Hostname: page-component-84c44f86f4-r5dbb Total loading time: 0 Render date: 2025-10-14T09:46:59.826Z Has data issue: false hasContentIssue false

Is there still a role for nasal closure in hereditary haemorrhagic telangiectasia?

Published online by Cambridge University Press:  21 October 2024

Robert Bickerton*
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK
Benjamin Kennard
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK
Nikita Mehtani
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK
Elizabeth Bullock
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK
Talisa Ross
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK EvidENT Team, University College London, London, UK
Vikas Acharya
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK
Catherine Rennie
Affiliation:
Department of Ear, Nose and Throat Surgery, Imperial College Healthcare Trust, London, UK
*
Corresponding author: Robert Bickerton; Email: r.bickerton1@nhs.net

Abstract

Objective

Hereditary haemorrhagic telangiectasia (HHT) is characterised by recurrent, severe epistaxis. While nasal closure is a relatively well-established treatment for HHT patients with intractable epistaxis, recent studies highlight the efficacy of bevacizumab in this subgroup. We aim to evaluate the effectiveness of nasal closure for patients with contraindications to bevacizumab.

Methods

A case series of five patients with HHT and severe refractory transfusion-dependent epistaxis who were treated with nasal closure.

Results

All patients had subjective improvement in epistaxis. Haemoglobin concentrations increased in all patients, with none requiring transfusion for epistaxis post-operatively. Four patients experienced complete cessation in epistaxis. Four returned positive Glasgow Benefit Inventory scores.

Conclusion

Nasal closure appears to be a safe and effective option for the management of epistaxis in patients with severe, refractory HHT-related epistaxis. Treatment improved quality of life, reduced severity of epistaxis and increased haemoglobin concentrations. Nasal closure should be considered for HHT patients with severe, refractory epistaxis, particularly in cases where bevacizumab is contraindicated.

Information

Type
Main Article
Copyright
Copyright © The Author(s), 2024. Published by Cambridge University Press on behalf of J.L.O. (1984) LIMITED

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.)

Article purchase

Temporarily unavailable

Footnotes

Robert Bickerton takes responsibility for the integrity of the content of the paper

