Skip to main content
Log in

Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations

  • Hepatobiliary-Pancreas
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

At present there is no established therapy for treating patients with hereditary hemorrhagic telangiectasia (HHT) and symptomatic hepatic involvement. We present the results of a prospective study with 15 consecutive patients who were treated with staged hepatic artery embolization (HAE). Branches of the hepatic artery were selectively catheterized and embolized in stages using polyvinyl alcohol particles (PVA) and platinum microcoils or steel macrocoils. Prophylactic antibiotics, analgesics and anti-emetics were administered after every embolization. Clinical symptomatology and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 28 months; range 10–136 months). Five patients had abdominal pain and four patients had symptoms of portal hypertension. The cardiac output was raised in all patients, with cardiac failure being present in 11 patients. After treatment, pain resolved in all five patients, and portal hypertension improved in two of the four patients. The mean cardiac output decreased significantly (P<0.001) from 12.57±3.27 l/min pre-treatment to 8.36±2.60 l/min at the end of follow-up. Symptoms arising from cardiac failure resolved or improved markedly in all but one patient. Cholangitis and/or cholecystitis occurred in three patients of whom two required a cholecystectomy. One patient with pre-existent hepatic cirrhosis died as a complication of the procedure. Staged HAE yields long-term relief of clinical symptoms in patients with HHT and hepatic involvement. Patients with pre-existing hepatic cirrhosis may be poor candidates for HAE.

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

Access this article

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

Similar content being viewed by others

References

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

    CAS  PubMed  Google Scholar 

  2. Reilly PJ, Nostrant TT (1984) Clinical manifestations of hereditary haemorrhagic teleangiectasia. Am J Gastroenterol 79:363–367

    CAS  PubMed  Google Scholar 

  3. Bernard G, Mion F, Henry L, Plauchu H, Paliard P (1993) Hepatic involvement in hereditary haemorrhagic teleangiectasia: clinical, radiological and hemodynamic studies of 11 cases. Gastroenterology 105:482–487

    CAS  PubMed  Google Scholar 

  4. Guttmacher AE, Marchuk DA, White RI (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med 333(14):918–924

    Article  CAS  PubMed  Google Scholar 

  5. Haitjema T, Westermann CJ, Overtoom TT et al (1996) Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): new insights into pathogenesis, complications and treatment. Arch Intern Med 156:714–719

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  7. Geisthoff UW, Schneider G, Fischinger J, Plinkert PK (2002) Hereditäre hämorrhagische Teleangiektasie (Morbus Osler): Eine interdisziplinäre Herausforderung. HNO 50:114–128

    Article  CAS  PubMed  Google Scholar 

  8. Chavan A, Galanski M, Wagner S, Caselitz M, Schlitt HJ, Gratz KF, Manns M (1998) Hereditary hemorrhagic telangiectasia: effective protocol for embolization of hepatic vascular malformations—experience in five patients. Radiology 209:735–739

    CAS  PubMed  Google Scholar 

  9. DeLorimier A, Simpson E, Baum R et al (1967) Hepatic artery ligation for hepatic hemangiomatosis. N Engl J Med 277:333–337

    CAS  PubMed  Google Scholar 

  10. Radtke WE, Smith HC, Fulton RE, Adson MA (1978) Misdiagnosis of atrial septal defect in patients with hereditary telangiectasia (Osler-Rendu-Weber disease) and hepatic arteriovenous fistulas. Am Heart J 95:235–242

    Article  CAS  PubMed  Google Scholar 

  11. Zentler-Munro PL, Howard ER, Karani J, Williams R (1989) Variceal haemorrhage in hereditary hemorrhagic telangiectasia. Gut 30:1293–1297

    CAS  PubMed  Google Scholar 

  12. Neumann U-P, Knoop M, Langrehr J-M et al (1998) Effective therapy for hepatic M. Osler with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation. Transpl Int 11:323–326

    Article  CAS  PubMed  Google Scholar 

  13. Zieren J, Buttemeyer R, Muller JM (1998) Adjustable “banding” of the hepatic artery in treatment of shunt-induced heart failure in Osler-Rendu-Weber disease. Chirurg 69:639–641

