[Nasal cavity dermoplasty as a treatment of recurrent bleeding in Rendu-Osler-Weber's disease]

Abstract

Introduction: Rendu-Osler-Weber's (ROW) disease is a systemic disorder of blood vessels classified as a capillary malformation. The most common manifestation of hereditary hemorrhagic teleangiectasia is recurrent epistaxis. The diagnosis of ROW disease is made clinically using the Curacao criteria. Management of epistaxis is usually difficult and many types of treatment have been described.

Material and methods: We report 12 patients, 7 female and 5 male, aged 52 to 78 with ROW disease and recurrent epistaxis with frequency varying from 5 episodes monthly to 3 - 5 episodes daily. In most of them blood transfusion was required. The patients were treated with intranasal dermoplasty described by Saunders. The dermoplasty was performed on the septal, lateral or both walls of the anterior part of the nasal cavities.

Results: In all the patients the graft was taken completely. Two patients had scars and narrowing of nasal valve region. Recurrence and increase in the intensity of nasal bleeding were observed in 3 patients. In the course of further therapy the second partial septal dermoplasty was successfully performed in 2 of them. The second surgery revealed new bleeding teleangiectases beyond of the dermal graft.

Conclusion: Nasal cavity dermoplasty remains an effective way of reducing epistaxis and blood transfusion in patients with ROW disease and subjectively improves their quality of life. The surgical treatment can be repeated, if nasal bleeding recur.