Skip to main content
SpringerLink
Log in

Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients

  • Gastrointestinal
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Purpose

To analyse quantitatively and qualitatively asymptomatic hepatic and pancreatic involvement in hereditary haemorrhagic telangiectasia (HHT) using 64-section helical CT.

Materials and methods

The 64-section helical CT examinations of 19 patients with HHT (8 men, 11 women; mean age, 58.6 years) were quantitatively and qualitatively analysed and compared to those of 19 control subjects who were matched for age and sex. Comparisons were made using univariate analysis.

Results

Dilated and tortuous intrahepatic arterial branches was the most discriminating independent variable (P < 0.0001) and had the highest specificity (100%; 19/19; 95%CI: 82%–100%) and accuracy (97%; 37/38; 95%CI: 86%–100%) for the diagnosis of HHT. Heterogeneous enhancement of hepatic parenchyma, intrahepatic telangiectases, hepatic artery to hepatic vein shunting, hepatic artery enlargement (i.e. diameter > 6.5 mm) and portal vein enlargement (i.e. diameter > 13 mm) were other variables that strongly correlated with the presence of HHT. Intrapancreatic telangiectases and arteriovenous malformations were found in 42% and 16% of patients with HHT, respectively.

Conclusion

Liver and pancreatic involvement in asymptomatic HHT patients is associated with myriad suggestive findings on 64-section helical CT. It can be anticipated that familiarity with these findings would result in more confident diagnosis of HHT.

Key Points

• HHT hepatic and pancreatic involvement is associated with myriad findings shown by CT.

• 64-section helical CT depicts hepatic and pancreatic involvement in asymptomatic HHT patients

• Multidetector CT shows discriminating intrahepatic abnormalities for the diagnosis of HHT

• CT heightens confidence in diagnosing hereditary haemorrhagic telangiectasia in equivocal cases

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8

Similar content being viewed by others

References

  1. Guttmacher AE, Marchuk DA, White RI (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med 333:918–924

    Article  PubMed  CAS  Google Scholar 

  2. Begbie ME, Wallace GM, Shovlin CL (2003) Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J 79:18–24

    Article  PubMed  CAS  Google Scholar 

  3. Fuchizaki U, Miyamori H, Kitagawa S, Kaneko S, Kobayashi K (2003) Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease). Lancet 362:1490–1494

    Article  PubMed  Google Scholar 

  4. Reilly PJ, Nostrant TT (1984) Clinical manifestations of hereditary hemorrhagic telangiectasis. Am J Gastroenterol 79:363–367

    PubMed  CAS  Google Scholar 

  5. Buscarini E, Buscarini L, Civardi G, Arruzzoli S, Bossalini G, Piantanida M (1994) Hepatic vascular malformations in hemorrhagic hereditary telangiectasia: imaging findings. AJR Am J Roentgenol 163:1105–1110

    PubMed  CAS  Google Scholar 

  6. Ravard G, Soyer P, Boudiaf M, Terem C, Abitbol M, Yeh JF et al (2004) Hepatic involvement in hereditary hemorrhagic telangiectasia: helical computed tomography features in 24 consecutive patients. J Comput Assist Tomogr 28:488–495

    Article  PubMed  Google Scholar 

  7. Wu JS, Saluja S, Garcia-Tsao G, Chong A, Henderson KJ, White RI Jr (2006) Liver involvement in hereditary hemorrhagic telangiectasia: CT and clinical findings do not correlate in symptomatic patients. AJR Am J Roentgenol 187:W399–W405

    Article  PubMed  Google Scholar 

  8. Ouchi K, Matsubara S, Mikuni J, Katayose Y, Endo K, Matsuno S (1994) The radiologic presentation of Osler-Weber-Rendu disease of the liver. Am J Gastroenterol 89:425–428

    PubMed  CAS  Google Scholar 

  9. Siddiki H, Doherty MG, Fletcher JG, Stanson AW, Vrtiska TJ, Hough DM et al (2008) Abdominal findings in hereditary hemorrhagic telangiectasia: pictorial essay on 2D and 3D findings with isotropic multiphase CT. Radiographics 28:171–184

    Article  PubMed  Google Scholar 

  10. Cooney T, Sweeney EC, Coll R, Greally M (1977) Pseudocirrhosis in hereditary haemorrhagic telangiectasia. J Clin Pathol 30:1134–1141

    Article  PubMed  CAS  Google Scholar 

  11. Hatzidakis AA, Gogas C, Papanikolaou N, Samonakis D, Kofteridis D, Gourtsoyiannis NC (2002) Hepatic involvement in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Eur Radiol Suppl 3:S51–S55

    Google Scholar 

  12. Lacout A, Pelage JP, Lesur G, Chinet T, Beauchet A, Roume J, Lacombe P (2010) Pancreatic involvement in hereditary hemorrhagic telangiectasia: assessment with multidetector helical CT. Radiology 254:479–484

    Article  PubMed  Google Scholar 

  13. Jaskolka J, Wu L, Chan RP, Faughnan ME (2004) Imaging of hereditary hemorrhagic telangiectasia. AJR Am J Roentgenol 183:307–314

    PubMed  Google Scholar 

  14. Ianora AA, Memeo M, Sabba C, Cirulli A, Rotondo A, Angelelli G (2004) Hereditary hemorrhagic telangiectasia: multi-detector row helical CT assessment of hepatic involvement. Radiology 230:250–259

