The role of otorhinolaryngologists in the treatment of hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease which affects the entire vascular system. Symptoms vary from mild complaints to severe complications and are highly individual. Therapy is strictly symptomatic and creates an interdisciplinary challenge with relevant involvement of otorhinolaryngologists (ORL). The aim of this study is to evaluate this involvement. A retrospective analysis between 01/2006 and 12/2023 at Ulm University Medical Center was conducted. Two collectives were defined, the first one representing all patients who have been treated in any department for HHT, in order to draw a comparison between the departments. The second focused specifically on patients treated in the ORL department, providing a detailed analysis that includes emergency visits, hospital admissions, interventions and blood transfusions. In the 1st collective 252 patients were identified generating 1092 cases. 43.3% of the patients were primarily treated at the ORL department, 24.0% at the dermatology department and 13.1% in internal medicine. The ORL patients generated significantly more cases as other specialties (p = 0.0001). The 2nd collective consisted of 105 patients. Men were more likely to be hospitalized (p = 0.0001) and to receive blood transfusions (p = 0.0045). There were no other gender specific differences. Symptoms and the need for medical help were independent of the season. A vast variety of visits, admissions and interventions could be shown. ORL specialists play a critical role in the management of HHT patients. The study shows the year-round necessity for specialized care. Sex-related differences should be taken into account. Besides multidisciplinary therapy strategies further prospective research is essential.