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Am J Ophthalmol Case Rep. 2017 Sep;7:14-19. doi: 10.1016/j.ajoc.2017.04.001. Epub 2017 Apr 12.

Dysflective cones: Visual function and cone reflectivity in long-term follow-up of acute bilateral foveolitis.

American journal of ophthalmology case reports

Joanna H Tu, Katharina G Foote, Brandon J Lujan, Kavitha Ratnam, Jia Qin, Michael B Gorin, Emmett T Cunningham, William S Tuten, Jacque L Duncan, Austin Roorda

Affiliations

  1. Department of Ophthalmology, 10 Koret Way, University of California San Francisco, San Francisco, CA, USA.
  2. College of Physicians and Surgeons, Columbia University, New York, NY, USA.
  3. School of Optometry and Vision Science Graduate Group, University of California Berkeley, Berkeley, CA, USA.
  4. West Coast Retina Medical Group, San Francisco, CA, USA.
  5. Stein Eye Institute, Department of Ophthalmology, University of California Los Angeles, Los Angeles, CA, USA.
  6. Department of Ophthalmology, California Pacific Medical Center, San Francisco, CA, USA.
  7. Department of Ophthalmology, Stanford University School of Medicine, Stanford, CA, USA.
  8. The Francis I. Proctor Foundation, University of California San Francisco, School of Medicine, San Francisco, CA, USA.

PMID: 29057371 PMCID: PMC5644392 DOI: 10.1016/j.ajoc.2017.04.001

Abstract

PURPOSE: Confocal adaptive optics scanning laser ophthalmoscope (AOSLO) images provide a sensitive measure of cone structure. However, the relationship between structural findings of diminished cone reflectivity and visual function is unclear. We used fundus-referenced testing to evaluate visual function in regions of apparent cone loss identified using confocal AOSLO images.

METHODS: A patient diagnosed with acute bilateral foveolitis had spectral-domain optical coherence tomography (SD-OCT) (Spectralis HRA + OCT system [Heidelberg Engineering, Vista, CA, USA]) images indicating focal loss of the inner segment-outer segment junction band with an intact, but hyper-reflective, external limiting membrane. Five years after symptom onset, visual acuity had improved from 20/80 to 20/25, but the retinal appearance remained unchanged compared to 3 months after symptoms began. We performed structural assessments using SD-OCT, directional OCT (non-standard use of a prototype on loan from Carl Zeiss Meditec) and AOSLO (custom-built system). We also administered fundus-referenced functional tests in the region of apparent cone loss, including analysis of preferred retinal locus (PRL), AOSLO acuity, and microperimetry with tracking SLO (TSLO) (prototype system). To determine AOSLO-corrected visual acuity, the scanning laser was modulated with a tumbling E consistent with 20/30 visual acuity. Visual sensitivity was assessed in and around the lesion using TSLO microperimetry. Complete eye examination, including standard measures of best-corrected visual acuity, visual field tests, color fundus photos, and fundus auto-fluorescence were also performed.

RESULTS: Despite a lack of visible cone profiles in the foveal lesion, fundus-referenced vision testing demonstrated visual function within the lesion consistent with cone function. The PRL was within the lesion of apparent cone loss at the fovea. AOSLO visual acuity tests were abnormal, but measurable: for trials in which the stimulus remained completely within the lesion, the subject got 48% correct, compared to 78% correct when the stimulus was outside the lesion. TSLO microperimetry revealed reduced, but detectible, sensitivity thresholds within the lesion.

CONCLUSIONS AND IMPORTANCE: Fundus-referenced visual testing proved useful to identify functional cones despite apparent photoreceptor loss identified using AOSLO and SD-OCT. While AOSLO and SD-OCT appear to be sensitive for the detection of abnormal or absent photoreceptors, changes in photoreceptors that are identified with these imaging tools do not correlate completely with visual function in every patient. Fundus-referenced vision testing is a useful tool to indicate the presence of cones that may be amenable to recovery or response to experimental therapies despite not being visible on confocal AOSLO or SD-OCT images.

Keywords: Adaptive optics scanning laser; Functional testing; Microperimetry; Multimodal imaging; Optical coherence tomography; ophthalmoscopy

Conflict of interest statement

Conflict of interest Austin Roorda has two patents on technology related to the Adaptive Optics Scanning Laser Ophthalmoscope. (USPTO #7,118,216 #6,890,076) These patents are assigned to both the Univ

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