Nasal closure for severe hereditary haemorrhagic telangiectasia in 100 patients. The Lund modification of the Young's procedure: a 22-year experience.

Abstract

INTRODUCTION Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease characterized by recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. The nosebleeds can be life-threatening and in these circumstances, radical treatment is required. METHODS Since 1994, closure of the nose has been undertaken to prevent severe nasal bleeding in patients meeting specific selection criteria. Outcome data collected on this cohort pre- and post-operatively is available for analysis. RESULTS From a cohort of 515 HHT patients, 100 have undergone nasal closure, bilateral ab initio in 81%. Fifty patients completed pre- and post-operative epistaxis severity questionnaires and provided information on post-operative problems and 28 completed a Glasgow Benefit Inventory (GBI). Overall most patients derived significant benefit from the procedure with complete cessation of nasal bleeding in 94%, a highly significant improvement in the epistaxis score and a mean GBI score of 53.4. Loss of smell and taste was the most frequent post-operative complaint. CONCLUSION HHT can be associated with life-threatening epistaxis significantly affecting patients quality of life. Permanent prevention of airflow is associated with complete or near-total cessation of epistaxis in the majority of patients.