SUMMARY Objectives Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disease characterised by epistaxis. Surgical procedures for epistaxis vary from diathermocoagulation to nasal closure. The aim of this paper is to report our experience in endoscopic surgical management of epistaxis in HHT patients. Methods This is a descriptive, longitudinal study carried out at the Otorhinolaryngology Department of IRCCS Policlinico San Matteo in Pavia, a reference centre for the treatment and diagnosis of HHT. We retrospectively evaluated HHT patients who underwent surgery for epistaxis from 1996 to 2015, including only those treated with endoscopic surgery. Results Among the 591 patients hospitalised and screened for HHT, 323 (54.7%) underwent endoscopic surgery for epistaxis, for a total of 679 procedures. General anaesthesia was used in 77.2% of procedures; argon plasma coagulation was the instrument of choice in the majority of patients, followed by lasers and quantum molecular resonance technology. Conclusions We report one of the largest cohorts undergoing endoscopic treatment of epistaxis in HHT patients. This mini-invasive surgical treatment allowed us to control epistaxis without major complications and nasal packaging and can be repeated over time. For these reasons, we recommend it as first choice in case of epistaxis in HHT patients.

Clivus pathologies from diagnosis to surgical multidisciplinary treatment. Review of the literature Patologie del clivus dalla diagnosi al trattamento chirurgico multidisciplinare. Revisione della letteratura

Question 1Is it necessary to distinguish between congenital medial capillary macula and lateral congenital capillary macula? Recommendation of good clinical practice, based on the experience of the panel: GGP grade. It is no supported by EtiologyMost VM are sporadic. However, inherited forms with autosomal dominant inheritance have been reported.