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      Endoscopic surgical treatment of epistaxis in hereditary haemorrhagic telangiectasia: our experience Translated title: Il trattamento endoscopico dell’epistassi nella teleangectasia emorragica ereditaria: la nostra esperienza

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          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.

          RIASSUNTO

          Obiettivi

          L’obiettivo di questo studio è riportare la nostra esperienza nel trattamento endoscopico dell’epistassi nei pazienti con Teleangectasia Emorragica Ereditaria (HHT).

          Metodi

          Si tratta di uno studio longitudinale retrospettivo svolto presso l’UOC di Otorinolaringoiatria della Fondazione IRCCS Policlinico San Matteo di Pavia, centro di riferimento per la diagnosi e la cura dell’HHT. Sono stati valutati i pazienti sottoposti dal 1996 al 2015 a trattamento chirurgico delle epistassi ricorrenti, includendo solo quelli trattati con tecnica endoscopica.

          Risultati

          Dei 591 pazienti ricoverati e sottoposti a screening per HHT, 323 (54.7%) sono stati sottoposti a trattamento chirurgico endoscopico per epistassi, per un totale di 679 procedure. Il 77,2% delle procedure è stato eseguito in anestesia generale; il sistema ad Argon Plasma è stato lo strumento di scelta nella maggior parte dei pazienti, seguito dai laser e dalla quantum molecular resonance.

          Conclusioni

          Riportiamo una delle più ampie casistiche nel trattamento endoscopico dell’epistassi nei pazienti con HHT. Tale approccio consente di gestire i pazienti senza necessità di tamponamento nasale, è scevro da complicanze maggiori, ripetibile nel tempo e riteniamo che debba essere preso in considerazione come trattamento di prima scelta in caso di epistassi in pazienti con HHT.

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          Most cited references45

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          International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia.

          HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults. The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease. The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches. The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.
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            Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia

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              Tranexamic acid for epistaxis in hereditary hemorrhagic telangiectasia patients: a European cross-over controlled trial in a rare disease.

              Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder associated with abnormal angiogenesis and disabling epistaxis. Tranexamic acid (TA) has been widely used in the treatment of these severe bleeds but with no properly designed trial.
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                Author and article information

                Journal
                Acta Otorhinolaryngol Ital
                Acta Otorhinolaryngol Ital
                AOI
                Acta Otorhinolaryngologica Italica
                Pacini Editore Srl
                0392-100X
                1827-675X
                19 March 2021
                February 2021
                : 41
                : 1
                : 59-68
                Affiliations
                [1 ] Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo , Pavia, Italy
                [2 ] University of Pavia , Italy
                [3 ] Clinical Epidemiology and Biometric Unit, Fondazione I.R.C.C.S. Policlinico San Matteo , Pavia, Italy
                [4 ] General Biology and Medical Genetics , Department of Molecular Medicine, Pavia, Italy
                Author notes
                Correspondence Eugenia Maiorano Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, piazzale Golgi 19, 27100 Pavia, Italy E-mail: eugenia_maiorano@ 123456libero.it

                Funding

                None.

                Conflict of interest

                The Authors declare no conflict of interest.

                Article
                10.14639/0392-100X-N0915
                7982753
                33746224
                52a8251c-b693-481f-9617-11708eed1157
                Società Italiana di Otorinolaringoiatria e Chirurgia Cervico-Facciale, Rome, Italy

                This is an open access article distributed in accordance with the CC-BY-NC-ND (Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International) license. The article can be used by giving appropriate credit and mentioning the license, but only for non-commercial purposes and only in the original version. For further information: https://creativecommons.org/licenses/by-nc-nd/4.0/deed.en

                History
                : 10 June 2020
                : 16 October 2020
                Page count
                Figures: 5, Tables: 3, Equations: 0, References: 45, Pages: 10
                Categories
                Rhinology

                Otolaryngology
                hereditary haemorrhagic telangiectasia,rendu osler weber disease,epistaxis,nosebleeds,endoscopy,argon plasma coagulation,teleangectasia emorragica ereditaria,malattia di rendu osler weber,epistassi,endoscopia,argon plasma

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