Medical treatment of epistaxis in hereditary hemorrhagic telangiectasia: an evidence-based review. Int Forum Allergy Rhinol 2018 Jun;8(6):713-728
Date
02/03/2018Pubmed ID
29393992DOI
10.1002/alr.22094Scopus ID
2-s2.0-85041282427 (requires institutional sign-in at Scopus site) 13 CitationsAbstract
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder resulting in vascular dysplasia and formation of arteriovenous malformations. Recurrent epistaxis is a hallmark of the disease. An array of medical therapies are used in this patient population, but robust evidence-based recommendations regarding the medical treatment of epistaxis are lacking. This systematic review was performed to look at the current literature and make meaningful evidence-based recommendations.
METHODS: A search of the Ovid MEDLINE, Embase, and Cochrane databases was conducted by a research librarian. Abstracts in the English language and published in a peer-review journal were reviewed for relevance and inclusion. PRISMA guidelines were followed.
RESULTS: Eighteen studies met the inclusion criteria. In a few small studies, thalidomide was shown to consistently improve severity and frequency of epistaxis and improve hemoglobin concentrations while decreasing the need for transfusion. Tranexamic acid appeared to only impact the epistaxis severity score and not other clinical outcomes. Selective estrogen modulators (SERMs), propranolol, rose geranium oil, and N-acetylcysteine, have demonstrated promising efficacy in small trials.
CONCLUSION: Appropriate medical therapies for epistaxis outcomes in HHT remain undefined, and there is no "gold standard." Many of the studies are small and the data reported are heterogeneous, and therefore the ability to make strong evidence-based recommendations is limited. However, many different medications appear to be promising options.
Author List
Halderman AA, Ryan MW, Clark C, Sindwani R, Reh DD, Poetker DM, Invernizzi R, Marple BFAuthor
David M. Poetker MD Chief, Professor in the Otolaryngology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Administration, OralAdministration, Topical
Angiogenesis Inhibitors
Epistaxis
Estriol
Estrogen Replacement Therapy
Evidence-Based Medicine
Female
Humans
Male
Recurrence
Selective Estrogen Receptor Modulators
Telangiectasia, Hereditary Hemorrhagic
Thalidomide
Tranexamic Acid