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Key Features

Essentials of Diagnosis

  • Right upper quadrant pain and tenderness

  • Ascites

  • Imaging study showing occlusion/absence of flow in the hepatic vein(s) or inferior vena cava

  • Similar clinical picture in sinusoidal obstruction syndrome but major hepatic veins are patent

General Considerations

  • Cases in India, China, and South Africa

    • Often the result of occlusion of the hepatic portion of the inferior vena cava, presumably due to prior thrombosis

    • Clinical presentation is mild but the course is frequently complicated by hepatocellular carcinoma

  • Sinusoidal obstruction syndrome

    • Occlusion of terminal venules, which mimics Budd-Chiari syndrome clinically

    • May occur in patients who have undergone hematopoietic stem cell transplantation, particularly those with pretransplant serum aminotransferase elevations or fever during cytoreductive therapy with cyclophosphamide, azathioprine, carmustine, busulfan, etoposide, or gemtuzumab ozogamicin

    • Also common in those receiving high-dose cytoreductive therapy or high-dose total body irradiation

    • Can be caused by Comfrey or “bush teas” (pyrrolizidine alkaloids)

Etiologies

  • Hypercoagulable state

  • Caval webs

  • Myeloproliferative neoplasms, eg, polycythemia vera

  • Right-sided heart failure or constrictive pericarditis

  • Neoplasm compressing the hepatic vein

  • Paroxysmal nocturnal hemoglobinuria

  • Behçet syndrome

  • Blunt abdominal trauma

  • Oral contraceptives or pregnancy

Clinical Findings

Symptoms and Signs

  • Presentation is most commonly subacute but may be fulminant, acute, or chronic

  • Tender, painful hepatic enlargement

  • Jaundice; splenomegaly; and ascites

  • With chronic disease, bleeding varices and hepatic encephalopathy may be evident

  • Hepatopulmonary syndrome may occur

Differential Diagnosis

  • Cholecystitis

  • Shock liver

  • Cirrhosis

  • Hepatic congestion from right-sided heart failure

  • Metastatic cancer involving the liver

Diagnosis

Laboratory Tests

  • Liver biochemical test abnormalities are nonspecific

  • Jaundice may or may not be present

  • Very high serum alanine aminotransferase/aspartate aminotransferase levels (ALT/AST) (> 1000 units/L) suggest occlusion of hepatic and portal veins

  • Signs of decompensated liver disease (low albumin, coagulopathy) indicate poor prognosis

Imaging Studies

  • Hepatic imaging studies may show a prominent caudate lobe, since its venous drainage may be occluded

  • Contrast-enhanced, color or pulsed-Doppler ultrasonography

    • Screening test of choice

    • Has a sensitivity of 85% for detecting evidence of hepatic venous or inferior vena caval thrombosis

  • MRI with spin-echo and gradient-echo sequences and intravenous gadolinium injection allows visualization of the obstructed veins and collateral vessels

  • Direct venography can delineate caval webs and occluded hepatic veins

Diagnostic Procedures

  • Percutaneous or transjugular liver biopsy

    • Frequently shows a characteristic centrilobular congestion and fibrosis and often multiple large regenerative nodules

    • Often contraindicated in sinusoidal obstruction syndrome because of thrombocytopenia, and the diagnosis is based on clinical findings

Treatment

Medications

  • Lifelong anticoagulation and treatment of the underlying myeloproliferative disease is often required

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