title: Optical coherence tomography in the diagnosis of optic pathway gliomas.
abstract: OBJECTIVE
  To compare visual function assessment, optic disc evaluation by indirect ophthalmoscopy, and retinal nerve fiber layer analysis by optical coherence tomography (OCT) for the screening of optic pathway gliomas in pediatric patients (2-15 years old) affected by neurofibromatosis type 1.
  
  
  METHODS
  Fifty-seven consecutive patients with neurofibromatosis type 1 with recent (<6 months) orbital/brain magnetic resonance images (MRI) were included. Patients underwent visual function assessment (Hyvarinen symbols chart and/or Snellen charts) and optic disc evaluation by indirect ophthalmoscopy performed by experienced, masked pediatric ophthalmologists. Spectral domain OCT was performed to assess retinal nerve fiber layer.
  
  
  RESULTS
  Fifteen of 57 enrolled patients (26%) were affected by MRI-proven optic pathway gliomas. Visual function assessment, optic disc evaluation, and retinal nerve fiber layer analysis by OCT were feasible in 84%, 95%, and 88% of patients, respectively. Visual function assessment, retinal nerve fiber layer analysis, and optic disc evaluation results correlated with the presence of optic pathway gliomas (P = 0.007, P < 0.0001, and P = 0.03, respectively). Specificity and negative predictive value of each test were statistically significant in detecting optic pathway glioma (P < 0.0001), whereas only retinal nerve fiber layers analysis reached statistically significant sensitivity and positive predictive value (P = 0.0386).
  
  
  CONCLUSIONS
  Retinal nerve fiber layer analysis assessment using spectral domain OCT is superior to visual function assessment and optic disc evaluation as a clinical screening tool for optic pathway gliomas.
authors.affiliation: GB Bietti Foundation, IRCCS, Ocular Oncology and Toxicology Research Unit, Rome, Italy.
authors.forename: Raffaele
authors.initials: R
authors.lastname: Parrozzani
authors.forename: Maurizio
authors.initials: M
authors.lastname: Clementi
authors.forename: Olympia
authors.initials: O
authors.lastname: Kotsafti
authors.forename: Giacomo
authors.initials: G
authors.lastname: Miglionico
authors.forename: Eva
authors.initials: E
authors.lastname: Trevisson
authors.forename: Gloria
authors.initials: G
authors.lastname: Orlando
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Pilotto
authors.forename: Edoardo
authors.initials: E
authors.lastname: Midena
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009898:Optic Disk / Q000473:pathology*; D020339:Optic Nerve Glioma / Q000175:diagnosis*; D011237:Predictive Value of Tests; D015203:Reproducibility of Results; D012165:Retinal Ganglion Cells / Q000473:pathology; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence / Q000379:methods*
keywords: OCT; glioma; neurofibromatosis