Reversal of Young's procedure in hereditary haemorrhagic telangiectasia

Abstract

Hereditary haemorrhagic telangiectasia is an autosomal dominant condition characterised by multiple venous malformations of the skin and mucous membranes which can bleed on contact. A Young's procedure is used to control severe epistaxis in patients with this condition. However, there has been no previous report of a reversal of Young's procedure in such a patient. A patient with hereditary haemorrhagic telangiectasia had his Young's procedure reversed under general anaesthetic by dividing the mucocutaneous flaps. Nasal vestibule patency was maintained using a custom-made silicone nasal mould. Intra-operative photographs show the patient's nasal mucosa shortly following reversal of his Young's procedure, and also illustrate the creation of the custom-made nasal mould. Stopping airflow through the nasal cavity via a Young's procedure prevents the telangiectasia from bleeding but does not make them disappear completely. Young's procedure does not seem to have any long-lasting effect on the nasal mucosa of patients with hereditary haemorrhagic telangiectasia.