Hereditary hemorrhagic telangiectasia-related epistaxis

Innovations in understanding and management

Nathan Sautter, Timothy Smith

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Background: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT), affecting approximately 90% of patients at some point during their lifetime. Bleeding is chronic and varies from mild, self-limited episodes to severe, transfusion-dependent or life-threatening epistaxis. Treatment options vary from conservative, nonsurgical management to more aggressive surgical approaches. A number of treatment options have been introduced in recent years. There is little consensus in the literature regarding treatment algorithms. The objective of this investigation was to provide a contemporary review of HHT-related epistaxis, including pathophysiology, disease manifestations, and state-of-the-art treatment modalities. Methods: A systematic review of the literature for HHT-related epistaxis was performed using the search terms "hereditary hemorrhagic telangiectasia" and "epistaxis." Additional literature search regarding current recommendations for HHT evaluation and recent developments in genetic mechanisms, pathophysiology, and treatment of HHT was also performed. Results: A total of 308 articles were identified and reviewed for appropriateness of inclusion whereas 64 articles met inclusion criteria. Treatment options range from topical and hormonal therapy to more aggressive surgical modalities. Most treatment descriptions are case series, with few randomized controlled trials. A number of new and novel therapies have been introduced in recent years. Conclusion: HHT is a heterogeneous disease requiring multidisciplinary evaluation and treatment. Therapeutic options for HHT-related epistaxis vary from conservative, nonsurgical measures to more aggressive surgical treatments. A graduated treatment plan is recommended. Patients present with a wide degree in variation of severity of epistaxis, and treatment is best tailored to the individual patient.

Original languageEnglish (US)
Pages (from-to)422-431
Number of pages10
JournalInternational Forum of Allergy and Rhinology
Volume2
Issue number5
DOIs
StatePublished - Sep 2012

Fingerprint

Hereditary Hemorrhagic Telangiectasia
Epistaxis
Therapeutics

Keywords

  • Chronic disease
  • Epistaxis
  • Hemorrhagic disorders
  • Hereditary
  • Review
  • Therapeutics

ASJC Scopus subject areas

  • Immunology and Allergy
  • Otorhinolaryngology

Cite this

Hereditary hemorrhagic telangiectasia-related epistaxis : Innovations in understanding and management. / Sautter, Nathan; Smith, Timothy.

In: International Forum of Allergy and Rhinology, Vol. 2, No. 5, 09.2012, p. 422-431.

Research output: Contribution to journalArticle

@article{3e5ecc5aa1754b4caa2bb7779ec8e142,
title = "Hereditary hemorrhagic telangiectasia-related epistaxis: Innovations in understanding and management",
abstract = "Background: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT), affecting approximately 90{\%} of patients at some point during their lifetime. Bleeding is chronic and varies from mild, self-limited episodes to severe, transfusion-dependent or life-threatening epistaxis. Treatment options vary from conservative, nonsurgical management to more aggressive surgical approaches. A number of treatment options have been introduced in recent years. There is little consensus in the literature regarding treatment algorithms. The objective of this investigation was to provide a contemporary review of HHT-related epistaxis, including pathophysiology, disease manifestations, and state-of-the-art treatment modalities. Methods: A systematic review of the literature for HHT-related epistaxis was performed using the search terms {"}hereditary hemorrhagic telangiectasia{"} and {"}epistaxis.{"} Additional literature search regarding current recommendations for HHT evaluation and recent developments in genetic mechanisms, pathophysiology, and treatment of HHT was also performed. Results: A total of 308 articles were identified and reviewed for appropriateness of inclusion whereas 64 articles met inclusion criteria. Treatment options range from topical and hormonal therapy to more aggressive surgical modalities. Most treatment descriptions are case series, with few randomized controlled trials. A number of new and novel therapies have been introduced in recent years. Conclusion: HHT is a heterogeneous disease requiring multidisciplinary evaluation and treatment. Therapeutic options for HHT-related epistaxis vary from conservative, nonsurgical measures to more aggressive surgical treatments. A graduated treatment plan is recommended. Patients present with a wide degree in variation of severity of epistaxis, and treatment is best tailored to the individual patient.",
keywords = "Chronic disease, Epistaxis, Hemorrhagic disorders, Hereditary, Review, Therapeutics",
author = "Nathan Sautter and Timothy Smith",
year = "2012",
month = "9",
doi = "10.1002/alr.21046",
language = "English (US)",
volume = "2",
pages = "422--431",
journal = "International Forum of Allergy and Rhinology",
issn = "2042-6976",
publisher = "Wiley-Blackwell",
number = "5",

