Update on Clinical Strategies in Hereditary Hemorrhagic Telangiectasia from an ENT Point of View

Abstract

Objectives: Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of vascular malformations with an absence of capillaries between arteries and veins. One major manifestation site is the nasal mucous membrane where recurrent nosebleeds occur. Our clinical strategy to treat patients with HHT has the aim to reduce nasal bleeding long-term with minimal local and general side effects.

Methods: We describe staged diagnosis and therapy including individual medical treatments of 97 patients with HHT. The success of treatment is monitored with a systematic questionnaire.

Results: The neodymium-doped yttrium aluminium garnet (Nd:YAG) laser therapy remains standard treatment of choice with no major side effects despite the need for repeated treatment. In addition new treatment strategies like nasal occlusion, local drug therapy, and nasal septal splinting show initial success.

Conclusion: Improvement of the quality of life of HHT patients can be achieved by a multimodal concept. Several new treatment strategies like nasal septal splinting and nasal occlusion successfully expand the range of established methods. Further studies have to prove the safety and long-term effectiveness of the described individual medical treatments.

Keywords: Arteriovenous Malformations; Bevacizumab; Epistaxis; Hereditary Hemorrhagic Telangiectasia; Nd:YAG Laser; Osler-Rendu-Weber Syndrome.

Fig. 1.

Comparison of answers to the hereditary hemorrhagic telangiectasia questionnaire of the latest 10 patients being treated 2–4 times with the neodymium-doped yttrium aluminium garnet (Nd:YAG) laser. VAS, visual analogue scale.

Fig. 2.

Comparison of answers to the hereditary hemorrhagic telangiectasia questionnaire of the latest 6 patients before and 2 to 3 months after receiving treatment after general anaesthesia including septal splints. VAS, visual analogue scale.