A case of Rendu-Osler-Weber disease with septal perforation treated with facial artery musculomucosal (FAMM) flap and septal dermoplasty

Sohei Endo; Kenji Ootsuka; Akinori Kida; Akira Yasuda; Kazutaka Shiba; Kenji Sasaki; Masaki Takeuchi

doi:10.5106/jjshns.14.121

Sohei Endo, Kenji Ootsuka, Akinori Kida, Akira Yasuda, Kazutaka Shiba, Kenji Sasaki, Masaki Takeuchi

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Keywords: hereditary hemorrhagic teleangiectasia ( HHT), septal perforation, septal dermoplasty, facial artery musculomucosal (FAMM) flap

JOURNAL FREE ACCESS

2004 Volume 14 Issue 2 Pages 121-125

DOI https://doi.org/10.5106/jjshns.14.121

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Published: October 05, 2004 Received: - Available on J-STAGE: July 27, 2010 Accepted: - Advance online publication: - Revised: -
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Date of correction: July 27, 2010 Reason for correction: - Correction: CITATION Details: Wrong : 1) Pribaz JJ, Stephens W, Crespo L et al.: Anew intraoral flap : facial artery musculo mucosal (FAMM) flap. Plast Reconst Surg 90 : 421-429, 1992.
2) Pribaz JJ, Meara JG, Wright S et al.: Lip and vermilion reconstruction with the facial artery musculomucosal flap. Plast Reconst Surg 105 : 864-869, 2004.
3) Saunders WH : Septal dermoplasty for cont rolof nosebleeds caused by hereditary hemorrhagic teleangiectasia or septal perforations. Trans Acac Ophthalmol Otolaryngol 64 : 500-506, 1960.
4) Saunders WH : Septal dermoplasty; ten years experience. Trans Am Acad Ophthal mol Otolaryngol 72 : 153-160, 1968.
6) Stecker RH, Lake CF: Hereditary hemor rhagic telangiectasia: Review of 102 cases and presentation of an innovation to septoder moplasty. Arch Otolaryngol 82 : 522 - 526, 1965.
7) McCabe WP, Kelly AP : Management of epistaxis in Olser- Weber- Rendu disease. Plast Reconst Surg 50 : 114-118, 1972.
8) McCaffrey TV, Kern EB, Lake CF: Manage ment of epistaxis in hereditary hemorrhagic telangiectasia. Review of 80 cases. Arch Otolaryngol 103 : 627-630, 1977.
9) Ulso C, Vase P, Stochksted P : Long-term results of dermoplasty in the treatment of hereditary hemorrhagic teleangiectasia. J Laryngol 97 : 223-226, 1983.
10) Siegel MB, Keane WK, Atkins JP et al.: Control of epistaxis in patients with hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 105 : 675-679, 1991.
11) Hirshowitz B, Moscona R, Eliachar I : Closure of septal perforation in Osler Weber-Rendu's disease by bilateral labial buccal flaps. Plast Reconst Surg 62 : 296-299, 1978.
12) Laurian N, Lalmanovitch M, Shimberg R: Amniotic graftingin the management of severe epistaxis due to hereditary haemor rhagic telangiectasia. J Laryngol Oto 193 : 589-595, 1979.
13) Kock HJ, Escher GC, Lewis JS: Hormonal management of hereditary hemorrhagic telangiectasia. J Am Med Assoc 149 : 1276-1380, 1952.
15) Ben-Bessat M, Kapla I, Levy R: Treatment of hereditary haemorrhagic telangiectasia of the nasal mucosa with the carbon dioxide laser. Br J Plast Surg 31 : 157-158, 1978.
16) Parkin JL & Dixon JA: Laser photoco agulation in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 89 : 204-208, 1981.
17) Shapshay SM & Oliver P : Treatment of hereditary hemorrhagic telagniectasia by Nd-YAG laser photocoagulation. Laryngoscope 94 : 1554-1556, 1984.

Right : 1) Pribaz JJ, Stephens W, Crespo L et al.: Anew intraoral flap : facial artery musculo mucosal (FAMM) flap. Plast Reconst Surg 90 : 421-429, 1992.
2) Pribaz JJ, Meara JG, Wright S et al.: Lip and vermilion reconstruction with the facial artery musculomucosal flap. Plast Reconst Surg 105 : 864-869, 2004.
3) Saunders WH : Septal dermoplasty for cont rolof nosebleeds caused by hereditary hemorrhagic teleangiectasia or septal perforations. Trans Acac Ophthalmol Otolaryngol 64 : 500-506, 1960.
4) Saunders WH : Septal dermoplasty; ten years experience. Trans Am Acad Ophthal mol Otolaryngol 72 : 153-160, 1968.
6) Stecker RH, Lake CF: Hereditary hemor rhagic telangiectasia: Review of 102 cases and presentation of an innovation to septoder moplasty. Arch Otolaryngol 82 : 522 - 526, 1965.
7) McCabe WP, Kelly AP : Management of epistaxis in Olser- Weber- Rendu disease. Plast Reconst Surg 50 : 114-118, 1972.
8) McCaffrey TV, Kern EB, Lake CF: Manage ment of epistaxis in hereditary hemorrhagic telangiectasia. Review of 80 cases. Arch Otolaryngol 103 : 627-630, 1977.
9) Ulso C, Vase P, Stochksted P : Long-term results of dermoplasty in the treatment of hereditary hemorrhagic teleangiectasia. J Laryngol 97 : 223-226, 1983.
10) Siegel MB, Keane WK, Atkins JP et al.: Control of epistaxis in patients with hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 105 : 675-679, 1991.
11) Hirshowitz B, Moscona R, Eliachar I : Closure of septal perforation in Osler Weber-Rendu's disease by bilateral labial buccal flaps. Plast Reconst Surg 62 : 296-299, 1978.
12) Laurian N, Lalmanovitch M, Shimberg R: Amniotic graftingin the management of severe epistaxis due to hereditary haemor rhagic telangiectasia. J Laryngol Oto 193 : 589-595, 1979.
13) Kock HJ, Escher GC, Lewis JS: Hormonal management of hereditary hemorrhagic telangiectasia. J Am Med Assoc 149 : 1276-1380, 1952.
15) Ben-Bessat M, Kapla I, Levy R: Treatment of hereditary haemorrhagic telangiectasia of the nasal mucosa with the carbon dioxide laser. Br J Plast Surg 31 : 157-158, 1978.
16) Parkin JL & Dixon JA: Laser photoco agulation in hereditary hemorrhagic telangiectasia. Otolaryngol Head Neck Surg 89 : 204-208, 1981.
17) Shapshay SM & Oliver P : Treatment of hereditary hemorrhagic telagniectasia by Nd-YAG laser photocoagulation. Laryngoscope 94 : 1554-1556, 1984.

Abstract

A 69-year-old female was referred to our clinic with recurrent nasal bleeding and resultant anemia (Hb 7.3 g/dl) . The patient had septal perforation as a result of frequent laser vaporization and the margins of the perforation were the sources of nasal bleeding. She had teleangiectatic lesions on her tongue, positive Rumpel-Leede test, and a brother with similar episodes of nasal bleeding, so she was diagnosed as having hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber disease). To treat the septal perforation as well as recurrent nasal bleeding, the anterior aspects of the nasal septal mucosa were denuded and replaced with a facial artery musculomucosal (FAMM) flap on the left side, and with a split thick skin on the right side. She is now almost free from nasal bleeding. The FAMM flap is useful for treating patients with HHT especially with septal perforation.

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