Abstract
PurposeHereditary hemorrhagic telangiectasia (HHT) is a vascular disorder that presents with recurrent, intractable epistaxis. The aim of this study was to retrospectively analyze the efficacy of various treatment options for epistaxis in patients with HHT, over a period of 18 years, and to correlate these findings with available evidence in the literature.MethodsRecords of patients with HHT, treated for epistaxis between 2000 and 2018 were analyzed. Treatment procedures carried out and their efficacy were extracted and analyzed.ResultsForty-three records were evaluated. All patients were given nasal humidifying ointments, 93% required acute treatment with bipolar electrocautery, and 60% underwent atraumatic nasal packing. Recurrent cases were treated medically with tranexamic acid (26%), oestrogen (19%), and bevacizumab (2%). Laser photocoagulation was done in selected cases (40%) and if unsuccessful, septal dermoplasty was performed (2.3%). Endovascular embolization was reserved for life-threatening emergencies (7%).ConclusionEpistaxis in HHT is not curable, but can be managed by employing a comprehensive stepwise approach. An algorithm for effective and comprehensive management has been presented.
Highlights
Hereditary haemorrhagic telangiectasia (HHT), known as Osler–Weber–Rendu disease, is a rare autosomal dominant disorder causing angiodysplasia
A total of 51 records of patients suffering from HHT were obtained
The present study shows that despite numerous options for treatment, epistaxis continues to be a recurrent problem in HHT patients
Summary
Hereditary haemorrhagic telangiectasia (HHT), known as Osler–Weber–Rendu disease, is a rare autosomal dominant disorder causing angiodysplasia. It occurs in one of every 5000 persons, and is believed to be underdiagnosed [1]. In HHT, capillaries of the skin, mucous membranes, and specific organs such as lungs and liver are absent, and there is direct communication between the dilated arteries and veins This causes extreme fragility of the vessels, which can Epistaxis is the most common clinical manifestation of HHT. The frequency and severity of epistaxis often leads to iron deficiency anaemia, requiring multiple blood transfusions. This has a negative impact on the quality of life [2]
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