Treatment of Hereditary Hemorrhagic Telangiectasia-Related Epistaxis

Otolaryngol Clin North Am. 2016 Jun;49(3):639-54. doi: 10.1016/j.otc.2016.02.010.

Abstract

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an incidence of 1:5000. Recurrent, spontaneous epistaxis is the most common presenting symptom. Severity of epistaxis varies widely, from mild, self-limited nosebleeds to severe, life-threatening nasal hemorrhage. Treatment of HHT-related epistaxis presents a challenge to the otolaryngologist due to the recurrent, persistent nature of epistaxis often requiring multiple treatments. Treatment modalities range from conservative topical therapies to more aggressive surgical treatments.

Keywords: Bevacizumab; Epistaxis; Hereditary hemorrhagic telangiectasia (HHT); Laser photocoagulation; Osler-Weber-Rendu; Septodermoplasty; Young’s procedure.

Publication types

  • Review

MeSH terms

  • Combined Modality Therapy / methods
  • Endoscopy* / adverse effects
  • Endoscopy* / methods
  • Epistaxis / surgery*
  • Hemostasis, Surgical / methods*
  • Hemostatic Techniques*
  • Hemostatics / pharmacology*
  • Humans
  • Outcome and Process Assessment, Health Care
  • Secondary Prevention / methods
  • Severity of Illness Index
  • Telangiectasia, Hereditary Hemorrhagic / complications*

Substances

  • Hemostatics