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There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis.
Method:
The Medline and Embase databases were interrogated on 15 November 2013 using the search items ‘hereditary haemorrhagic telangiectasia’ (title), ‘epistaxis’ (title) and ‘treatment’ (title and abstract), and limiting the search to articles published in English.
Results:
A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3 in vitro studies.
Conclusion:
There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by dermal, mucosal, and visceral telangiectases as well as pulmonary and cerebral arteriovenous malformations. Recurrent epistaxis occurs in the majority of patients, and by the very nature of the thin walled vessels involved it is often refractory to conventional forms of treatment. We present the case of an 82-year-old lady with intractable epistaxis secondary to HHT, that was successfully controlled by the application of fibrin glue.
Two cases of hereditary haemorrhagic telangiectasia are presented. Epistaxis was intractable in both patients. Young's procedure proved successful in controlling nose bleeds. One of the patients has been followed-up for 14 years. Methods of treatment are discussed with a review of the literature.
A new technique for the treatment of severe epistaxis associated with hereditary haemorrhagic telangiectasia is described. The nasal septum and inferior turbinates, surgically denuded of respiratory epithelium, were grafted using autografts of cultured epithelial sheets derived from buccal epithelium. All patients upon whom this technique has been used have shown considerable lessening in the frequency and severity of their epistaxes although two patients received grafts on two occasions, in each case approximately three months apart. It is postulated that a nasal lining of stratified squamous epithelium is likely to be more resistant to trauma than the normal respiratory type, and this is supported by the observation that bleeds very seldom occur from the oral cavity in this syndrome.
The technique of infra red coagulation is well suited to the destruction of superficial blood vessels in the skin and/or mucosal surfaces. A method is described here for the destruction of resistant bleeding telangiectasia of the palate and lip in Osier-Weber-Rendu syndrome.
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