}

TY - JOUR

T1 - Hereditary hemorrhagic telangiectasia-related epistaxis

T2 - Innovations in understanding and management

AU - Sautter, Nathan

AU - Smith, Timothy

PY - 2012/9

Y1 - 2012/9

N2 - Background: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT), affecting approximately 90% of patients at some point during their lifetime. Bleeding is chronic and varies from mild, self-limited episodes to severe, transfusion-dependent or life-threatening epistaxis. Treatment options vary from conservative, nonsurgical management to more aggressive surgical approaches. A number of treatment options have been introduced in recent years. There is little consensus in the literature regarding treatment algorithms. The objective of this investigation was to provide a contemporary review of HHT-related epistaxis, including pathophysiology, disease manifestations, and state-of-the-art treatment modalities. Methods: A systematic review of the literature for HHT-related epistaxis was performed using the search terms "hereditary hemorrhagic telangiectasia" and "epistaxis." Additional literature search regarding current recommendations for HHT evaluation and recent developments in genetic mechanisms, pathophysiology, and treatment of HHT was also performed. Results: A total of 308 articles were identified and reviewed for appropriateness of inclusion whereas 64 articles met inclusion criteria. Treatment options range from topical and hormonal therapy to more aggressive surgical modalities. Most treatment descriptions are case series, with few randomized controlled trials. A number of new and novel therapies have been introduced in recent years. Conclusion: HHT is a heterogeneous disease requiring multidisciplinary evaluation and treatment. Therapeutic options for HHT-related epistaxis vary from conservative, nonsurgical measures to more aggressive surgical treatments. A graduated treatment plan is recommended. Patients present with a wide degree in variation of severity of epistaxis, and treatment is best tailored to the individual patient.

AB - Background: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT), affecting approximately 90% of patients at some point during their lifetime. Bleeding is chronic and varies from mild, self-limited episodes to severe, transfusion-dependent or life-threatening epistaxis. Treatment options vary from conservative, nonsurgical management to more aggressive surgical approaches. A number of treatment options have been introduced in recent years. There is little consensus in the literature regarding treatment algorithms. The objective of this investigation was to provide a contemporary review of HHT-related epistaxis, including pathophysiology, disease manifestations, and state-of-the-art treatment modalities. Methods: A systematic review of the literature for HHT-related epistaxis was performed using the search terms "hereditary hemorrhagic telangiectasia" and "epistaxis." Additional literature search regarding current recommendations for HHT evaluation and recent developments in genetic mechanisms, pathophysiology, and treatment of HHT was also performed. Results: A total of 308 articles were identified and reviewed for appropriateness of inclusion whereas 64 articles met inclusion criteria. Treatment options range from topical and hormonal therapy to more aggressive surgical modalities. Most treatment descriptions are case series, with few randomized controlled trials. A number of new and novel therapies have been introduced in recent years. Conclusion: HHT is a heterogeneous disease requiring multidisciplinary evaluation and treatment. Therapeutic options for HHT-related epistaxis vary from conservative, nonsurgical measures to more aggressive surgical treatments. A graduated treatment plan is recommended. Patients present with a wide degree in variation of severity of epistaxis, and treatment is best tailored to the individual patient.

KW - Chronic disease

KW - Epistaxis

KW - Hemorrhagic disorders

KW - Hereditary

KW - Review

KW - Therapeutics

UR - http://www.scopus.com/inward/record.url?scp=84867621226&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84867621226&partnerID=8YFLogxK

U2 - 10.1002/alr.21046

DO - 10.1002/alr.21046

M3 - Article

VL - 2

SP - 422

EP - 431

JO - International Forum of Allergy and Rhinology

JF - International Forum of Allergy and Rhinology

SN - 2042-6976

IS - 5

ER -