title: [Long-term outcome of gliomas of the visual pathway in type 1 neurofibromatosis].
abstract: BACKGROUND
  Optic gliomas are frequently associated with neurofibromatosis type 1 (NF1) and belong to the diagnostic criteria of NF1. Different growth rates require differentiated strategies for screening and observation.
  
  
  METHODS
  29 patients (25 children < 11 years, 4 adults > 18 years) with visual pathway tumors and NF1 were examined neurologically, ophthalmogically, by means of MRI and VEP. Results were set into context with preceding investigations (mean follow up time 6.5 years).
  
  
  RESULTS
  11 children showed a stable condition, 14 an unfavorable process with substantial loss of vision. Children with an unfavorable process showed a lower age at diagnosis (3.2 versus 5.8 years; p < 0.05), more frequently strabism (11/14 versus 1/11; p < 0.05), optic atrophy (12/14 versus 1/11; p < 0.05), pathological VEP (9/9 versus 2/10; p = 0.001), visual field defects (9/9 versus 1/9) and involvement of the optic chiasm (11/14 versus 3/11; p < 0.05) than children with a stable condition. 3 of 4 adults had no visual symptoms despite involvement of the optic chiasm.
  
  
  CONCLUSIONS
  The crucial prognostic factor is the patient's age at the time of diagnosis. Optic gliomas which become symptomatic in early childhood (< 6 years) grow rapidly and require frequent ophthalmologic investigations and MRI. Tumors diagnosed in late childhood (> 6 years) do not progress, allowing for gradual extension of intervals between ophthalmological investigations.
authors.affiliation: Neurologische Klinik, Universitätskrankenhaus Hamburg-Eppendorf, Hamburg.
authors.forename: S
authors.initials: S
authors.lastname: Schröder
authors.forename: U
authors.initials: U
authors.lastname: Baumann-Schröder
authors.forename: W
authors.initials: W
authors.lastname: Hazim
authors.forename: W
authors.initials: W
authors.lastname: Haase
authors.forename: V F
authors.initials: VF
authors.lastname: Mautner
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005074:Evoked Potentials, Visual / Q000502:physiology; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis*; D009897:Optic Chiasm / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology; D020339:Optic Nerve Glioma / Q000175:diagnosis*; D019574:Optic Nerve Neoplasms / Q000175:diagnosis*; D011379:Prognosis; D014792:Visual Acuity / Q000502:physiology