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Peripheral-to-central ratio of Guttae: validity and reliability of an objective method to characterize severity of Fuchs endothelial corneal dystrophy

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Abstract

Purpose

Common methods of measuring severity of Fuchs endothelial corneal dystrophy (FECD) are limited in objectivity, reliability, or start with a variable baseline that prevents distinguishing healthy from affected eyes. The aim of this study was to describe a method of grading FECD that overcomes these limitations.

Methods

Fifteen patients with Fuchs endothelial corneal dystrophy were included in the study. Guttae were imaged with a slit lamp beam 8 mm tall; the bottom 4 mm half of each image was divided into two equally-sized sections. Guttae were counted by four independent graders blinded to disease severity scores. The peripheral:central guttae ratio was compared to modified Krachmer clinical severity scores. The peripheral:central guttae ratio was compared between mild (severity 0.5–3) versus moderate-to-severe (severity 4–5) disease. Receiver operating characteristics defined optimal ratio cutoffs for mild versus moderate-to-severe disease.

Results

Increased peripheral guttae and peripheral:central guttae ratio correlated with Krachmer severity (p = 0.021 and p = 0.009, respectively). The difference between mild and moderate-to-severe cases for the peripheral:central guttae ratio was significant (p < 0.001). Inter-rater reliability of total guttae count was high (coefficient = 0.82, p < 0.001). A peripheral:central guttae ratio of 0.16 was the ideal cut-off point (area under the curve = 0.79, sensitivity = 0.78, and specificity = 0.80).

Conclusion

In this pilot study, the peripheral:central ratio of guttae correlates with subjective clinical severity of Fuchs dystrophy. It starts at a common baseline, has good inter-rater reliability, does not require dilation, and can be conducted with a smartphone and slit-lamp.

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Data availability

Study data is maintained by the investigators and is available upon request.

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Funding

No funding was received for conducting this study. Allen O Eghrari is provided salary support by Research to Prevent Blindness Sybil B. Harrington Special Scholar Award and the Tolsma family. All other authors have no funding sources to disclose.

Author information

Author notes

  1. Rohan P. Bajaj and Michael J. Fliotsos contributed equally to this work.

Authors and Affiliations

  1. Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Rohan P. Bajaj, Michael J. Fliotsos, Tejus Pradeep & Allen O. Eghrari

  2. Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD, 21287, USA

    Allen O. Eghrari

Authors
  1. Rohan P. Bajaj
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  2. Michael J. Fliotsos
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  3. Tejus Pradeep
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  4. Allen O. Eghrari
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Contributions

Rohan P Bajaj (ORCID 0000–0002–3934-3444): data curation; formal analysis; conceptualization; methodology.

Michael J Fliotsos (ORCID 0000–0001–8429-0831): writing – original draft; writing – review & editing; visualization; project administration.

Tejus Pradeep (ORCID 0000–0002-0506-069X): formal analysis; writing – review & editing.

Allen O Eghrari (ORCID 0000–0003-2798-038X): supervision; conceptualization; methodology; validation; data curation.

Corresponding author

Correspondence to Allen O. Eghrari.

Ethics declarations

Conflict of interest

Allen O Eghrari has an ownership interest in Treyetech and LuckyVision, LLC. All other authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Informed consent / ethical approval

This study was reviewed by the Johns Hopkins University School of Medicine Institutional Review Board (IRB) under study identifier IRB00217273. All study activities adhered to the Declaration of Helsinki. The study was deemed exempt by the IRB under the United States Department of Health and Human Services (DHHS) regulations as a retrospective review of images and data existing in the medical record. The IRB, acting as a HIPAA (Health Insurance Portability and Accountability Act) Privacy Board, reviewed and approved the HIPAA consent waiver via the expedited mechanism as “secondary research” under 45 CFR 46.104(d)(4)(iii) of DHHS regulations.

Sources of support

A. O. Eghrari is supported by the Research to Prevent Blindness Sybil B. Harrington Special Scholar Award and the Tolsma family.

The remaining authors have no funding disclosures to report.

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Supplemental Fig. 1

Receiving operating characteristics curve for peripheral:central ratio of guttae. (JPG 35 kb).

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Bajaj, R.P., Fliotsos, M.J., Pradeep, T. et al. Peripheral-to-central ratio of Guttae: validity and reliability of an objective method to characterize severity of Fuchs endothelial corneal dystrophy. Graefes Arch Clin Exp Ophthalmol 259, 685–690 (2021). https://doi.org/10.1007/s00417-020-04985-1

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  • DOI: https://doi.org/10.1007/s00417-020-04985-1

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