    Article  CAS  PubMed  Google Scholar 

  14. Graham WP, Eiseman B, Pryor R (1964) Hepatic artery aneurysm with portal vein fistula in a patient with familial hereditary telangiectasia. Ann Surg 159:362–367

    PubMed  Google Scholar 

  15. Göthlin JH, Nordgard K, Jonsson K, Nyman U (1982) Hepatic telangiectasia in Osler’s disease treated with arterial embolisation. Report of 2 cases. Eur J Radiol 2:27–30

    PubMed  Google Scholar 

  16. Brohee D, Franken P, Fievez M et al (1984) High-output right ventricular failure secondary to hepatic arteriovenous microfistulae. Selective arterial embolization treatment. Arch Intern Med 144:1282–1284

    Article  CAS  PubMed  Google Scholar 

  17. Allison D, Jordon J, Hennessey O (1985) Therapeutic embolization of the hepatic artery: a review of seventy-five procedures. Lancet 1:595–599

    Article  CAS  PubMed  Google Scholar 

  18. Roman CF, Do Cha S, Incarvito J, Cope C, Maranhao V (1987) Transcatheter embolization of hepatic arteriovenous fistula in Osler-Weber-Rendu disease—a case report. Angiology 38:484–488

    CAS  PubMed  Google Scholar 

  19. Derauf BJ, Hunter DW, Sirr SA, Cardella JF, Castaneda-Zuniga W, Amplatz K (1987) Peripheral embolization of diffuse hepatic arteriovenous malformations in a patient with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol 10:80–83

    CAS  PubMed  Google Scholar 

  20. Nikolopoulos N, Xynos E, Vassilakis JS (1988) Familial occurrence of hyperdynamic circulation status due to intrahepatic fistulae in hereditary hemorrhagic telangiectasia. Hepatogastroenterology 35:167–168

    CAS  PubMed  Google Scholar 

  21. Bourgeois N, Delcour C, Deviere J et al (1990) Osler–Weber–Rendu disease associated with hepatic involvement and high output heart failure. J Clin Gastroenterol 12:236–238

    CAS  PubMed  Google Scholar 

  22. Whiting JH Jr, Morton KA, Datz FL, Patch GG, Miller FJ Jr (1992) Embolization of hepatic arteriovenous malformations using radiolabeled and nonradiolabeled polyvinyl alcohol sponge in a patient with hereditary hemorrhagic telangiectasia: case report. J Nucl Med 33(2):260–262

    PubMed  Google Scholar 

  23. Caselitz M, Wagner S, Chavan A, Gebel M, Bleck JS, Wu A, Schlitt HJ, Galanski M, Manns M (1998) Clinical outcome of transfemoral embolisation in patients with arteriovenous malformations of the liver in hereditary haemorrhagic telangiectasia (Weber-Rendu-Osler disease). Gut 42:123–126

    CAS  PubMed  Google Scholar 

  24. Trotter JF, Suhocki PV, Lina JR, Martin LW, Parrish JL, Swantkowski T (1998) Hereditary hemorrhagic telangiectasia causing high output cardiac failure: treatment with transcatheter embolization. Am J Gastroenterol 93:1569–1571

    Article  CAS  PubMed  Google Scholar 

  25. Odorico JS, Hakim NM, Becker YT et al (1998) Liver transplantation as definitive therapy for complications after arterial embolization for hepatic manifestations of hereditary hemorrhagic telangiectasia. Liver Transpl Surg 4:483–490

    Article  CAS  PubMed  Google Scholar 

  26. Chavan A, Galanski M, Wagner S, Caselitz M, Schlitt HJ, Gratz KF, Manns M (1999) Hereditary hemorrhagic telangiectasia (reply—letters to the editor). Radiology 213:928–930

    PubMed  Google Scholar 

  27. Stockx L, Raat H, Caerts B et al (1999) Transcatheter embolization of hepatic arteriovenous fistulas in Rendu-Osler-Weber disease: a case report and review of the literature. Eur Radiol 9(7):1434–1437

