33. Vascular Diseases of the Liver

  1. Eugene R. Schiff MD, MACP, FRCP3,
  2. Willis C. Maddrey MD, MACP, FRCP4 and
  3. Michael F. Sorrell MD, FACP5
  1. Ranjeeta Bahirwani1,
  2. Mark A. Rosen1 and
  3. Thomas W. Faust MD, MBE2

Published Online: 31 OCT 2011

DOI: 10.1002/9781119950509.ch33

Schiff's Diseases of the Liver, Eleventh Edition

Schiff's Diseases of the Liver, Eleventh Edition

How to Cite

Bahirwani, R., Rosen, M. A. and Faust, T. W. (2011) Vascular Diseases of the Liver, in Schiff's Diseases of the Liver, Eleventh Edition (eds E. R. Schiff, W. C. Maddrey and M. F. Sorrell), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781119950509.ch33

Editor Information

  1. 3

    Center for Liver Diseases and Schiff Liver Institute, University of Miami Miller School of Medicine, Miami, FL, USA

  2. 4

    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA

  3. 5

    University of Nebraska College of Medicine, Omaha, NE, USA

Author Information

  1. 1

    Division of Gastroenterology, Department of Internal Medicine, The University of Pennsylvania School of Medicine, Philadelphia, PA, USA

  2. 2

    University of Pennsylvania Health System, Philadelphia, PA, USA

Publication History

  1. Published Online: 31 OCT 2011
  2. Published Print: 9 DEC 2011

ISBN Information

Print ISBN: 9780470654682

Online ISBN: 9781119950509

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Keywords:

  • Budd–Chiari syndrome;
  • hereditary hemorrhagic telangectasia;
  • peliosis hepatitis;
  • vascular disorders;
  • liver

Summary

Budd–Chiari syndrome refers to the thrombotic obstruction of the hepatic venous outflow system. Occlusion may occur in hepatic venules, hepatic veins, and the inferior vena cava up to the right atrium. Hypercoagulable states are responsible for most cases of Budd–Chiari syndrome, and anticoagulation is the mainstay of medical treatment. Clinical presentation is variable and depends on the extent and speed of occlusion, as well as the development of collateral circulation. Hepatic venography is the diagnostic standard and is important for management. Angioplasty and thrombolysis may be used for therapy; transjugular intrahepatic portosystemic shunting and surgical decompression are options for progressive liver disease despite anticoagulation. Patients with decompensated cirrhosis or acute liver failure may require liver transplantation. Hereditary hemorrhagic telangiectasia is characterized by the presence of epistaxis, mucocutaneous telangiectasias, and visceral arteriovenous malformations. Hepatic involvement is usually asymptomatic. Peliosis hepatitis, characterized by cystic, blood-filled lesions in the liver, occurs in the setting of immunosuppression or human immunodeficiency virus infection.