Posted by in ULTRASONOGRAPHY | Comments Off on Sinusoidal Obstruction Syndrome (Venoocclusive Disease)

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