PMID- 28384671
OWN - NLM
STAT- MEDLINE
DCOM- 20170719
LR  - 20191210
IS  - 2168-6173 (Electronic)
IS  - 2168-6165 (Print)
IS  - 2168-6165 (Linking)
VI  - 135
IP  - 5
DP  - 2017 May 1
TI  - Acute Zonal Cone Photoreceptor Outer Segment Loss.
PG  - 487-490
LID - 10.1001/jamaophthalmol.2017.0451 [doi]
AB  - IMPORTANCE: 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. OBJECTIVE: To describe the 
      structural and functional associations found in a patient with acute zonal occult 
      photoreceptor loss. DESIGN, SETTING, AND PARTICIPANTS: A case report of an 
      adolescent boy with acute visual field loss despite a normal fundus examination 
      performed at a university teaching hospital. MAIN OUTCOMES AND MEASURES: 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). RESULTS: 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. CONCLUSIONS AND RELEVANCE: 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.
FAU - Aleman, Tomas S
AU  - Aleman TS
AD  - 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.
FAU - Sandhu, Harpal S
AU  - Sandhu HS
AD  - Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of 
      Pennsylvania, Philadelphia.
FAU - Serrano, Leona W
AU  - Serrano LW
AD  - Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of 
      Pennsylvania, Philadelphia.
FAU - Traband, Anastasia
AU  - Traband A
AD  - Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of 
      Pennsylvania, Philadelphia.
FAU - Lau, Marisa K
AU  - Lau MK
AD  - Scheie Eye Institute at the Perelman Center for Advanced Medicine, University of 
      Pennsylvania, Philadelphia.
FAU - Adamus, Grazyna
AU  - Adamus G
AD  - Ocular Immunology Laboratory, Casey Eye Institute, Oregon Health and Science 
      University, Portland.
FAU - Avery, Robert A
AU  - Avery RA
AD  - 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.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - JAMA Ophthalmol
JT  - JAMA ophthalmology
JID - 101589539
RN  - Acute zonal occult outer retinopathy
SB  - IM
MH  - Adolescent
MH  - Color Vision/*physiology
MH  - Electroretinography
MH  - Fluorescein Angiography
MH  - Fundus Oculi
MH  - Humans
MH  - Male
MH  - Retinal Cone Photoreceptor Cells/*pathology
MH  - Scotoma/*diagnosis/physiopathology
MH  - Tomography, Optical Coherence/methods
MH  - *Visual Acuity
MH  - *Visual Fields
MH  - White Dot Syndromes
PMC - PMC5847106
COIS- Conflict of Interest Disclosures: All authors have completed and submitted the 
      ICMJE Form for Disclosure of Potential Conflicts of Interest and none were 
      reported.
EDAT- 2017/04/07 06:00
MHDA- 2017/07/20 06:00
PMCR- 2018/04/06
CRDT- 2017/04/07 06:00
PHST- 2017/04/07 06:00 [pubmed]
PHST- 2017/07/20 06:00 [medline]
PHST- 2017/04/07 06:00 [entrez]
PHST- 2018/04/06 00:00 [pmc-release]
AID - 2613415 [pii]
AID - ebr170005 [pii]
AID - 10.1001/jamaophthalmol.2017.0451 [doi]
PST - ppublish
SO  - JAMA Ophthalmol. 2017 May 1;135(5):487-490. doi: 
      10.1001/jamaophthalmol.2017.0451.