title: Using spectral-domain optical coherence tomography to detect optic neuropathy in patients with craniosynostosis.
abstract: BACKGROUND
  Detecting and monitoring optic neuropathy in patients with craniosynostosis is a clinical challenge due to limited cooperation, and subjective measures of visual function. The purpose of this study was to appraise the correlation of peripapillary retinal nerve fiber layer (RNFL) thickness measured by spectral-domain ocular coherence tomography (SD-OCT) with indication of optic neuropathy based on fundus examination.
  
  
  METHODS
  The medical records of all patients with craniosynostosis presenting for ophthalmic evaluation during 2013 were retrospectively reviewed. The following data were abstracted from the record: diagnosis, historical evidence of elevated intracranial pressure, current ophthalmic evaluation and visual field results, and current peripapillary RNFL thickness.
  
  
  RESULTS
  A total of 54 patients were included (mean age, 10.6 years [range, 2.4-33.8 years]). Thirteen (24%) had evidence of optic neuropathy based on current fundus examination. Of these, 10 (77%) demonstrated either peripapillary RNFL elevation and papilledema or depression with optic atrophy. Sensitivity for detecting optic atrophy was 88%; for papilledema, 60%; and for either form of optic neuropathy, 77%. Specificity was 94%, 90%, and 83%, respectively. Kappa agreement was substantial for optic atrophy (κ = 0.73) and moderate for papilledema (κ = 0.39) and for either form of optic neuropathy (κ = 0.54). Logistic regression indicated that peripapillary RNFL thickness was predictive of optic neuropathy (P < 0.001). Multivariable analysis demonstrated that RNFL thickness measurements were more sensitive at detecting optic neuropathy than visual field testing (likelihood ratio = 10.02; P = 0.002). Sensitivity and specificity of logMAR visual acuity in detecting optic neuropathy were 15% and 95%, respectively.
  
  
  CONCLUSIONS
  Peripapillary RNFL thickness measured by SD-OCT provides adjunctive evidence for identifying optic neuropathy in patients with craniosynostosis and appears more sensitive at detecting optic atrophy than papilledema.
authors.affiliation: Department of Ophthalmology, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts. Electronic address: Linda.Dagi@childrens.harvard.edu.
authors.forename: Linda R
authors.initials: LR
authors.lastname: Dagi
authors.affiliation: Department of Ophthalmology, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts.
authors.forename: Laura M
authors.initials: LM
authors.lastname: Tiedemann
authors.affiliation: Department of Ophthalmology, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts.
authors.forename: Gena
authors.initials: G
authors.lastname: Heidary
authors.affiliation: Department of Radiology, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts.
authors.forename: Caroline D
authors.initials: CD
authors.lastname: Robson
authors.affiliation: Department of Anesthesia, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts.
authors.forename: Amber M
authors.initials: AM
authors.lastname: Hall
authors.affiliation: Department of Anesthesia, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts; Department of Surgery, Boston Children's Hospital; Harvard Medical School, Boston, Massachusetts.
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003398:Craniosynostoses / Q000175:diagnosis*; D005189:False Positive Reactions; D005260:Female; D006801:Humans; D007427:Intracranial Pressure; D008297:Male; D009412:Nerve Fibers / Q000473:pathology*; D009896:Optic Atrophy / Q000175:diagnosis*; D010211:Papilledema / Q000175:diagnosis*; D011237:Predictive Value of Tests; D012165:Retinal Ganglion Cells / Q000473:pathology*; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D041623:Tomography, Optical Coherence; D014794:Visual Fields / Q000502:physiology; D055815:Young Adult