Fundus autofluorescence and ellipsoid zone (EZ) line width can be an outcome measurement in RHO-associated autosomal dominant retinitis pigmentosa

  • Vitor K. L. Takahashi
  • Júlia T. Takiuti
  • Jose R. L. Carvalho-Jr
  • Christine L. Xu
  • Jimmy K. Duong
  • Vinit B. Mahajan
  • Stephen H. TsangEmail author
Retinal Disorders



To evaluate the progression of retinitis pigmentosa (RP) due to mutations in rhodopsin (RHO) by measuring the short-wavelength autofluorescence (SW-AF) increased autofluorescence ring and ellipsoid zone (EZ)-line width.


Fundus autofluorescence (FAF) and spectral domain optical coherence tomography (SD-OCT) images were obtained from 10 patients with autosomal dominant RP due to mutations in the RHO gene. Measurements of ring area on FAF images, as well as the EZ line width on SD-OCT images and horizontal, vertical diameter, were performed by two independent masked graders.


The ring area decreased by a rate of 0.6 ± 0.2 mm2 per year. We observed that the EZ line width decreased by an average of 152 ± 37 μm per year, while the horizontal and vertical diameters decreased by 106 ± 35 μm and 125 ± 29 μm per year, respectively. Progression rates were similar between eyes.


We observed SW-AF ring constriction and a progressive loss of EZ line width over time.


Retinitis pigmentosa RHO Autosomal dominant Disease progression 



The Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory are supported by the National Institutes of Health [P30EY019007, R01EY018213, R01EY024698, R01EY026682, R21AG050437], National Cancer Institute Core [5P30CA013696], Foundation Fighting Blindness [TA-NMT-0116-0692-COLU], the Research to Prevent Blindness (RPB) Physician-Scientist Award, and unrestricted funds from RPB, New York, NY, USA. S.H.T. is a member of the RD-CURE Consortium and is supported by ARVO/Genentech, Kobi and Nancy Karp, the Crowley Family Fund, the Rosenbaum Family Foundation, the Tistou and Charlotte Kerstan Foundation, the Schneeweiss Stem Cell Fund, New York State [C029572], and the Gebroe Family Foundation.

Compliance with ethical standards

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.

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

417_2018_4234_MOESM1_ESM.docx (19 kb)
ESM 1 (DOCX 19 kb)


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Copyright information

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

Authors and Affiliations

  • Vitor K. L. Takahashi
    • 1
    • 2
    • 3
  • Júlia T. Takiuti
    • 1
    • 2
    • 4
  • Jose R. L. Carvalho-Jr
    • 1
    • 2
    • 3
    • 5
  • Christine L. Xu
    • 1
    • 2
  • Jimmy K. Duong
    • 6
  • Vinit B. Mahajan
    • 7
    • 8
  • Stephen H. Tsang
    • 1
    • 2
    • 9
    • 10
    Email author return OK on get
  1. 1.Department of OphthalmologyColumbia UniversityNew YorkUSA
  2. 2.Jonas Children’s Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Departments of Ophthalmology, Pathology & Cell Biology, Columbia Stem Cell Initiative, Institute of Human NutritionColumbia UniversityNew YorkUSA
  3. 3.Department of OphthalmologyFederal University of São PauloSao PauloBrazil
  4. 4.Division of OphthalmologyUniversity of São Paulo Medical SchoolSao PauloBrazil
  5. 5.Empresa Brasileira de Servicos Hospitalares (EBSERH) - Hospital das Clinicas de Pernambuco (HCPE), Departament of OphthalmologyFederal University of Pernambuco (UFPE)RecifeBrazil
  6. 6.Department of BiostatisticsColumbia UniversityNew YorkUSA
  7. 7.Omics Laboratory, Department of Ophthalmology, Byers Eye InstituteStanford University School of MedicinePalo AltoUSA
  8. 8.Veterans Affairs Palo Alto Health Care SystemPalo AltoUSA
  9. 9.Department of Pathology & Cell Biology, Stem Cell Initiative (CSCI), College of Physicians and Surgeons, Institute of Human NutritionColumbia UniversityNew YorkUSA
  10. 10.Columbia University Medical CenterHarkness Eye InstituteNew YorkUSA

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