Hostname: page-component-cd9895bd7-8ctnn Total loading time: 0 Render date: 2024-12-28T00:57:25.706Z Has data issue: false hasContentIssue false

Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia

Published online by Cambridge University Press:  04 March 2015

I Syed*
Affiliation:
St George's Hospital, Blackshaw Road, London, UK
V S Sunkaraneni
Affiliation:
Royal Surrey County Hospital, Guildford, UK
*
Address for correspondence: Mr I Syed, St George's Hospital, Blackshaw Road, London SW17 0QT, UK E-mail: irfan.syed@doctors.org.uk

Abstract

Background:

There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis.

Method:

The Medline and Embase databases were interrogated on 15 November 2013 using the search items ‘hereditary haemorrhagic telangiectasia’ (title), ‘epistaxis’ (title) and ‘treatment’ (title and abstract), and limiting the search to articles published in English.

Results:

A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3 in vitro studies.

Conclusion:

There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

Type
Review Articles
Copyright
Copyright © JLO (1984) Limited 2015 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Plauchu, H, de Chadarévian, JP, Bideau, A, Robert, JM. Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidemiologically recruited population. Am J Med Genet 1989;32:291–7CrossRefGoogle Scholar
2Geirdal, , Dheyauldeen, S, Bachmann-Harildstad, G, Heimdal, K. Quality of life in patients with hereditary hemorrhagic telangiectasia in Norway: a population based study. Am J Med Genet 2012;158:1269–78CrossRefGoogle Scholar
3Hoag, JB, Terry, P, Mitchell, S, Reh, D, Merlo, CA. An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope 2010;120:838–43CrossRefGoogle ScholarPubMed
4Pagella, F, Colombo, A, Matti, E, Giourgos, G, Tinelli, C, Olivieri, C et al. Correlation of severity of epistaxis with nasal telangiectasias in hereditary hemorrhagic telangiectasia (HHT) patients. Am J Rhinol 2009;23:52–8CrossRefGoogle ScholarPubMed
5Faughnan, ME, Palda, VA, Garcia-Tsao, G, Geisthoff, UW, McDonald, J, Proctor, DD et al. HHT Foundation International – Guidelines Working Group. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 2011;48:7387CrossRefGoogle Scholar
6Sadick, H, Naim, R, Oulmi, J, Hörmann, K, Bergler, W. Plasma surgery and topical estriol: effects on the nasal mucosa and long-term results in patients with Osler's disease. Otolaryngol Head Neck Surg 2003;129:233–8CrossRefGoogle ScholarPubMed
7Kwaan, HC, Silverman, S. Fibrinolytic activity in lesions of hereditary hemorrhagic telangiectasia. Arch Dermatol 1973;107:571573CrossRefGoogle ScholarPubMed
8Klepfish, ABerrebi, A, Schattner, A.Intranasal tranexamic acid treatment for severe epistaxis in hereditary hemorrhagic telangiectasia. Arch Intern Med 2001;161:767CrossRefGoogle ScholarPubMed
9Flanagan, BA, Collins, C, Parra, S. Intranasal tranexamic acid for the treatment of hereditary hemorrhagic telangiectasia: a case report and review of treatment options. Cutis 2012;89:6972Google ScholarPubMed
10Sabbà, C, Gallitelli, M, Palasciano, G. Efficacy of unusually high doses of tranexamic acid for the treatment of epistaxis in hereditary hemorrhagic telangiectasia. N Engl J Med 2001;345:926CrossRefGoogle ScholarPubMed
11Karnezis, TT, Davidson, TM. Treatment of hereditary hemorrhagic telangiectasia with submucosal and topical bevacizumab therapy. Laryngoscope 2012;122:495–7CrossRefGoogle ScholarPubMed
12Karnezis, TT, Davidson, TM. Efficacy of intranasal Bevacizumab (Avastin) treatment in patients with hereditary hemorrhagic telangiectasia-associated epistaxis. Laryngoscope 2011;121:636–8CrossRefGoogle ScholarPubMed
