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[Orbito-temporal plexiform neurofibroma: 6 cases].

A Abouchadi1, M Nassih, A Rzin

  • 1Service de Chirurgie Plastique et Reconstructrice, Hôpital Roger Salengro, Lille. abouchadi.abdeljalil@wanadoo.fr

Revue De Stomatologie Et De Chirurgie Maxillo-Faciale
|November 18, 2005
PubMed
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Orbito-temporal plexiform neurofibromas, associated with Von Recklinghausen neurofibromatosis, require surgical resection. While challenging, surgery can reduce tumors and improve cosmetic outcomes, though bony reconstruction presents difficulties.

Area of Science:

  • Neurosurgery
  • Oncology
  • Genetics

Context:

  • Orbito-temporal plexiform neurofibromas are a hallmark of Von Recklinghausen neurofibromatosis.
  • This study reviews six cases focusing on diagnosis, progression, and treatment.

Purpose:

  • To analyze the diagnostic, therapeutic, and prognostic aspects of orbito-temporal plexiform neurofibromas.
  • To present surgical outcomes and challenges in managing these complex tumors.

Summary:

  • Six patients with orbito-temporal plexiform neurofibromas underwent surgical resection between 1986 and 2003.
  • All patients had spheno-orbital dysplasia, often with meningoencephalocele and pulsatile exophthalmia, necessitating eye exenteration.
  • Surgical reduction of the tumor was achieved, with two patients requiring complex bony reconstruction.

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Impact:

  • Highlights the necessity and difficulty of surgical intervention for these tumors.
  • Emphasizes the challenges in bony reconstruction due to progressive osseous dysplasia.
  • Reports good cosmetic results despite the complexity of the cases.