References

Shovlin, CL, Buscarini, E, Sabbà, C, Mager, HJ, Kjeldsen, AD, Pagella, F, et al. The European Rare Disease Network for HHT Frameworks for management of hereditary haemorrhagic telangiectasia in general and speciality care. Eur J Med Genet 2022;65:10437010.1016/j.ejmg.2021.104370CrossRefGoogle Scholar
McDonald, J, Stevenson, DA. Hereditary Hemorrhagic Telangiectasia. Seattle, Washington: University of Washington, 2021Google ScholarPubMed
Thalgott, JH, Dos-Santos-Luis, D, Hosman, AE, Martin, S, Lamandé, N, Bracquart, D, et al. Decreased expression of vascular endothelial growth factor receptor 1 contributes to the pathogenesis of hereditary hemorrhagic telangiectasia type 2. Circulation 2018;138:2698–71210.1161/CIRCULATIONAHA.117.033062CrossRefGoogle Scholar
AAssar, OS, Friedman, CM, White, RI Jr. The natural history of epistaxis in hereditary hemorrhagic telangiectasia. Laryngoscope 1991;101:977–8010.1288/00005537-199109000-00008CrossRefGoogle ScholarPubMed
McDonald, J, Bayrak-Toydemir, P, Pyeritz, RE. Hereditary hemorrhagic telangiectasia: An overview of diagnosis, management, and pathogenesis. Genet Med 2011;13:607–1610.1097/GIM.0b013e3182136d32CrossRefGoogle ScholarPubMed
McDonald, J, Bayrak-Toydemir, P, DeMille, D, Wooderchak-Donahue, W, Whitehead, K. Curaçao diagnostic criteria for hereditary hemorrhagic telangiectasia is highly predictive of a pathogenic variant in ENG or ACVRL1 (HHT1 and HHT2). Genet Med 2020;22:1201–510.1038/s41436-020-0775-8CrossRefGoogle ScholarPubMed
Whitehead, KJ, Sautter, NB, McWilliams, JP, Chakinala, MM, Merlo, CA, Johnson, MH, et al. Effect of topical intranasal therapy on epistaxis frequency in patients with hereditary hemorrhagic telangiectasia. JAMA 2016;316:943–5110.1001/jama.2016.11724CrossRefGoogle Scholar
Geisthoff, UW, Seyfert, UT, Kübler, M, Bieg, B, Plinkert, PK, König, J. Treatment of epistaxis in hereditary hemorrhagic telangiectasia with tranexamic acid - A double-blind placebo-controlled cross-over phase IIIB study. Thromb Res 2014;134:565–7110.1016/j.thromres.2014.06.012CrossRefGoogle ScholarPubMed
Gaillard, S, Dupuis-Girod, S, Boutitie, F, Rivière, S, Morinière, S, Hatron, P-Y, et al. Tranexamic acid for epistaxis in hereditary hemorrhagic telangiectasia patients: A European cross-over controlled trial in a rare disease. J Thromb Haemost 2014;12:1494–50210.1111/jth.12654CrossRefGoogle ScholarPubMed
Al-Samkari, H, Kasthuri, RS, Parambil, JG, Albitar, HA, Almodallal, YA, Vázquez, C, et al. An international, multicenter study of intravenous bevacizumab for bleeding in hereditary hemorrhagic telangiectasia: The InHIBIT-Bleed study. Haematologica 2020;106:2161–910.3324/haematol.2020.261859CrossRefGoogle Scholar
Chen, H, Zhang, Z, Chen, X, Wang, C, Chen, M, Liao, H, et al. Meta-analysis of efficacy and safety of bevacizumab in the treatment of hereditary hemorrhagic telangiectasia epistaxis. Front Pharmacol 2023;14:108984710.3389/fphar.2023.1089847CrossRefGoogle ScholarPubMed
Dupuis-Girod, S, Shovlin, CL, Kjeldsen, AD, Mager, H-J, Sabba, C, Droege, F, et al. European Reference Network for Rare Vascular Diseases (VASCERN): When and how to use intravenous bevacizumab in Hereditary Haemorrhagic Telangiectasia (HHT)? Eur J Med Genet 2022;65:10457510.1016/j.ejmg.2022.104575CrossRefGoogle Scholar
Buscarini, E, Botella, LM, Geisthoff, U, Kjeldsen, AD, Mager, HJ, Pagella, F, et al. Safety of thalidomide and bevacizumab in patients with hereditary hemorrhagic telangiectasia. Orphanet J Rare Dis 2019;14:2810.1186/s13023-018-0982-4CrossRefGoogle ScholarPubMed
Faughnan, ME, Mager, JJ, Hetts, SW, Palda, VA, Lang-Robertson, K, Buscarini, E, et al. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med 2020;173:989100110.7326/M20-1443CrossRefGoogle ScholarPubMed
Rimmer, J, Lund, VJ. A modified technique for septodermoplasty in hereditary hemorrhagic telangiectasia. Laryngoscope 2014;124:67–910.1002/lary.24303CrossRefGoogle ScholarPubMed
Young, A. Closure of the nostrils in atrophic rhinitis. J Laryngol Otol 1967;81:515–2410.1017/S0022215100067426CrossRefGoogle ScholarPubMed
Lund, VJ, Howard, DJ. Closure of the nasal cavities in the treatment of refractory hereditary haemorrhagic telangiectasia. J Laryngol Otol 1997;111:30–310.1017/S0022215100136369CrossRefGoogle ScholarPubMed
Gluckman, JL, Portugal, LG. Modified Young's procedure for refractory epistaxis due to hereditary hemorrhagic telangiectasia. Laryngoscope 1994;104:1174–710.1288/00005537-199409000-00023CrossRefGoogle ScholarPubMed
Lund, VJ, Darby, Y, Rimmer, J, Amin, M, Husain, S. Nasal closure for severe hereditary haemorrhagic telangiectasia in 100 patients. The Lund modification of the Young's procedure: A 22-year experience. Rhinology 2017;55:135–4110.4193/Rhin16.315CrossRefGoogle Scholar
Thomson, RS, Molin, NL, Whitehead, KJ, Ashby, S, Johnson, L, Ward, PD, et al. The effects of nasal closure on quality of life in patients with hereditary hemorrhagic telangiectasia. Laryngoscope Investig Otolaryngol 2018;3:178–8110.1002/lio2.157CrossRefGoogle ScholarPubMed
Andersen, JH, Kjeldsen, AD. Patient-recorded benefit from nasal closure in a Danish cohort of patients with hereditary haemorrhagic telangiectasia. Eur Arch Otorhinolaryngol 2020;277:79180010.1007/s00405-019-05758-1CrossRefGoogle Scholar
Richer, 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 Allergy 2012;26:401–410.2500/ajra.2012.26.3809CrossRefGoogle ScholarPubMed
Ichimura, K, Kikuchi, H, Imayoshi, S, Yamauchi, T, Ishikawa, K. Are patients with severe epistaxis caused by hereditary hemorrhagic telangiectasia satisfied with nostril closure surgery? Auris Nasus Larynx 2012;39:596410.1016/j.anl.2011.03.003CrossRefGoogle ScholarPubMed
Droege, F, Thangavelu, K, Stuck, BA, Stang, A, Lang, S, Geisthoff, U. Life expectancy and comorbidities in patients with hereditary hemorrhagic telangiectasia. Vasc Med 2018;23:377–8310.1177/1358863X18767761CrossRefGoogle ScholarPubMed
Nelson, EC, Eftimovska, E, Lind, C, Hager, A, Wasson, JH, Lindblad, S. Patient reported outcome measures in practice. BMJ 2015;350:781810.1136/bmj.g7818CrossRefGoogle ScholarPubMed
Kubba, H, Swan, IRC, Gatehouse, S. The Glasgow Children's Benefit Inventory: A new instrument for assessing health-related benefit after an intervention. Ann Otol Rhinol Laryngol 2004;113:980–610.1177/000348940411301208CrossRefGoogle ScholarPubMed
Hendry, J, Chin, A, Swan, IRC, Akeroyd, MA, Browdoining, GG. The Glasgow Benefit Inventory: A systematic review of the use and value of an otorhinolaryngological generic patient-recorded outcome measure. Clin Otolaryngol 2016;41:259–75CrossRefGoogle ScholarPubMed
Hoag, JB, Terry, P, Mitchell, S, Reh, D, Merlo, CA. An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope 2010;120:838–4310.1002/lary.20818CrossRefGoogle ScholarPubMed
Yin, LX, Reh, DD, Hoag, JB, Mitchell, SE, Mathai, SC, Robinson, GM, et al. The minimal important difference of the epistaxis severity score in hereditary hemorrhagic telangiectasia. Laryngoscope 2016;126:1029–3210.1002/lary.25669CrossRefGoogle ScholarPubMed