Clinical Strategy in Hereditary Hemorrhagic Telangiectasia

Kühnel, Thomas; Wagner, Birgit H; Schurr, Christian; Strutz, Jürgen

doi:10.1177/194589240501900515

Cited by 21 publications

(19 citation statements)

References 13 publications

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“…1,9 This procedure has a high complication rate and is contraindicated in anterior ethmoidal artery bleeding. 7,9 Furthermore, HHT patients require more frequent re-embolizations, since they often present an anterior epistaxis of the Kiesselbach's plexus (in contrast with refractory epistaxis due to other causes, which most frequently originates from branches of the sphenopalatine artery).…”

Section: 49mentioning

confidence: 99%

“…2,4,5 Epistaxis is the most common and earliest symptom. 1,[4][5][6][9][10][11] It is usually mild to moderate, however, in some patients it can be severe leading to significant impact on quality of life and requiring regular blood transfusions. 1,4,10,11 Although there is no definitive treatment, various treatments for epistaxis in HHT have been proposed.…”

Section: Introductionmentioning

confidence: 99%

“…1,[4][5][6][9][10][11] It is usually mild to moderate, however, in some patients it can be severe leading to significant impact on quality of life and requiring regular blood transfusions. 1,4,10,11 Although there is no definitive treatment, various treatments for epistaxis in HHT have been proposed. [2][3][4]7,10,11 This paper reports a case of a patient with HHT, in which an episode of refractory epistaxis was successfully treated with radiofrequency.…”

Section: Introductionmentioning

confidence: 99%

“…It is a disease with equal gender prevalence and has its highest prevalence in Curacao Island (Netherlands Antilles) in the southern Caribbean. [1][2][3][4][5][6][7][8] The Curacao criteria establish a definite diagnosis of HHT when three of the following four manifestations are found: recurrent epistaxis, mucocutaneous telangiectasia, arteriovenous malformations and positive family history. 2,4,5 Epistaxis is the most common and earliest symptom.…”

Section: Introductionmentioning

confidence: 99%

“…They turn the nasal cavity into a moist chamber, which has a positive impact on the frequency of epistaxis. 1,9 The improvement of protection group includes septodermoplasty and sexual hormone therapy. Septodermoplasty (Saunders' procedure), involves the replacement of the anterior part of the septal mucosa (with preservation of the underlying perichondrium) by a free skin graft.…”

mentioning

confidence: 99%

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Radiofrequency for Treatment of Refractory Epistaxis in Hereditary Hemorrhagic Telangiectasia

et al. 2018

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Hereditary hemorrhagic telangiectasia is a rare multi-systemic autosomal dominant disorder characterized by dysplasia of the vascular connective tissue and recurrent bleeding tendency. Epistaxis is the most common and earliest symptom. It is usually mild to moderate, however, in some patients it can be severe with significant interference in quality of life. We report a case of a patient with hereditary hemorrhagic telangiectasia, in which an episode of refractory epistaxis was treated with radiofrequency and fibrin sealant. Control of acute bleeding and elimination of telangiectasia was possible with this technique. After six months follow-up, there was no epistaxis recurrence. Various treatments for epistaxis in this disease have been described in the literature, but there is no consensus about the gold-standard procedure. Radiofrequency ablation of telangiectasia is a recent technique that has shown to be safe, effective and well tolerated, even in patients who underwent other previous treatments. Keywords: Epistaxis; Radiofrequency; Telangiectasia, Hereditary Hemorrhagic RESUMO A telangiectasia hemorrágica hereditária é uma doença autossómica dominante, multissistémica e rara, caracterizada pela displasia do tecido conectivo vascular com tendência à hemorragia recorrente. O sintoma mais comum e precoce é a epistaxis. Habitualmente é ligeira a moderada, no entanto, em alguns doentes, pode ser severa com interferência significativa na sua qualidade de vida. É reportado o caso de um doente com telangiectasia hemorrágica hereditária, em que um episódio de epistaxis refractária foi tratado com radiofrequência e um quelante de fibrina. Com esta técnica foi conseguido não só o controlo da hemorragia activa, mas também a eliminação das telangiectasias. Após seis meses de seguimento, não houve recorrência da epistaxis. Há vários tratamentos descritos na literatura para a epistaxis nesta doença, não havendo no entanto, consenso acerca do gold standard. A ablação com radiofrequência das telangiectasias é uma técnica recente que se tem mostrado segura, eficaz e bem tolerada, mesmo em doentes submetidos previamente a outros tratamentos.

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