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Nosebleeds (epistaxis) are usually minor. Medical intervention is only necessary in about 6% of cases. The source of bleeding is frequently located in the anterior region of the nose (Kiesselbach's plexus). The estimated lifetime prevalence of epistaxis is 60%. Diffuse epistaxis is often a manifestation of systemic disease. Epistaxis is the leading symptom of Rendu-Osler-Weber disease (hereditary hemorrhagic telangiectasia, HHT). If intervention is required, the first-choice of treatment is bidigital compression for several minutes. Common therapeutic measures include local hemostasis using electrocoagulation or chemical agents, e.g., silver nitrate. Resorbable anterior nasal tampons or tampons with a smooth surface are also frequently employed. In case of failed surgical closure of the sphenopalatine artery, angiographic embolization is the method of choice.Meist handelt es sich beim Nasenbluten (Epistaxis) um kleinere Blutungen. Medizinische Maßnahmen sind nur in etwa 6 % der Fälle erforderlich. Oft liegt die Blutungsquelle im vorderen Nasenabschnitt (Locus Kiesselbachii). Die geschätzte Gesamtlebenszeitprävalenz für Epistaxis beträgt 60 %. Tritt die Epistaxis in diffuser Form auf, so ist das häufig die Manifestation einer systemischen Erkrankung. Beim M. Rendu-Osler-Weber (hereditäre hämorrhagische Teleangiektasie, HHT) stellt die Epistaxis das Leitsymptom dar. Erstmaßnahme der Wahl bei Interventionsbedarf ist die bidigitale Kompression über mehrere Minuten. Verbreitete Therapiemaßnahmen sind u. a. die lokale Blutstillung mittels Elektrokoagulation oder mit chemischen Mitteln wie Silbernitrat. Auch resorbierbare vordere Nasentamponaden oder Tamponaden mit glatter Oberfläche werden häufig eingesetzt. Beim Versagen des chirurgischen Gefäßverschlusses der A. sphenopalatina ist die angiographische Embolisation Methode der Wahl.

[Background] Hereditary hemorrhagic telangiectasia (HHT) is a multisystemic inherited vascular dysplasia that leads to nosebleeds and visceral arteriovenous malformations (AVMs). Anti-angiogenic drugs thalidomide and bevacizumab have been increasingly used off-label with variable results. The HHT working group within the ERN for Rare Multisystemic Vascular Diseases (VASCERN), developed a questionnaire-based retrospective capture of adverse events (AEs) classified using the Common Terminology Criteria for Adverse Events., [Results] Sixty-nine HHT patients received bevacizumab, 37 (50.6%) for high output cardiac failure/hepatic AVMs, and 32 (49.4%) for bleeding; the 69 patients received bevacizumab for a mean of 11 months for a total of 63.8 person/years treatment. 67 received thalidomide, all for epistaxis and/or gastrointestinal bleeding; they received thalidomide for a mean of 13.4 months/patient for a total of 75 person/years treatment. AEs were reported in 58 patients, 33 with bevacizumab, 37 with thalidomide. 32 grade 1–3 AEs related to bevacizumab were reported with an average incidence rate of 50 per 100 person-years. 34 grade 1–3 AEs related to thalidomide were reported with an average incidence rate of 45.3 per 100 person-years. Bevacizumab AEs were more common in females (27 AEs in 46 women) than males (6 in 23, p, [Conclusions] With potential increase in use of Bevacizumab and Thalidomide in HHT patients, data presented support appropriate weighing of the toxicities which can arise in HHT settings and the practice recommendations for their prevention and management.

Hereditary hemorrhagic teleangiectasia (HHT or Rendu-Osler-Weber Syndrome) is an inherited autosomal dominant disorder of the vascular connective tissue. The resulting vascular malformations can occur in virtually any organ. Nosebleeds can massively impact on the quality of life of those afflicted. However, visceral manifestations are likely to be more serious, and may be comparable with a "ticking time bomb". Most commonly affected are the vascular systems of the lungs, liver, brain and gastrointestinal tract. Screening is recommended--at least with regard to the lungs. Difficult constellations of this complex condition may be successfully managed by an interdisciplinary approach.