title: Acute Zonal Cone Photoreceptor Outer Segment Loss.
abstract: The diagnostic path presented narrows down the cause of acute vision loss to the cone photoreceptor outer segment and will refocus the search for the cause of similar currently idiopathic conditions.
  
  
  
  To describe the structural and functional associations found in a patient with acute zonal occult photoreceptor loss.
  
  
  
  A case report of an adolescent boy with acute visual field loss despite a normal fundus examination performed at a university teaching hospital.
  
  
  
  Results of a complete ophthalmic examination, full-field flash electroretinography (ERG) and multifocal ERG, light-adapted achromatic and 2-color dark-adapted perimetry, and microperimetry. Imaging was performed with spectral-domain optical coherence tomography (SD-OCT), near-infrared (NIR) and short-wavelength (SW) fundus autofluorescence (FAF), and NIR reflectance (REF).
  
  
  
  The patient was evaluated within a week of the onset of a scotoma in the nasal field of his left eye. Visual acuity was 20/20 OU, and color vision was normal in both eyes. Results of the fundus examination and of SW-FAF and NIR-FAF imaging were normal in both eyes, whereas NIR-REF imaging showed a region of hyporeflectance temporal to the fovea that corresponded with a dense relative scotoma noted on light-adapted static perimetry in the left eye. Loss in the photoreceptor outer segment detected by SD-OCT co-localized with an area of dense cone dysfunction detected on light-adapted perimetry and multifocal ERG but with near-normal rod-mediated vision according to results of 2-color dark-adapted perimetry. Full-field flash ERG findings were normal in both eyes. The outer nuclear layer and inner retinal thicknesses were normal.
  
  
  
  Localized, isolated cone dysfunction may represent the earliest photoreceptor abnormality or a distinct entity within the acute zonal occult outer retinopathy complex. Acute zonal occult outer retinopathy should be considered in patients with acute vision loss and abnormalities on NIR-REF imaging, especially if multimodal imaging supports an intact retinal pigment epithelium and inner retina but an abnormal photoreceptor outer segment.
authors.affiliation: Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia2Division of Ophthalmology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania3Department of Ophthalmology, University of Pennsylvania, Philadelphia.
authors.forename: Tomas S
authors.initials: TS
authors.lastname: Aleman
authors.affiliation: Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia.
authors.forename: Harpal S
authors.initials: HS
authors.lastname: Sandhu
authors.affiliation: Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia.
authors.forename: Leona W
authors.initials: LW
authors.lastname: Serrano
authors.affiliation: Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Traband
authors.affiliation: Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia.
authors.forename: Marisa K
authors.initials: MK
authors.lastname: Lau
authors.affiliation: Ocular Immunology Laboratory, Casey Eye Institute, Oregon Health and Science University, Portland.
authors.forename: Grazyna
authors.initials: G
authors.lastname: Adamus
authors.affiliation: Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of Pennsylvania, Philadelphia2Division of Ophthalmology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania3Department of Ophthalmology, University of Pennsylvania, Philadelphia.
authors.forename: Robert A
authors.initials: RA
authors.lastname: Avery
mesh_terms: D000293:Adolescent; D055253:Color Vision / Q000502:physiology*; D004596:Electroretinography; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D017949:Retinal Cone Photoreceptor Cells / Q000473:pathology*; D012607:Scotoma / Q000175:diagnosis* / Q000503:physiopathology; D041623:Tomography, Optical Coherence / Q000379:methods; D014792:Visual Acuity; D014794:Visual Fields; D000080363:White Dot Syndromes