title: Handheld optical coherence tomography during sedation in young children with optic pathway gliomas.
abstract: OBJECTIVE
  Monitoring young children with optic pathway gliomas (OPGs) for visual deterioration can be difficult owing to age-related noncompliance. Optical coherence tomography (OCT) measures of retinal nerve fiber layer (RNFL) thickness have been proposed as a surrogate marker of vision but this technique is also limited by patient cooperation.
  
  
  OBJECTIVE
  To determine whether measures of circumpapillary RNFL thickness, acquired with handheld OCT (HH-OCT) during sedation, can differentiate between young children with and without vision loss from OPGs.
  
  
  METHODS
  This cross-sectional analysis of a prospective observational study was conducted at a tertiary-care children's hospital. Children with an OPG (sporadic or secondary to neurofibromatosis type 1) who were cooperative for visual acuity testing, but required sedation to complete magnetic resonance imaging, underwent HH-OCT imaging of the circumpapillary RNFL while sedated.
  
  
  METHODS
  Area under the curve of the receiver operating characteristic, sensitivity, specificity, positive predictive value, and negative predictive value of the average and quadrant-specific RNFL thicknesses.
  
  
  RESULTS
  Thirty-three children (64 eyes) met inclusion criteria (median age, 4.8 years; range, 1.8-12.6 years). In children with vision loss (abnormal visual acuity and/or visual field), RNFL thickness was decreased in all quadrants compared with the normal-vision group (P < .001 for all comparisons). Using abnormal criteria of less than 5% and less than 1%, the area under the curve was highest for the average RNFL thickness (0.96 and 0.97, respectively) compared with specific anatomic quadrants. The highest discrimination and predictive values were demonstrated for participants with 2 or more quadrants meeting less than 5% (sensitivity = 93.3; specificity = 97.9; positive predictive value = 93.3; and negative predictive value = 97.9) and less than 1% (sensitivity = 93.3; specificity = 100; positive predictive value = 100; and negative predictive value = 98.0) criteria.
  
  
  CONCLUSIONS
  Measures of RNFL thickness acquired with HH-OCT during sedation can differentiate between young children with and without vision loss from OPGs. For young children who do not cooperate with vision testing, HH-OCT measures may be a surrogate marker of vision. Longitudinal studies are needed to delineate the temporal relationship between RNFL decline and vision loss.
authors.affiliation: The Gilbert Family Neurofibromatosis Institute, Children's National Medical Center, Washington, DC2Department of Neurology, Children's National Medical Center, Washington, DC3Department of Ophthalmology, Children's National Medical Center, Washington, DC7.
authors.forename: Robert A
authors.initials: RA
authors.lastname: Avery
authors.affiliation: Department of Oncology, Children's National Medical Center, Washington, DC7The Brain Tumor Institute, Children's National Medical Center, Washington, DC.
authors.forename: Eugene I
authors.initials: EI
authors.lastname: Hwang
authors.affiliation: University of Pittsburgh Medical Center Eye Center, Eye and Ear Institute, Ophthalmology and Visual Science Research Center, Department of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania10Department of Bioengineering.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishikawa
authors.affiliation: The Gilbert Family Neurofibromatosis Institute, Children's National Medical Center, Washington, DC2Department of Neurology, Children's National Medical Center, Washington, DC8Center for Neuroscience and Behavior, Children's National Medical Center, Washin.
authors.forename: Maria T
authors.initials: MT
authors.lastname: Acosta
authors.affiliation: Department of Ophthalmology, Children's National Medical Center, Washington, DC.
authors.forename: Kelly A
authors.initials: KA
authors.lastname: Hutcheson
authors.affiliation: Department of Anesthesiology, Children's National Medical Center, Washington, DC.
authors.forename: Domiciano
authors.initials: D
authors.lastname: Santos
authors.affiliation: Department of Genetics, Children's National Medical Center, Washington, DC.
authors.forename: Dina J
authors.initials: DJ
authors.lastname: Zand
authors.affiliation: Department of Oncology, Children's National Medical Center, Washington, DC7The Brain Tumor Institute, Children's National Medical Center, Washington, DC.
authors.forename: Lindsay B
authors.initials: LB
authors.lastname: Kilburn
authors.affiliation: Department of Genetics, Children's National Medical Center, Washington, DC.
authors.forename: Kenneth N
authors.initials: KN
authors.lastname: Rosenbaum
authors.affiliation: Department of Oncology, Children's National Medical Center, Washington, DC7The Brain Tumor Institute, Children's National Medical Center, Washington, DC.
authors.forename: Brian R
authors.initials: BR
authors.lastname: Rood
authors.affiliation: University of Pittsburgh Medical Center Eye Center, Eye and Ear Institute, Ophthalmology and Visual Science Research Center, Department of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania10Department of Bioengineering.
authors.forename: Joel S
authors.initials: JS
authors.lastname: Schuman
authors.affiliation: The Gilbert Family Neurofibromatosis Institute, Children's National Medical Center, Washington, DC2Department of Neurology, Children's National Medical Center, Washington, DC6Department of Oncology, Children's National Medical Center, Washington, DC7The B.
authors.forename: Roger J
authors.initials: RJ
authors.lastname: Packer
mesh_terms: D019540:Area Under Curve; D002648:Child; D002675:Child, Preschool; D016292:Procedural Sedation; D003430:Cross-Sectional Studies; D005189:False Positive Reactions; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009412:Nerve Fibers / Q000473:pathology*; D020339:Optic Nerve Glioma / Q000175:diagnosis* / Q000503:physiopathology; D019574:Optic Nerve Neoplasms / Q000175:diagnosis* / Q000503:physiopathology; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012165:Retinal Ganglion Cells / Q000473:pathology*; D012680:Sensitivity and Specificity; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000175:diagnosis / Q000503:physiopathology; D014792:Visual Acuity / Q000502:physiology; D014794:Visual Fields / Q000502:physiology; D014795:Visual Pathways / Q000473:pathology*