13Rohrmeier, C, Sachs, HG, Kuehnel, TS. A retrospective analysis of low dose, intranasal injected bevacizumab (Avastin) in hereditary haemorrhagic telangiectasia. Eur Arch Otorhinolaryngol 2012;269:531–6CrossRefGoogle ScholarPubMed
14Chen, S, Karnezis, T, Davidson, TM. Safety of intranasal Bevacizumab (Avastin) treatment in patients with hereditary hemorrhagic telangiectasia-associated epistaxis. Laryngoscope 2011;121:644–6CrossRefGoogle ScholarPubMed
15Dheyauldeen, S, Østertun Geirdal, A, Osnes, T, Vartdal, LS, Dollner, R. Bevacizumab in hereditary hemorrhagic telangiectasia-associated epistaxis: effectiveness of an injection protocol based on the vascular anatomy of the nose. Laryngoscope 2012;122:1210–4CrossRefGoogle ScholarPubMed
16Alderman, C, Corlett, J, Cullis, J. The treatment of recurrent epistaxis due to hereditary haemorrhagic telangiectasia with intranasal bevacizumab. Br J Haematol 2013;162:547–8CrossRefGoogle ScholarPubMed
17Vase, P. Estrogen treatment of hereditary hemorrhagic telangiectasia. A double-blind controlled clinical trial. Acta Med Scand 1981;209:393–6CrossRefGoogle ScholarPubMed
18Flessa, HC, Glueck, HI. Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) management of epistaxis in nine patients using systemic hormone therapy. Arch Otolaryngol 1977;103:148–51CrossRefGoogle ScholarPubMed
19Richtsmeier, W, Weaver, G, Streck, W, Jacobson, H, Dewell, R, Olson, J. Estrogen and progesterone receptors in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 1984;92:564570CrossRefGoogle ScholarPubMed
20Yaniv, E, Preis, M, Hadar, T, Shvero, J, Haddad, M. Antiestrogen therapy for hereditary hemorrhagic telangiectasia: a double-blind placebo-controlled clinical trial. Laryngoscope 2009;119:284–8CrossRefGoogle ScholarPubMed
21Yaniv, E, Preis, M, Shevro, J, Nageris, B, Hadar, T.Anti-estrogen therapy for hereditary hemorrhagic telangiectasia – a long-term clinical trial. Rhinology 2011;49:214–6CrossRefGoogle ScholarPubMed
22Morais, D, Millás, T, Zarrabeitia, R, Botella, LM, Almaraz, A. Local sclerotherapy with polydocanol (Aethoxysklerol®) for the treatment of Epistaxis in Rendu-Osler-Weber or Hereditary Hemorrhagic Telangiectasia (HHT): 15 years of experience. Rhinology 2012;50:80–6CrossRefGoogle ScholarPubMed
23Boyer, H, Fernandes, P, Duran, O, Hunter, D, Goding, G. Office-based sclerotherapy for recurrent epistaxis due to hereditary hemorrhagic telangiectasia: a pilot study. Int Forum Allergy Rhinol 2011;1:319–23CrossRefGoogle ScholarPubMed
24Ghaheri, BA, Fong, KJ, Hwang, PH. The utility of bipolar electrocautery in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 2006;134:1006–9CrossRefGoogle ScholarPubMed
25Pagella, F, Matti, E, Chu, F, Pusateri, A, Tinelli, C, Olivieri, C et al. Argon plasma coagulation is an effective treatment for hereditary hemorrhagic telangiectasia patients with severe nosebleeds. Acta Otolaryngol 2013;133:174–80CrossRefGoogle ScholarPubMed
26Pagella, F, Semino, L, Olivieri, C, Corno, S, Dore, R, Draghi, F et al. Treatment of epistaxis in hereditary hemorrhagic telangiectasia patients by argon plasma coagulation with local anesthesia. Am J Rhinol 2006;20:421–5CrossRefGoogle ScholarPubMed
27Mortuaire, G, Boute, O, Hatron, PY, Chevalier, D. Pilot study of submucosal radiofrequency for epistaxis in hereditary hemorrhagic telangiectasia. Rhinology 2013;51:355–60CrossRefGoogle ScholarPubMed
28Joshi, H, Woodworth, BA, Carney, AS. Coblation for epistaxis management in patients with hereditary haemorrhagic telangiectasia: a multicentre case series. J Laryngol Otol 2011;125:1176–80CrossRefGoogle ScholarPubMed
