Comprehensive management of hereditary hemorrhagic telangiectasia.

Abstract

PURPOSE OF REVIEW:

Hereditary hemorrhagic telangiectasia (HHT), or Osler Weber-Rendu disease, is a rare inherited disorder of fibrovascular tissue affecting various organs. Epistaxis is the most common symptom of HHT but as the disease affects multiple organs, a multisystem and multidisciplinary approach to management is required. The purpose of this article is to provide an overview of the multidisciplinary approach to HHT for the otolaryngologist and to discuss the current pharmacologic and procedural treatment options available for HHT-related epistaxis.

RECENT FINDINGS:

Multidisciplinary expert guidelines have better defined what screening tests are advised for the multisystem evaluation of the HHT patient. New pharmacologic therapies including bevacizumab (Avastin) used submucosally or topically have shown promise as in-office treatment modalities. Sclerotherapy of telangiectasia, including in-office applications, has recently proven safe and effective.

SUMMARY:

HHT remains a difficult disease to treat. Being aware of the common organ systems involved by the disease will help the practicing otolaryngologist to ensure the patient receives appropriate multidisciplinary care. For HHT-related epistaxis, new medical and surgical options allow for a wider range of treatments than were previously available.

• Authors: Michael D Lupa, Sarah K Wise
• Journal: Current opinion in otolaryngology & head and neck surgery (Curr Opin Otolaryngol Head Neck Surg) Vol. 25 Issue 1 Pg. 64-68 (Feb 2017) ISSN: 1531-6998 [Electronic] United States
• PMID: 27841771 (Publication Type: Journal Article, Review)

Topics

• Combined Modality Therapy (methods)
• Comprehensive Health Care (methods)
• Disease Management
• Disease Progression
• Epistaxis (physiopathology, therapy)
• Female
• Humans
• Intracranial Arteriovenous Malformations (physiopathology, therapy)
• Laser Coagulation (methods)
• Lung Diseases (physiopathology, therapy)
• Male
• Multimorbidity
• Prognosis
• Rare Diseases
• Risk Assessment
• Telangiectasia, Hereditary Hemorrhagic (genetics, physiopathology, therapy)
• Treatment Outcome