Abstract
Cases of hereditary hemorrhagic telangiectasia (HHT), an autosomal-dominant inherited disorder adversely affecting skin, mucous membrane, and visceral blood vessels, usually seen first by otolaryngologists because epistaxis is the most common manifestation. HHT varies greatly in epistaxis course and severity, requiring hospitalization and multiple transfusions in about one-third of all cases. None of the many available treatment strategies has proven entirely successful for all individuals. Since the early 1990s we have conducted 30 nasal dermoplasties (ND) treating those with moderate or severe HHT. To reduce epistaxis incidence by protecting fragile abnormal vessels in the superficial nasal mucosa from trauma by applying a split-thickness skin graft. We found ND to be effective in HHT subjects with moderate or severe nasal bleeding.
Saunders, who pioneered this technique, recommended anterior septal mucosal removal, but we have gone on to remove mucosa from the anterior septum, the nasal floor and the inferior turbinate, covering the denuded area with a split-skin graft. ND reduces bleeding frequency. Bleeding may, however, occur after ND, due to telangiectatic mucosal areas in the anterior superior septal mucosa, left uncovered at the suture margin. We have thus added further removal of the most anterosuperior septal mucosa, grafting the split-thickness skin circumferentially. This refinement has not produced nasal valve stenosis and has, in fact, improved postoperative intranasal findings. In the presence of septal perforation, the perforation should be repaired before or at least during skin grafting.
