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Competing Interests: Interessenkonflikt: Urban Geisthoff erhielt ein Reisestipendium und ein Vortragshonorar von Cook Medical. Die Firmen Bess Medizintechnik GmbH (Berlin) und Cook Deutschland GmbH (Mönchengladbach) stellten Material und z.T. auch Personal (Bess) unterstützend zur Erstellung des Films kostenlos zur Verfügung.

Diagnosis and treatment of rare diseases are difficult. Hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber disorder) is a relatively common rare disease. Because epistaxis is the most frequent manifestation of this disease, affected individuals often approach otorhinolaryngologists first. The latter ones often have a key position for early diagnosis and screening for visceral involvement which can prevent life-threatening complications. Treatment of epistaxis can improve quality of life of affected persons. Diagnosis and treatment of rare diseases can be complex and it can be very helpful to use information resources to ensure a correct management. Various possibilities to access these resources are presented in general and in relation to HHT., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Epistaxis is the leading symptom of hereditary hemorrhagic teleangiectasia (HHT). Over the last years several laser systems have been used for therapy. Only a few studies have published results of Nd:YAG laser therapy evaluating a larger number of patients., Patients and Methods: From 1987 through 1996, forty-one patients with HHT were treated with a Nd:YAG laser due to recurrent epistaxis at the Department of Otorhinolaryngology of Kiel University. Thirty-two patients were followed-up over a period of at least 24 months. A centripetal technique was used for endonasal laser light application (15-25 Watts; 0.1-0.5 s), i.e., the laser light was applied from the periphery towards the center of the angiodysplasia. Intensive preoperative and postoperative nasal care with ointments was performed., Results: Bleeding frequency and intensity decreased in 30 (94%) patients. Duration of the therapeutical success differed individually and varied from seven weeks to 14 months. Repeated laser therapy was necessary in 27 of 32 patients., Conclusion: The Nd:YAG laser is a suitable tool in the treatment of epistaxis in HHT. This procedure is less traumatic, less painful, and can be repeated multiple times. Nd:YAG laser therapy and other therapeutic options cannot obtain a lasting cure of Rendu-Osler-Weber syndrome. However, in the vast majority of cases recurrent nasal bleeding as the dominant symptom can be reduced in frequency as well as in intensity.