Supported in part by March of Dimes grant HHT-FY04–677 and Josephine Lawrence Hopkins Foundation and General Clinical Research Center NIH M01-RR-00125 grant (r.i.w. and k.j.h.).
Outcome of Septal Dermoplasty in Patients With Hereditary Hemorrhagic Telangiectasia†
Article first published online: 3 JAN 2009
Copyright © 2005 The Triological Society
Volume 115, Issue 2, pages 301–305, February 2005
How to Cite
Fiorella, M. L., Ross, D., Henderson, K. J. and White, R. I. (2005), Outcome of Septal Dermoplasty in Patients With Hereditary Hemorrhagic Telangiectasia. The Laryngoscope, 115: 301–305. doi: 10.1097/01.mlg.0000154754.39797.43
- Issue published online: 3 JAN 2009
- Article first published online: 3 JAN 2009
- Manuscript Accepted: 3 AUG 2004
- Septal dermoplasty;
- hereditary hemorrhagic telangiectasia
Objectives/Hypothesis: Septal dermoplasty has been recommended as the treatment of choice for life-threatening epistaxis in patients with hereditary hemorrhagic telangiectasia. The purpose of the study was to evaluate the effectiveness and outcomes of septal dermoplasty for management of transfusion-dependent epistaxis.
Study Design: Retrospective study.
Methods: Between 1994 and 2004, septal dermoplasty was performed on 67 consecutive patients with severe epistaxis attributable to hereditary hemorrhagic telangiectasia. The numbers of units of blood received 1 year before and 1 year after septal dermoplasty were obtained. A subjective appraisal of the results of the surgery as well as second procedures after septal dermoplasty was determined. Patients were screened for pulmonary and cerebral arteriovenous malformations, gastrointestinal tract bleeding, and symptomatic liver disease.
Results: Data were obtained in 66 of 67 (98%) patients with a mean age of 61.5 years (mean follow-up, 3.9 y). Accurate transfusion requirements 1 year before and 1 year after septal dermoplasty were available in 32 of 66 (48%) patients. In these 32 patients, the mean units of blood received decreased from 21 units (range, 2–100 units) 1 year before septal dermoplasty to 1 unit (range, 0–10 units) in the year after septal dermoplasty (P < .001). Improved quality of life was claimed in 57 patients. Second therapies, ranging from cautery to repeat partial septal dermoplasty, were required in 15 patients during follow-up. Among the 67 patients, 31 (46%) had pulmonary arteriovenous malformation, 14 (21%) had gastrointestinal tract bleeding, 7 (10%) had symptomatic liver disease, and 5 (7%) had cerebral arteriovenous malformation. During the follow-up, 14 patients died of other complications of hereditary hemorrhagic telangiectasia (11 patients) and unrelated causes (3 patients).
Conclusion: Septal dermoplasty remains an effective way of reducing transfusion requirements in patients with hereditary hemorrhagic telangiectasia and subjectively improves their quality of life. The otolaryngologist caring for patients with hereditary hemorrhagic telangiectasia should be familiar with other organ involvement by hereditary hemorrhagic telangiectasia to prevent complications during surgery.