title: Orbitofacial neurofibromatosis: clinical characteristics and treatment outcome.
abstract: OBJECTIVE
  To report clinical observations and surgical management in a large series of patients with orbitofacial neurofibromatosis type 1 (OFNF).
  
  
  METHODS
  Patients were identified and medical records reviewed for demographic data, ophthalmologic examinations, surgical interventions, and procedure outcome to create a retrospective, non-comparative case series of patients with OFNF seen at one medical centre over a 23-year period.
  
  
  RESULTS
  Sixty patients with OFNF (31 females and 29 males; mean age, 14 years) were followed for an average of 5.7 years. Presenting signs and symptoms included eyelid swelling in all patients, ptosis in 56 (93.3%), proptosis in 34 (56.6%), dystopia or strabismus in 30 (50%), and decreased visual acuity in 50 (83.3%). Surgical intervention included ptosis repair in 54 (90%; mean 1.6 surgical procedures), facial and orbital tumour debulking in 54 (90%; mean 2.3 surgeries), and canthoplasty in 28 (46.6%) patients. Eleven patients required enucleation or exenteration of a blind eye.
  
  
  CONCLUSIONS
  Patients with OFNF often require multiple procedures to preserve vision, prevent additional disfigurement, and achieve cosmetic rehabilitation. Patients need regular ophthalmological monitoring given the potential for progressive visual and cosmetic consequences.
authors.affiliation: The King Khaled Eye Specialist Hospital, Al-Aruba Street, Riyadh, Saudi Arabia. orbitdr@yahoo.com
authors.forename: I A
authors.initials: IA
authors.lastname: Chaudhry
authors.forename: J
authors.initials: J
authors.lastname: Morales
authors.forename: F A
authors.initials: FA
authors.lastname: Shamsi
authors.forename: W
authors.initials: W
authors.lastname: Al-Rashed
authors.forename: E
authors.initials: E
authors.lastname: Elzaridi
authors.forename: Y O
authors.initials: YO
authors.lastname: Arat
authors.forename: C
authors.initials: C
authors.lastname: Jacquemin
authors.forename: D T
authors.initials: DT
authors.lastname: Oystreck
authors.forename: T M
authors.initials: TM
authors.lastname: Bosley
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005153:Facial Neoplasms / Q000473:pathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000473:pathology / Q000601:surgery*; D009918:Orbital Neoplasms / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D012529:Saudi Arabia; D014057:Tomography, X-Ray Computed; D014792:Visual Acuity; D055815:Young Adult