Early detection of cone photoreceptor cell loss in retinitis pigmentosa using adaptive optics scanning laser ophthalmoscopy

  • Shunji Nakatake
  • Yusuke MurakamiEmail author
  • Jun Funatsu
  • Yoshito Koyanagi
  • Masato Akiyama
  • Yukihide Momozawa
  • Tatsuro Ishibashi
  • Koh-Hei Sonoda
  • Yasuhiro Ikeda
Retinal Disorders



The purpose of the study was to investigate the characteristics of the parafoveal cone density changes in patients with retinitis pigmentosa (RP) using adaptive optics scanning laser ophthalmoscopy (AO-SLO).


A total of 14 eyes of RP patients and 10 eyes of control subjects were examined. High-resolution images of cone photoreceptor cells were obtained with a Canon AO-SLO system in the four retinal regions of the superior, inferior, temporal, and nasal areas located 1.0 mm from the central fovea. The relationships of cone density with optical coherence tomography (OCT) findings and the visual sensitivity of the static perimetry tests were analyzed in RP patients.


The averaged cone densities in RP patients were decreased at 1.0 mm eccentricity from the fovea (11,899 cells/mm2) compared with those in control subjects (16,647 cells/mm2; P < 0.01). The cone density was substantially decreased even in RP patients with an intact interdigitation zone at the examined area (12,865 cells/mm2; P < 0.01 vs. controls) and preserved visual sensitivity with > 35 dB (13,019 cells/mm2; P < 0.001 vs. controls).


In RP, cone photoreceptor cell loss occurred in the parafoveal region with a preserved EZ/IZ or visual sensitivity. AO-SLO may be a useful modality to detect early changes of cone photoreceptor cells in RP patients.


Retinitis pigmentosa Cone photoreceptor cell Cone density Adaptive optics scanning laser ophthalmoscopy (AO-SLO) 



We thank K. Fujiwara, Y. Kaizu, and T. Hisatomi for their support for data analysis and K. Nomura and T. Kitamura for their technical assistance with the AO-SLO imaging.


This work was supported by a Japanese Ministry of Education, Culture, Sports, Science, and Technology grant (no. 16H06268 to YM), a Japan Intractable Disease Research Foundation grant (to YM), a Japanese Retinitis Pigmentosa Society grant (to YM), and a Rohto Award (to YM).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.

Supplementary material

417_2019_4307_MOESM1_ESM.pdf (30 kb)
Fig 1 The hexagon ratio and average (Ave) nearest-neighbor distance (NND)/expected (Exp) NND ratio of control subjects and RP patients. The quantification of the hexagon ratio (A) and the Ave NND/Exp NND ratio (B) of control subjects and RP patients. Data are presented as whisker-box-plots. The central horizontal bars indicate the medians, boxes indicate 25th to 75th percentiles, and whiskers indicate 1.5 times the interquartile range from the bottom and the top of the box. Wilcoxon rank sum tests were performed to assess significance. **p < 0.01. (PDF 30 kb)
417_2019_4307_MOESM2_ESM.pdf (30 kb)
Fig 2 The cone density according to the status of FAF ring at 1.0 mm eccentricity from the fovea in RP patients. The quantification of the average cone density of normal subjects, RP patients FAF ring wider than 1.0 mm or narrower than 1.0 mm from foveas. Data are presented as whisker-box-plots. The central horizontal bars indicate the medians, boxes indicate the 25th to 75th percentiles, and whiskers indicate 1.5 times the interquartile range from the bottom and the top of the box. Steel’s test was performed to assess significance. **p < 0.01. (PDF 29 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Shunji Nakatake
    • 1
  • Yusuke Murakami
    • 1
    Email author
  • Jun Funatsu
    • 1
  • Yoshito Koyanagi
    • 1
  • Masato Akiyama
    • 1
  • Yukihide Momozawa
    • 2
  • Tatsuro Ishibashi
    • 1
  • Koh-Hei Sonoda
    • 1
  • Yasuhiro Ikeda
    • 1
  1. 1.Department of Ophthalmology, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  2. 2.Laboratory for Genotyping Development, RIKEN Center for Integrative Medical SciencesYokohamaJapan

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