Abstract
Hereditary haemorrhagic teleangiectasia (HHT) is most notably characterized by vulnerable vascular formations of the nasal superficial mucosa. Epistaxis is one of the most common symptoms of the afflicted patients, with an incidence of more than 90 %. A variable series of treatments have been described, ranging from nasal ointments to the complete surgical occlusion of the nose. The objective of this pilot study is the presentation of first experiences in treating patients suffering from HHT and chronically recurrent epistaxis with an individual nasal olive made from silicone. Eleven patients (six men, five women) aging from 44 to 80 years with known HHT were treated at the ENT department of Homburg/Saar between October 2008 and July 2012 because of nasal bleeding by Nd:YAG laser or argon plasma coagulation. After the surgical treatment, an imprint of the nasal aditus was taken to manufacture an individual custom-made silicone nasal olive. Patients were wearing the nasal olive for 3–8 h a day. Check-ups were made every 6 months. Epistaxis severity score (ESS) was used pre- and post-nasal olive application. The observation period was 12–48 months. The utilization of the silicone nasal olive led to a distinct reduction of epistaxis events. Apart from the nasal olive, our patients needed no further treatment of the nose during the observation period except for a nasal ointment. Insertion and removal of the nasal olive were handled by the patients themselves. The local manipulation in handling the nasal olive caused no epistaxis itself. A significant improvement of the ESS and satisfaction was reported in all patients. Use of an individually manufactured silicone nasal olive is a promising extension to the established treatments of epistaxis in HHT patients. Tolerance towards this treatment by the patients was high due to the low personal burden and encumbrance. The extended use of the presented method in HHT patients may be beneficial. However, a more prolonged observation period is necessary in the future to judge the long-term efficiency of individual nasal olives.
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References
Rendu M (1896) Épistaxis répétées chez un sujet porteur de petits angiomes cutanés et muqueux. Bull Mém Soc Méd Hôp Paris 13:731–733
Osler W (1901) On a family form of recurring epistaxis, associated with multiple telangiectases of the skin and mucous membranes. Johns Hopkins Hops Bull 12:333–337
Weber FP (1907) Multiple hereditary developmental angiomata (telangiectases) of the skin and mucous membranes associated with recurring haemorrhages. Lancet 2:160–162
Hanes FM (1907) Multiple hereditary telangiectases cause hemorrhage (hereditary hemorrhagic telangiectasia). Johns Hopkins Hosp Bull 20:63–73
Parkin JL, Dixon JA (1985) Argon laser treatment of head and neck vascular lesions. Otolaryngol Head Neck Surg 93:211–216
Shapsay SM (1984) Oliver P treatment of hereditary hemorrhagic telangiectasia by Nd-YAG laser photocoagulation. Laryngoscope 94:1554–1556
Werner JA, Lippert BM, Geisthoff UW, Rudert H (1997) Nd:YAG laser therapy of recurrent epistaxis in hereditary hemorrhagic telangiectasia. Laryngorhinootologie 76:495–501 (Article in German)
Shah RK, Dhingra JK, Shapshay SM (2002) Hereditary hemorrhagic telangiectasia: a review of 76 cases. Laryngoscope 112:767–773
Byahatti SV, Rebeiz EE, Shapshay SM (1997) Hereditary hemorrhagic telangiectasia: what the otolaryngologist should know. Am J Rhinol 11:55–62
Vickery CL, Kuhn FA (1996) Using the KTP/532 laser to control epistaxis in patients with hereditary hemorrhagic telangiectasia. South Med J 89:78–80
Karapantzos I, Tsimpiris N, Goulis DG, Van Hoecke H, Van Cauwenberge P, Danielides V (2005) Management of epistaxis in hereditary hemorrhagic telangiectasia by Nd:yAG laser and quality of life assessment using the HR-QoL questionnaire. Eur Arch Otorhinolaryngol 262:830–833 (Epub 2005 Mar 1)
Kardos TB, Holt T, Ferguson MM (1989) Histological evaluation of the effect of a miniature carbon dioxide laser on oral mucosa. Int J Oral Maxillofac Surg 18:117–120
Bergler W, Götte K, Riedel F, Back W, Hörmann K (1998) Argon plasma coagulation in treatment of hereditary hemorrhagic telangiectasia of the nasal mucosa. HNO 46:228–232 (Article in German)
Saunders WH (1960) Septal dermoplasty for control of nosebleeds caused by hereditary hemorrhagic telangiectasia or septal perforations. Trans Am Acad Ophthalmol Otolaryngol 64:500–506
McCaffrey TV, Kern EB, Lake CF (1977) Management of epistaxis in hereditary hemorrhagic telangiectasia. Review of 80 cases. Arch Otolaryngol 103:627–630