    Article  CAS  PubMed  Google Scholar 

  28. Hisamatsu K, Ueeda M, Ando M et al (1999) Peripheral arterial coil embolization for hepatic arteriovenous malformation in Osler–Weber–Rendu disease; useful for controlling high output heart failure, but harmful to the liver. Intern Med 38:962–968

    CAS  PubMed  Google Scholar 

  29. Whiting JH Jr, Korzenik JR, Miller FJ Jr et al (2000) Fatal outcome after embolotherapy for hepatic arteriovenous malformations of the liver in two patients with hereditary hemorrhagic telangiectasia. J Vasc Interv Radiol 11:855–858

    PubMed  Google Scholar 

  30. Pfitzmann R, Heise M, Langrehr JM, Jonas S, Steinmüller T, Podrabsky P, Ewert R, Settmacher U, Neuhaus R, Neuhaus P (2001) Liver transplantation for treatment of intrahepatic Osler’s disease: first experiences. Transplantation 72:237–241

    Article  CAS  PubMed  Google Scholar 

  31. Bauer T, Britton P, Lomas D, Wight DG, Friend PJ, Alexander GJ (1995) Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia. J Hepatol 22:586–590

    Article  CAS  PubMed  Google Scholar 

  32. Saxena R, Hytiroglou P, Atillasoy EO et al (1998) Coexistence of hereditary hemorrhagic telangiectasia and fibropolycystic liver disease. Am J Surg Pathol 22:368–372

    Article  CAS  PubMed  Google Scholar 

  33. McInroy B, Zajko AB, Pinna AD (1998) Biliary necrosis due to hepatic involvement with hereditary hemorrhagic telangiectasia. Am J Roentgenol 170:413–415

    CAS  Google Scholar 

  34. Boillot O, Bianco F, Viale J-P et al (1999) Liver transplantation resolves the hyperdynamic circulation in hereditary hemorrhagic telangiectasia with hepatic involvement. Gastroenterology 116:187–192

    CAS  PubMed  Google Scholar 

  35. Le Corre F, Golkar B, Tessier C et al (2000) Liver transplantation for hepatic arteriovenous malformation with high-output cardiac failure in hereditary hemorrhagic telangiectasia; hemodynamic study. J Clin Anesth 12:339–342

    Article  PubMed  Google Scholar 

  36. Hillert C, Broering DC, Gundlach M et al (2001) Hepatic involvement in hereditary hemorrhagic telangiectasia: an unusual indication for liver transplantation. Liver Transpl 7:266–268

    Article  CAS  PubMed  Google Scholar 

  37. Caselitz M, Chavan A, Manns MP, Wagner S (2001) Die Hereditäre Hämorrhagische Teleangiectasie (Morbus Osler–Rendu–Weber) und ihre Manifestation an der Leber. Z Gastroenterol 39:533–542

    Article  CAS  PubMed  Google Scholar 

  38. Larson AM (2003) Liver disease in hereditary hemorrhagic telangiectasia. J Clin Gastroenterol 36(2):149–158

    Article  CAS  PubMed  Google Scholar 

  39. Jackson JE (1999) Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: treatment with embolization (Letter to the editor). Radiology 213:927–928

    CAS  PubMed  Google Scholar 

  40. Miller FJ, Whiting JH, Korzenik JR, White RI (1999) Caution with use of hepatic embolization in treatment of hereditary hemorrhagic telangiectasia (Letter to the editor). Radiology 213:928

    PubMed  Google Scholar 

  41. Miller FJ, Mineau DE (1983) Transcatheter arterial embolization—major complications and their prevention. Cardiovasc Intervent Radiol 6:141–149

    PubMed  Google Scholar 

  42. Jain A, Reyes J, Kashyap R et al (2000) Long-term survival after liver transplantation in 4,000 consecutive patients at a single center. Ann Surg 232(4):490–500

    Article  CAS  PubMed  Google Scholar 

  43. 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 12:S51–S55

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Ajay Chavan.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Chavan, A., Caselitz, M., Gratz, KF. et al. Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations. Eur Radiol 14, 2079–2085 (2004). https://doi.org/10.1007/s00330-004-2455-5

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00330-004-2455-5

Keywords

Navigation