Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies

Abstract This report explores the prevalence of keratoconus in a population-based cohort of adults aged 40 or older according to ten different definitions. All Rotterdam Study participants with reliable Pentacam scans and no prior corneal refractive surgery were cross-sectionally analysed (n = 2660)...

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Main Authors: Hasan Shabani, Bart T. H. van Dooren, Magda A. Meester-Smoor, Annette A. J. M. Geerards, Caroline C. W. Klaver, Wishal D. Ramdas
Format: Article
Language:English
Published: Nature Portfolio 2025-01-01
Series:Scientific Reports
Online Access:https://doi.org/10.1038/s41598-025-87532-4
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author Hasan Shabani
Bart T. H. van Dooren
Magda A. Meester-Smoor
Annette A. J. M. Geerards
Caroline C. W. Klaver
Wishal D. Ramdas
author_facet Hasan Shabani
Bart T. H. van Dooren
Magda A. Meester-Smoor
Annette A. J. M. Geerards
Caroline C. W. Klaver
Wishal D. Ramdas
author_sort Hasan Shabani
collection DOAJ
description Abstract This report explores the prevalence of keratoconus in a population-based cohort of adults aged 40 or older according to ten different definitions. All Rotterdam Study participants with reliable Pentacam scans and no prior corneal refractive surgery were cross-sectionally analysed (n = 2660). First, we applied a novel evidence-based definition. Suspected keratoconus was defined as having at least one eye with a final D-index (BAD-D) ≥ 2.6. Manifest keratoconus was defined as having at least one eye with: (1) BAD-D ≥ 2.6; and (2) a score of at least 4/10 on the novel Rotterdam Keratoconus Scale (RKS); and (3) a confirming assessment of the relevant Pentacam maps; and (4) meeting Holladay’s criteria in case of recent contact lens usage. Using this proposed definition, 72 participants (2.71%, 95%CI: 2.16–3.40%) had suspected keratoconus, while 10 participants (0.38%, 95%CI: 0.20–0.69%) had manifest keratoconus. To assess reproducibility, two specialists independently applied the proposed definition, with a substantial inter-observer agreement (Kappa = 0.74). Interestingly, 6(60%) patients were unaware of having keratoconus. Applying nine alternative definitions from similar screening studies produced prevalence estimates ranging from 0.19 to 9.29% in the same cohort. Moreover, counting solely on a BAD-D cutoff of 2.6 to define keratoconus was unreliable, with a low positive predictive value of 14%. These findings explain partially the large heterogeneity in the reported keratoconus prevalences, underscoring the need for a standardized definition.
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spelling doaj-art-13bc099c80f1430cae16c515ef1bf5392025-02-02T12:22:12ZengNature PortfolioScientific Reports2045-23222025-01-0115111010.1038/s41598-025-87532-4Different keratoconus definitions can lead to substantial prevalence disparities in population-based studiesHasan Shabani0Bart T. H. van Dooren1Magda A. Meester-Smoor2Annette A. J. M. Geerards3Caroline C. W. Klaver4Wishal D. Ramdas5Department of Ophthalmology, Erasmus Medical CentreDepartment of Ophthalmology, Erasmus Medical CentreDepartment of Ophthalmology, Erasmus Medical CentreCornea Centre, The Rotterdam Eye HospitalDepartment of Ophthalmology, Erasmus Medical CentreDepartment of Ophthalmology, Erasmus Medical CentreAbstract This report explores the prevalence of keratoconus in a population-based cohort of adults aged 40 or older according to ten different definitions. All Rotterdam Study participants with reliable Pentacam scans and no prior corneal refractive surgery were cross-sectionally analysed (n = 2660). First, we applied a novel evidence-based definition. Suspected keratoconus was defined as having at least one eye with a final D-index (BAD-D) ≥ 2.6. Manifest keratoconus was defined as having at least one eye with: (1) BAD-D ≥ 2.6; and (2) a score of at least 4/10 on the novel Rotterdam Keratoconus Scale (RKS); and (3) a confirming assessment of the relevant Pentacam maps; and (4) meeting Holladay’s criteria in case of recent contact lens usage. Using this proposed definition, 72 participants (2.71%, 95%CI: 2.16–3.40%) had suspected keratoconus, while 10 participants (0.38%, 95%CI: 0.20–0.69%) had manifest keratoconus. To assess reproducibility, two specialists independently applied the proposed definition, with a substantial inter-observer agreement (Kappa = 0.74). Interestingly, 6(60%) patients were unaware of having keratoconus. Applying nine alternative definitions from similar screening studies produced prevalence estimates ranging from 0.19 to 9.29% in the same cohort. Moreover, counting solely on a BAD-D cutoff of 2.6 to define keratoconus was unreliable, with a low positive predictive value of 14%. These findings explain partially the large heterogeneity in the reported keratoconus prevalences, underscoring the need for a standardized definition.https://doi.org/10.1038/s41598-025-87532-4
spellingShingle Hasan Shabani
Bart T. H. van Dooren
Magda A. Meester-Smoor
Annette A. J. M. Geerards
Caroline C. W. Klaver
Wishal D. Ramdas
Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies
Scientific Reports
title Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies
title_full Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies
title_fullStr Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies
title_full_unstemmed Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies
title_short Different keratoconus definitions can lead to substantial prevalence disparities in population-based studies
title_sort different keratoconus definitions can lead to substantial prevalence disparities in population based studies
url https://doi.org/10.1038/s41598-025-87532-4
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