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Hereditary Hemorrhagic Telangiectasia
Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Hereditary hemorrhagic telangiectasia (HHT)
          • Synonyms

            • Osler-Weber-Rendu syndrome (OWR)
          • Definitions

            • Hereditary multiorgan disorder resulting in fibrovascular dysplasia with development of telangiectasias and arteriovenous malformations (AVMs)
            • Direct connection between arteries and veins with absence of capillaries; telangiectasias are small AVMs

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Gaujoux S et al: Liver resection in patients with hepatic hereditary hemorrhagic telangiectasia. Dig Surg. 30(4-6):410-4, 2013
                                        2. Barral M et al: Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients. Eur Radiol. 22(1):161-70, 2012
                                        3. Dupuis-Girod S et al: Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 307(9):948-55, 2012
                                        4. Buscarini E et al: Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia. Dig Dis Sci. 56(7):2166-78, 2011
                                        5. McDonald J et al: Hereditary hemorrhagic telangiectasia: an overview of diagnosis, management, and pathogenesis. Genet Med. 13(7):607-16, 2011
                                        6. Sekarski LA et al: Hereditary hemorrhagic telangiectasia: children need screening too. Pediatr Nurs. 37(4):163-8; quiz 169, 2011
                                        7. Siddiki H et al: Abdominal findings in hereditary hemorrhagic telangiectasia: pictorial essay on 2D and 3D findings with isotropic multiphase CT. Radiographics. 28(1):171-84, 2008
                                        8. Song X et al: Individualized management of hepatic diseases in hereditary hemorrhagic telangiectasia. Am Surg. 77(3):281-5, 2011
                                        9. Gincul R et al: Evaluation of previously nonscreened hereditary hemorrhagic telangiectasia patients shows frequent liver involvement and early cardiac consequences. Hepatology. 48(5):1570-6, 2008
                                        10. Khalid SK et al: Hepatic vascular malformations in hereditary hemorrhagic telangiectasia. Semin Liver Dis. 28(3):247-58, 2008
                                        11. Milot L et al: Hereditary-hemorrhagic telangiectasia: one-step magnetic resonance examination in evaluation of liver involvement. Gastroenterol Clin Biol. 32(8-9):677-85, 2008
                                        12. Sharathkumar AA et al: Hereditary haemorrhagic telangiectasia. Haemophilia. 14(6):1269-80, 2008
                                        13. Torabi M et al: CT of nonneoplastic hepatic vascular and perfusion disorders. Radiographics. 28(7):1967-82, 2008
                                        14. Ianora AA et al: Hereditary hemorrhagic telangiectasia: multi-detector row helical CT assessment of hepatic involvement. Radiology. 230(1):250-9, 2004
                                        15. Hashimoto M et al: Angiography of hepatic vascular malformations associated with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol. 26(2):177-80, 2003
                                        16. Larson AM: Liver disease in hereditary hemorrhagic telangiectasia. J Clin Gastroenterol. 36(2):149-58, 2003
                                        17. Matsumoto S et al: Intrahepatic porto-hepatic venous shunts in Rendu-Osler-Weber disease: imaging demonstration. Eur Radiol. 2003
                                        18. Hatzidakis AA et al: Hepatic involvement in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Eur Radiol. 12 Suppl 3:S51-5, 2002
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Hereditary hemorrhagic telangiectasia (HHT)
                                                • Synonyms

                                                  • Osler-Weber-Rendu syndrome (OWR)
                                                • Definitions

                                                  • Hereditary multiorgan disorder resulting in fibrovascular dysplasia with development of telangiectasias and arteriovenous malformations (AVMs)
                                                  • Direct connection between arteries and veins with absence of capillaries; telangiectasias are small AVMs

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Ultrasonographic Findings

                                                        • Angiographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Gaujoux S et al: Liver resection in patients with hepatic hereditary hemorrhagic telangiectasia. Dig Surg. 30(4-6):410-4, 2013
                                                                              2. Barral M et al: Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients. Eur Radiol. 22(1):161-70, 2012
                                                                              3. Dupuis-Girod S et al: Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 307(9):948-55, 2012
                                                                              4. Buscarini E et al: Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia. Dig Dis Sci. 56(7):2166-78, 2011
                                                                              5. McDonald J et al: Hereditary hemorrhagic telangiectasia: an overview of diagnosis, management, and pathogenesis. Genet Med. 13(7):607-16, 2011
                                                                              6. Sekarski LA et al: Hereditary hemorrhagic telangiectasia: children need screening too. Pediatr Nurs. 37(4):163-8; quiz 169, 2011
                                                                              7. Siddiki H et al: Abdominal findings in hereditary hemorrhagic telangiectasia: pictorial essay on 2D and 3D findings with isotropic multiphase CT. Radiographics. 28(1):171-84, 2008
                                                                              8. Song X et al: Individualized management of hepatic diseases in hereditary hemorrhagic telangiectasia. Am Surg. 77(3):281-5, 2011
                                                                              9. Gincul R et al: Evaluation of previously nonscreened hereditary hemorrhagic telangiectasia patients shows frequent liver involvement and early cardiac consequences. Hepatology. 48(5):1570-6, 2008
                                                                              10. Khalid SK et al: Hepatic vascular malformations in hereditary hemorrhagic telangiectasia. Semin Liver Dis. 28(3):247-58, 2008
                                                                              11. Milot L et al: Hereditary-hemorrhagic telangiectasia: one-step magnetic resonance examination in evaluation of liver involvement. Gastroenterol Clin Biol. 32(8-9):677-85, 2008
                                                                              12. Sharathkumar AA et al: Hereditary haemorrhagic telangiectasia. Haemophilia. 14(6):1269-80, 2008
                                                                              13. Torabi M et al: CT of nonneoplastic hepatic vascular and perfusion disorders. Radiographics. 28(7):1967-82, 2008
                                                                              14. Ianora AA et al: Hereditary hemorrhagic telangiectasia: multi-detector row helical CT assessment of hepatic involvement. Radiology. 230(1):250-9, 2004
                                                                              15. Hashimoto M et al: Angiography of hepatic vascular malformations associated with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol. 26(2):177-80, 2003
                                                                              16. Larson AM: Liver disease in hereditary hemorrhagic telangiectasia. J Clin Gastroenterol. 36(2):149-58, 2003
                                                                              17. Matsumoto S et al: Intrahepatic porto-hepatic venous shunts in Rendu-Osler-Weber disease: imaging demonstration. Eur Radiol. 2003
                                                                              18. Hatzidakis AA et al: Hepatic involvement in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Eur Radiol. 12 Suppl 3:S51-5, 2002