April 2014
Volume 55, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2014
Disambiguation of photoreceptor structure in transition zones of retinal degenerative diseases
Author Affiliations & Notes
  • Fouad R Zakka
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
  • Drew H Scoles
    Biomedical Engineering, University of Rochester, Rochester, NY
  • Christopher S Langlo
    Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, Milwaukee, WI
  • Benjamin S Liu
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
  • Dennis P Han
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
  • Kimberly E Stepien
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
  • Thomas B Connor
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
  • Alfredo Dubra
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
    Biophysics, Medical College of Wisconsin, Milwaukee, WI
  • Joseph Carroll
    Ophthalmology, Medical College of Wisconsin, Milwaukee, WI
    Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, Milwaukee, WI
  • Footnotes
    Commercial Relationships Fouad Zakka, None; Drew Scoles, None; Christopher Langlo, None; Benjamin Liu, None; Dennis Han, None; Kimberly Stepien, None; Thomas Connor, None; Alfredo Dubra, Canon USA Inc (C), US Patent 8,226,236 (P); Joseph Carroll, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science April 2014, Vol.55, 1591. doi:
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    • Get Citation

      Fouad R Zakka, Drew H Scoles, Christopher S Langlo, Benjamin S Liu, Dennis P Han, Kimberly E Stepien, Thomas B Connor, Alfredo Dubra, Joseph Carroll; Disambiguation of photoreceptor structure in transition zones of retinal degenerative diseases. Invest. Ophthalmol. Vis. Sci. 2014;55(13):1591.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract
 
Purpose
 

Inherited retinal degenerations are commonly associated with transition zones between normal and pathologic regions. Analysis of retinal structure within these zones provides important information about disease mechanism at the cellular level. Confocal adaptive optics scanning light ophthalmoscopy (AOSLO) imaging shows numerous reflective structures in these regions which may be erroneously identified as cones. This study uses non-confocal AOSLO imaging techniques to expose the origin of the reflective structures in transition zones and provide a more definitive assessment of residual cone photoreceptor structure.

 
Methods
 

Two subjects with Stargardt’s disease (STGD), 1 subject with retinitis pigmentosa (RP), and 1 subject with Usher’s syndrome were imaged using confocal, dark-field, and split-detector AOSLO (custom-built, 790nm light). Spectral domain ocular coherence tomography (SD-OCT) images were acquired and aligned to the AOSLO.

 
Results
 

At the transition zones of the RP and Usher’s subjects, confocal AOSLO shows scattered reflective structures that are difficult to define (Fig 1A). Using dark-field and split-detector modalities reveals an increasingly visible RPE mosaic and a decreasing photoreceptor density, respectively, through the transition zone (Fig 1B, C). The loss of photoreceptors correlates with the tapering of the ellipsoid zone seen on SD-OCT in the same region (Fig 1D). Confocal AOSLO of the transition zone in the two patients with STGD showed numerous reflective structures that resemble cone photoreceptors (Fig 2A). Split-detector AOSLO of this region revealed a majority of rods, scarce cones, possible large lipid-engorged RPE cells, and a significant area of debris (Fig 2B).

 
Conclusions
 

Reflective retinal structures in transition zones of inherited retinal degenerations could be wrongly attributed to photoreceptors when assessed solely with confocal AOSLO. Reflective RPE cells or debris can be better differentiated from surviving photoreceptors when a multimodal AOSLO imaging approach is applied in patients with these degenerative diseases.

 
 
A section of the transition zone in RP viewed with A) Confocal, B) Dark-field, C) Split-detector en face AOSLO, and D) B-scan SD-OCT. Scale bar 100µm
 
A section of the transition zone in RP viewed with A) Confocal, B) Dark-field, C) Split-detector en face AOSLO, and D) B-scan SD-OCT. Scale bar 100µm
 
 
Transition zone in STGD. A) Many reflective structures in confocal AOSLO. B) Split-detector AOSLO reveals only scarce cones (arrow), clusters of enlarged rods and debris. Scale bar 50µm
 
Transition zone in STGD. A) Many reflective structures in confocal AOSLO. B) Split-detector AOSLO reveals only scarce cones (arrow), clusters of enlarged rods and debris. Scale bar 50µm
 
Keywords: 550 imaging/image analysis: clinical • 696 retinal degenerations: hereditary • 648 photoreceptors  
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