title: Optical coherence tomography in the evaluation of neurofibromatosis type-1 subjects with optic pathway gliomas.
abstract: OBJECTIVE
  Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disorder, with an approximate incidence of 1 in 3,500. Optic pathway gliomas (OPGs) develop in 15% of individuals with NF1, commonly in childhood. OPGs are difficult to detect via a clinical inspection in children, often requiring magnetic resonance imaging (MRI). Given the significant visual risks associated with OPGs in NF1, there is a need for improved noninvasive techniques to diagnose OPGs in children; therefore, we studied optical coherence tomography (OCT) as a potential tool to assess optic nerve and retinal nerve fiber layer (RNFL) abnormalities. This prospective study was designed to evaluate OCT detection of RNFL loss from optic atrophy attributable to OPGs in a cohort of pediatric patients with NF1.
  
  
  METHODS
  With the use of Stratus OCT, directed testing with the Fast Macular Thickness and Fast RNFL Thickness protocol scans were performed on 9 subjects with NF1 and known OPGs, 6 subjects with NF1 without OPGs, and 15 controls.
  
  
  RESULTS
  NF1 subjects with OPGs had thinner RNFLs and macula when compared with age-matched controls and to NF1 subjects without OPGs. After applying the equivalence equation, the average RNFL thickness and macular volume in NF1 subjects without OPGs was equivalent to controls.
  
  
  CONCLUSIONS
  Our study suggests that OCT can be used to detect RNFL thinning secondary to OPGs in NF1 subjects. This objective tool shows promise as a useful adjunct to routine clinical ophthalmologic evaluation in children with NF1.
authors.affiliation: Duke Eye Center, Durham, North Carolina, USA.
authors.forename: Lan
authors.initials: L
authors.lastname: Chang
authors.forename: Mays A
authors.initials: MA
authors.lastname: El-Dairi
authors.forename: Tamiesha A
authors.initials: TA
authors.lastname: Frempong
authors.forename: Erica L
authors.initials: EL
authors.lastname: Burner
authors.forename: M Tariq
authors.initials: MT
authors.lastname: Bhatti
authors.forename: Terri L
authors.initials: TL
authors.lastname: Young
authors.forename: Fawn
authors.initials: F
authors.lastname: Leigh
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005910:Glioma / Q000175:diagnosis* / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008266:Macula Lutea / Q000473:pathology; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000453:epidemiology; D009900:Optic Nerve / Q000473:pathology*; D019574:Optic Nerve Neoplasms / Q000175:diagnosis* / Q000453:epidemiology; D011446:Prospective Studies; D041623:Tomography, Optical Coherence