What are the treatment options for severe epistaxis in Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
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Answer

In more severe cases, iron replacement may be indicated because of blood loss. Humidification of the ambient air helps decrease the amount of mucosal bleeding. Hormone therapy, including antiestrogen therapy with tamoxifen, for the treatment of epistaxis due to HHT has produced good responses, though its use remains controversial. [107] Antifibrinolytics (eg, aminocaproic acid) may be used to enhance hemostasis when fibrinolysis contributes to bleeding. [108]

Although estrogen-related hormones and antifibrinolytic agents are commonly used to manage bleeding, some studies suggest that this probably increases the risk of thrombotic events in OWRD patients with pulmonary AVMs. Accordingly, patients should undergo screening for the presence of pulmonary AVMs before treatment is started.

Pulsed dye laser treatment may be used to photocoagulate telangiectasias in the nasal mucosa. As many as three subsequent treatments may be necessary before any change in bleeding frequency or severity is observed.


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