29Harvey, RJ, Kanagalingam, J, Lund, VJ. The impact of septodermoplasty and potassium-titanyl-phosphate (KTP) laser therapy in the treatment of hereditary hemorrhagic telangiectasia-related epistaxis. Am J Rhinol 2008;22:182–7CrossRefGoogle ScholarPubMed
30Fiorella, ML, Lillo, L, Fiorella, R. Diode laser in the treatment of epistaxis in patients with hereditary haemorrhagic telangiectasia. Acta Otorhinolaryngol Ital 2012;32:164–9Google ScholarPubMed
31Karapantzos, I, Tsimpiris, N, Goulis, DG, Van Hoecke, H, Van Cauwenberge, P, Danielides, V. Management of epistaxis in hereditary hemorrhagic telangiectasia by Nd:YAG laser and quality of life assessment using the HR-QoL questionnaire. Eur Arch Otorhinolaryngol 2005;262:830–3CrossRefGoogle ScholarPubMed
32Lund, VJ, Howard, DJ. A treatment algorithm for the management of epistaxis in hereditary hemorrhagic telangiectasia. Am J Rhinol 1999;13:319–22CrossRefGoogle ScholarPubMed
33Haye, R, Austad, J. Hereditary hemorrhagic telangiectasia-argon laser. Rhinology 1991;29:59Google ScholarPubMed
34Bergler, W, Riedel, F, Baker-Schreyer, A, Juncker, C, Hörmann, K. Argon plasma coagulation for the treatment of hereditary hemorrhagic telangiectasia. Laryngoscope 1999;109:1520CrossRefGoogle ScholarPubMed
35Harries, PG, Brockbank, MJ, Shakespeare, PG, Carruth, JA. Treatment of hereditary haemorrhagic telangiectasia by the pulsed dye laser. J Laryngol Otol 1997;111:1038–41CrossRefGoogle ScholarPubMed
36Hartnick, CJ, Dailey, S, Franco, R, Zeitels, SM. Office-based pulsed dye laser treatment for hemorrhagic telangiectasias and epistaxis. Laryngoscope 2003;113:1085–7CrossRefGoogle ScholarPubMed
37Hitchings, AE, Lennox, PA, Lund, VJ, Howard, DJ. The effect of treatment for epistaxis secondary to hereditary hemorrhagic telangiectasia. Am J Rhinol 2005;19:75–8CrossRefGoogle ScholarPubMed
38Lesnik, GT, Ross, DA, Henderson, KJ, Joe, JK, Leder, SB, White, RI Jr.Septectomy and septal dermoplasty for the treatment of severe transfusion-dependent epistaxis in patients with hereditary hemorrhagic telangiectasia and septal perforation. Am J Rhinol 2007;21:312–5CrossRefGoogle ScholarPubMed
39Richer, SL, Geisthoff, UW, Livada, N, Ward, PD, Johnson, L, Mainka, A et al. The Young's procedure for severe epistaxis from hereditary hemorrhagic telangiectasia. Am J Rhinol 2012;26:401–4CrossRefGoogle ScholarPubMed
40Lund, VJ, Howard, DJ. Closure of the nasal cavities in the treatment of refractory hereditary haemorrhagic telangiectasia. J Laryngol Otol 1997;111:30–3CrossRefGoogle ScholarPubMed
41Gluckman, JL, Portugal, LG. Modified Youngs procedure for refractory epistaxis due to hereditary hemorrhagic telangiectasia. Laryngoscope 1994;104:1174–7CrossRefGoogle ScholarPubMed
42Hosni, A, A Innes, AJ. Hereditary hemorrhagic telangiectasia: Youngs procedure in the management of epistaxis. J Laryngol Otol 1994;108: 754–7CrossRefGoogle ScholarPubMed
43Strach, K, Schröck, A, Wilhelm, K, Greschus, S, Tschampa, H, Möhlenbruch, M, et al. Endovascular treatment of epistaxis: indications, management, and outcome. Cardiovasc Intervent Radiol 2011;34:1190–8CrossRefGoogle ScholarPubMed
44Trojanowski, P, Jargiello, T, Trojanowska, A, Klatka, J. Epistaxis in patients with hereditary hemorrhagic telangiectasia treated with selective arterial embolization Acta Radiol 2011;52:846–9CrossRefGoogle ScholarPubMed
45Woolford, TJ, Loke, D, Bateman, ND. The use of a nasal obturator in hereditary haemorrhagic telangiectasia: an alternative to Young's procedure. J Laryngol Otol 2002;116:455–6CrossRefGoogle ScholarPubMed
46Lobo, CJ, Hartley, C, Farrington, WT. Vestibular closure with a silastic obturator–an alternative to Young's procedure in bleeding diathesis. Rhinology 1999;37:131–2Google ScholarPubMed