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Documenta Ophthalmologica

, Volume 137, Issue 1, pp 15–23 | Cite as

Deferoxamine-induced electronegative ERG responses

  • Ruben Jauregui
  • Karen Sophia Park
  • Alexander G. Bassuk
  • Vinit B. Mahajan
  • Stephen H. Tsang
Clinical Case Report

Abstract

Purpose

To report a case of deferoxamine-induced retinopathy characterized by electroretinography (ERG), optical coherence tomography angiography (OCT-A), and other multimodal imaging.

Methods

This is an observational case report of one patient. Full-field ERG was performed. OCT-A, spectral-domain optical coherence tomography (SD-OCT), color fundus photography, and fundus autofluorescence were used to characterize the retinopathy induced by deferoxamine use.

Results

A 64-year-old man with a history of β-thalassemia intermedia presented with worsening visual acuity, nyctalopia, and electronegative ERG. OCT-A revealed atrophy of the choriocapillaris in areas of hypoautofluorescence, corresponding to regions of retinal atrophy. SD-OCT showed disruption of the ellipsoid zone, granular hyperreflective deposits within the retinal pigment epithelium, thinning of the retinal layers, and extensive choroidal sclerosis and atrophy of the retinal pigment epithelium.

Conclusion

Deferoxamine-induced retinopathy can manifest with electronegative maximal ERG responses, and OCT-A can be used to detect deferoxamine toxicity.

Keywords

Deferoxamine Retinopathy Electronegative OCT-A Multimodal imaging 

Notes

Acknowledgements

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], the Research to Prevent Blindness (RPB) Physician-Scientist Award, unrestricted funds from RPB, New York, NY, USA. R. J. is supported by the RPB medical student eye research fellowship. S. H. T. is a member of the RD-CURE Consortium and is supported by the Tistou and Charlotte Kerstan Foundation, the Schneeweiss Stem Cell Fund, New York State [C029572], and the Gebroe Family Foundation. VBM is supported by NIH Grants [R01EY024665, R01EY025225, R01EY024698, R21AG050437, P30EY026877], and Research to Prevent Blindness (RPB).

Author Contributions

R.J. collected and interpreted patient data and images. R.J. and K.S.P. composed the manuscript. S.H.T., A.G.B., and V.B.M. conceived the experimental design and approved the final interpretation of the data.

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflicts of interest.

Informed consent

The patient has given informed consent for submission of this case report to the journal.

Statement of human rights

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.

Statement on the welfare of animals

This article does not contain any studies with animals performed by any of the authors.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of OphthalmologyColumbia UniversityNew YorkUSA
  2. 2.Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma LaboratoryNew YorkUSA
  3. 3.Weill Cornell Medical CollegeNew YorkUSA
  4. 4.Department of PediatricsUniversity of IowaIowa CityUSA
  5. 5.Byers Eye Institute, Omics Laboratory, Department of OphthalmologyStanford University School of MedicinePalo AltoUSA
  6. 6.Palo Alto Veterans AdministrationPalo AltoUSA
  7. 7.Department of Pathology and Cell Biology, Stem Cell Initiative (CSCI), Institute of Human Nutrition, College of Physicians and SurgeonsColumbia UniversityNew YorkUSA
  8. 8.Edward S. Harkness Eye InstituteColumbia University Medical CenterNew YorkUSA

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