Fiorella ML, Ross D, Henderson KJ, White RI Jr (2005) Outcome of septal dermoplasty in patients with hereditary hemorrhagic telangiectasia. Laryngoscope 115:301–305
Levine CG, Ross DA, Henderson KJ, Leder SB, White RI Jr (2008) Long-term complications of septal dermoplasty in patients with hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 138:721–724
Young A (1967) Closure of the nostrils in atrophic rhinitis. J Laryngol Otol 81:515–524
Brooker DS, Cinnamond MJ (1991) Young’s procedure in the treatment of epistaxis. J Laryngol Otol 105:847–848
Gluckman JL, Portugal LG (1994) Modified Young’s procedure for refractory epistaxis due to hereditary hemorrhagic telangiectasia. Laryngoscope 104:1174–1177
Lund V, Howard DJ (1997) Closure of the nasal cavities in the treatment of refractory hereditary haemorrhagic telangiectasia. J Laryngology and Otology J Laryngol Otol 111:30–33
Pau H, Carney AS, Murty GE (2001) Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): otorhinolaryngological manifestations. Clin Otolaryngol Allied Sci 26:93–98
Strother CM, Newton TH (1976) Percutaneous embolization to control epistaxis in Rendu-Osler-Weber disease. Arch Otolaryngol 102:58–60
Weissman JL, Jungreis CA, Johnson JT (1995) Therapeutic embolization for control of epistaxis in a patient with hereditary hemorrhagic telangiectasia. Am J Otolaryngol 16:138–140
Harrison DFN (1982) Use of estrogen in treatment of familiar hemorrhagic teleangiectasia. Laryngoscope 92:314–319
Sadick H, Riedel F, Oulmi J, Hörmann K, Bergler WF (2003) Argon plasma coagulation and topically applied estriol. Long-term results in the treatment of hereditary hemorrhagic telangiectasia of the nasal mucosa. HNO 51:118–124 (Article in German)
Vase P (1981) Estrogen treatment of hereditary hemorrhagic telangiectasia. A double-blind controlled clinical trial. Acta Med Scand 209:393–396
Pizzi G, Turcato G, Polico R, Busetto M, Antonello M, Princivalli M (1995) Brachytherapy of epistaxis in Rendu-Osler disease. Indications, technic, results. Radiol Med 89:861–864 Article in Italian
Folz BJ, Zoll B, Alfke H, Toussaint A, Maier RF, Werner JA (2006) Manifestations of hereditary hemorrhagic telangiectasia in children and adolescents. Eur Arch Otorhinolaryngol 263(1):53–61 Epub 2005 Jun 24
Folz BJ, Wollstein AC, Alfke H, Dünne AA, Lippert BM, Görg K, Wagner HJ, Bien S, Werner JA (2004) The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study. Eur Arch Otorhinolaryngol 261(9):509–516 (Epub 2003 Dec 17)
Dupuis-Girod S, Ginon I, Saurin JC, Marion D, Guillot E, Decullier E, Roux A, Carette MF, Gilbert-Dussardier B, Hatron PY, Lacombe P, Lorcerie B, Rivière S, Corre R, Giraud S, Bailly S, Paintaud G, Ternant D, Valette PJ, Plauchu H, Faure F (2012) Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA 307:948–955
Guldmann R, Dupret A, Nivoix Y, Schultz P, Debry C (2012) Bevacizumab nasal spray: noninvasive treatment of epistaxis in patients with Rendu-Osler disease. Laryngoscope 122:953–955
Hoag JB, Terry P, Mitchell S, Reh D, Merlo CA (2010) An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope 120:838–843
Folz BJ, Lippert BM, Wollstein AC, Tennie J, Happle R, Werner JA (2004) Mucocutaneous telangiectases of the head and neck in individuals with hereditary hemorrhagic telangiectasia—analysis of distribution and symptoms. Eur J Dermatol. 14:407–411
Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ, Kjeldsen AD, Plauchu H (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 91:66–67
Al Kadah B, Bumm K, Wolf G, Schneider M, Schick B (2011) A new innovative approach in treatment of hereditary haemorrhagic teleangiectasia (HHT): a nasal olive. GMS Curr Posters Otorhinolaryngol Head Neck Surg 7:Doc61
Folz BJ, Chirtesiu AM, Konnerth CG (2013) Customized nasal breathing tubes as an alternative to Young’s procedure in HHT patients with epistaxis Preliminary results. Hematology Reports Volume 3(suppl 1):137
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The authors would like to acknowledge the worthful contribution of Dietmar Hecker in performing the statistical analysis.
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Al Kadah, B., Papaspyrou, G., Schneider, M. et al. First experiences with an individual nasal olive in patients with hereditary haemorrhagic telangiectasia (HHT). Eur Arch Otorhinolaryngol 272, 117–122 (2015). https://doi.org/10.1007/s00405-014-3086-3
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DOI: https://doi.org/10.1007/s00405-014-3086-3