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

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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 Rohan P. Bajaj 1

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Michael J. Fliotsos 1

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Tejus Pradeep 1

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Allen O. Eghrari 1,2

Received: 30 July 2020 / Revised: 9 October 2020 / Accepted: 15 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

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.

Rohan P. Bajaj and Michael J. Fliotsos contributed equally to this work. Supplementary Information The online version contains supplementary material available at https://doi.org/10.1007/s00417-020-04985-1. * Allen O. Eghrari [email protected] 1

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

2

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

Graefes Arch Clin Exp Ophthalmol

Key Messages Severity of Fuchs endothelial corneal dystrophy is often measured using methods that are limited in objectivity and repeatability, require dilation, or start at a non-zero baseline that prevents comparison of affected versus unaffected eyes. In a group of patients with varying clinical severity of Fuchs endothelial corneal dystrophy, we assessed clinical severity using the ratio of peripheral to central guttae counted in images obtained at a slit lamp. In thi