Improvement in epistaxis management: the experience of a dedicated hereditary haemorrhagic telangiectasia clinic. Issue 3 (1st November 2021)

• Record Type: Journal Article
• Authors: Anning, Rebecca; Huang, Johnson; Ronan, Anne; de Malmanche, Jillian; Asher, Rebecca; Low, Tsu‐Hui (Hubert)
• Is Part Of: ANZ journal of surgery. Volume 92:Issue 3(2022)
• Page Start: 499
• Page End: 504
• Publication Date: 2021-11-01
• Subjects: arteriovenous malformation -- autosomal dominant -- epistaxis -- hereditary haemorrhagic telangiectasia -- Osler–Weber–Rendu -- otolaryngology head & neck surgery; Surgery -- Periodicals; 617.005
• DOI: 10.1111/ans.17322
• Languages: English
• ISSNs: 1445-1433

Abstract

Abstract: Background: Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic vascular disorder which is characterised by the development of arteriovenous malformations and telangiectasias. A key clinical manifestation is recurrent epistaxis. This study examined the impact of a dedicated HHT clinic in a major Australian tertiary hospital on epistaxis symptoms and subjective quality of life. Methods: A multidisciplinary HHT clinic was established in 2015. All patients satisfied either genetic diagnosis or fulfilled Curacao criteria. A protocol based clinical assessment was performed and a standardised treatment regimen was implemented. Patients completed quality of life and epistaxis severity score (ESS) questionnaires at each review. Results: Participants of the dedicated clinic included 21 females (58.3%) and 15 males (41.7%), with a mean age of 49.0 ± 24.0 years. The ACVRL1 variant was the most common ( n = 20, 55.6%). A statistically significant reduction in epistaxis severity was noted between the baseline and second review ( P = 0.02) and was maintained to the third visit ( P = 0.015). Patients older than 50 years demonstrate a consistently higher ESS than those less than 50 years old ( P = 0.03). This trend is noted throughout the follow up period with the dedicated clinic. Conclusion: The introduction of a multidisciplinary, dedicated HHT clinic to provide enhanced assessment, monitored treatment regimen and greater access to care, resulted in improvement in the management of epistaxis and quality of life in these patients. Abstract : Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic vascular disorder which is characterised by the development of arteriovenous malformations and telangiectasias. This article reports the benefits of a dedicated multidisciplinary clinic on outcomes including epistaxis severity and quality of life.