    Article  PubMed  Google Scholar 

  15. Manfredi R, De Gaetano AM, Natale L (1993) Non-invasive integrated imaging of Rendu-Osler disease with hepatic involvement. Radiol Med 86:922–925

    PubMed  CAS  Google Scholar 

  16. Memeo M, Stabile Ianora AA, Scardapane A, Buonamico P, Sabba C, Angelelli G (2004) Hepatic involvement in hereditary hemorrhagic telangiectasia: CT findings. Abdom Imaging 29:211–220

    Article  PubMed  CAS  Google Scholar 

  17. Rydberg J, Liang Y, Teague SD (2003) Fundamentals of multichannel CT. Radiol Clin North Am 41:465–474

    Article  PubMed  Google Scholar 

  18. Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ et al (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 91:66–67

    Article  PubMed  CAS  Google Scholar 

  19. Vilgrain V, Boulos L, Vullierme MP, Denys A, Terris B, Menu Y (2000) Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics 20:379–397

    PubMed  CAS  Google Scholar 

  20. Emiroglu R, Coskun M, Yilmaz U, Sevmis S, Ozcay F, Haberal M (2006) Safety of multidetector computed tomography in calculating liver volume for living-donor liver transplantation. Transplant Proc 38:3576–3578

    Article  PubMed  CAS  Google Scholar 

  21. Zappa M, Dondero F, Sibert A, Vullierme MP, Belghiti J, Vilgrain V (2009) Liver regeneration at day 7 after right hepatectomy: global and segmental volumetric analysis by using CT. Radiology 252:426–432

    PubMed  Google Scholar 

  22. Moriyasu F, Ban N, Nishida O (1986) Clinical application of an ultrasonic duplex system in the quantitative measurement of portal blood flow. J Clin Ultrasound 14:579–588

    Article  PubMed  CAS  Google Scholar 

  23. Breiman L, Friedman JH, Olshen RA, Stone CJ (1984) Classification and regression trees. Wadsworth and Brooks, Monterey

    Google Scholar 

  24. Bernard G, Mion F, Henry L, Paliard P (1993) Hepatic involvement in hereditary hemorrhagic telangiectasia: clinical, radiological, and hemodynamic studies of 11 cases. Gastroenterology 105:482–487

    PubMed  CAS  Google Scholar 

  25. Martini GA (1978) The liver in hereditary hemorrhagic telangiectasia: an inborn error of vascular structure with multiple manifestations: a reappraisal. Gut 19:531–537

    Article  PubMed  CAS  Google Scholar 

  26. Vilgrain V, Menu Y (1991) Nahum H (1991) Doppler sonography in Osler-Weber-Rendu disease. AJR Am J Roentgenol 157:413–414

    PubMed  CAS  Google Scholar 

  27. Hutin JF, Grenier N, Schmitt N, Pledran B, Elie G, Philippe JC, Delmas J, Broussin J (1989) Angiography of hepatic angiomatosis in Rendu-Osler disease: a propos of 3 cases. J Radiol 70:621–627

    PubMed  CAS  Google Scholar 

  28. Goes R, van Tussenbroeck F, Cattenie F, Hulstaert J, Osteaux M (1987) Osler’s disease diagnosed by ultrasound. J Clin Ultrasound 15:129–131

    Article  PubMed  CAS  Google Scholar 

  29. Garcia-Tsao G, Korzenik R, Young L, Henderson J, Jain D, Byrd B, Pollak J, White R (2000) Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 343:931–936

    Article  PubMed  CAS  Google Scholar 

  30. Wanless JR, Gryse A (1986) Nodular transformation of the liver in hereditary hemorrhagic telangiectasia. Arch Pathol Lab Med 110:331–336

    PubMed  CAS  Google Scholar 

  31. Lu D, Lin J, Kadell B (1996) Congenital or idiopathic hepatic vascular malformations: CT findings. AJR Am J Roentgenol 167:1155–1157

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Abdominal Imaging, Hôpital Lariboisière-APHP, 2 rue Ambroise Paré, 75475, Paris cedex 10, France

    Matthias Barral, Marc Sirol, Vinciane Placé, Lounis Hamzi, Etienne Gayat, Mourad Boudiaf & Philippe Soyer

  2. Université Diderot-Paris 7, UFR de Médecine, 10 Avenue de Verdun, 75010, Paris, France

    Vinciane Placé & Philippe Soyer

  3. Deparment of Ear, Nose and Throat, Hôpital Lariboisière-APHP, 2 rue Ambroise Paré, 75475, Paris cedex 10, France

    Michel Borsik

  4. Unité 965 INSERM/Paris7, Hôpital Lariboisière-APHP, 2 rue Ambroise Paré, 75010, Paris, France

    Philippe Soyer

Authors
  1. Matthias Barral
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marc Sirol
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Vinciane Placé
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Lounis Hamzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Michel Borsik
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Etienne Gayat
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Mourad Boudiaf
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Philippe Soyer
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Philippe Soyer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Barral, M., Sirol, M., Placé, V. 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, 161–170 (2012). https://doi.org/10.1007/s00330-011-2243-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00330-011-2243-y

Keywords

Search

Navigation