title: Multiple intraorbital neurofibromas: a rare cause of proptosis.
abstract: BACKGROUND
  Orbital neurofibromas are rare, accounting for 0.5 to 2.4 % of all orbital tumors. Generally, they manifest as slowly progressive proptosis, in a young adult or middle-aged person, and are usually solitary lesions. Sometimes, they can be associated with type 1 neurofibromatosis. We present a case of proptosis related to multiple intraorbital neurofibromas in an 82-year-old woman without type 1 neurofibromatosis.
  
  
  METHODS
  An 82-year-old woman was referred for slowly progressive left proptosis associated with an ocular burning sensation. Neuro-ophthalmic examination revealed 9.5 mm of left exophthalmos, signs of minimal left optic neuropathy but normal extraocular movements. Magnetic resonance imaging revealed the presence of 4 intraorbital lesions.
  
  
  RESULTS
  The two most anterior tumors were removed. Pathological studies showed these tumors to be neurofibromas. Post-operative evolution was favorable with reduction of left proptosis to 7 mm and disappearance of the burning sensation of the left eye. No other signs of neurofibromatosis were found.
  
  
  CONCLUSIONS
  Multiple circumscribed intraorbital tumors are rare. Slowly progressive proptosis with radiological imaging of multiple round lesions should evoke the diagnosis of orbital neurofibromas, even in patients outside the typical age range or without neurofibromatosis.
authors.affiliation: Hôpital Ophtalmique Jules Gonin, Lausanne, Switzerland.
authors.forename: C
authors.initials: C
authors.lastname: Berney
authors.forename: B
authors.initials: B
authors.lastname: Spahn
authors.forename: C
authors.initials: C
authors.lastname: Oberhansli
authors.forename: S
authors.initials: S
authors.lastname: Uffer
authors.forename: F-X
authors.initials: FX
authors.lastname: Borruat
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005094:Exophthalmos / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D017253:Neurofibromatoses / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D009915:Orbit / Q000473:pathology / Q000601:surgery; D009918:Orbital Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery