Current Applications of Artificial Intelligence for Fuchs Endothelial Corneal Dystrophy: A Systematic Review

Abstract

Purpose: Fuchs endothelial corneal dystrophy (FECD) is a common, age-related cause of visual impairment. This systematic review synthesizes evidence from the literature on artificial intelligence (AI) models developed for the diagnosis and management of FECD.

Methods: We conducted a systematic literature search in MEDLINE, PubMed, Web of Science, and Scopus from January 1, 2000, to June 31, 2024. Full-text studies utilizing AI for various clinical contexts of FECD management were included. Data extraction covered model development, predicted outcomes, validation, and model performance metrics. We graded the included studies using the Quality Assessment of Diagnostic Accuracies Studies 2 tool. This review adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations.

Results: Nineteen studies were analyzed. Primary AI algorithms applied in FECD diagnosis and management included neural network architectures specialized for computer vision, utilized on confocal or specular microscopy images, or anterior segment optical coherence tomography images. AI was employed in diverse clinical contexts, such as assessing corneal endothelium and edema and predicting post-corneal transplantation graft detachment and survival. Despite many studies reporting promising model performance, a notable limitation was that only three studies performed external validation. Bias introduced by patient selection processes and experimental designs was evident in the included studies.

Conclusions: Despite the potential of AI algorithms to enhance FECD diagnosis and prognostication, further work is required to evaluate their real-world applicability and clinical utility.

Translational relevance: This review offers critical insights for researchers, clinicians, and policymakers, aiding their understanding of existing AI research in FECD management and guiding future health service strategies.

Figure 1.

PRISMA flow diagram outlining the study selection process for the systematic review. This diagram illustrates the steps taken to include and exclude studies based on predefined eligibility criteria, highlighting the number of studies at each stage of the review process, including identification, screening, eligibility, and inclusion.

Figure 2.

Heatmap of performance metrics across studies. Rows represent individual studies, and columns indicate accuracy, Dice coefficient, sensitivity, and specificity. Color intensity reflects performance, with darker shades indicating higher values. Metrics are categorized as ≤0.50 (lightest blue), 0.50 to 0.70 (light blue), 0.70 to 0.85 (medium blue), and ≥0.85 (darkest blue). Gray cells indicate that the corresponding study did not report this metric. High-performing models and variability in evaluation criteria can be readily observed in this figure. Comprehensive details on datasets, AI pipelines, and additional evaluation metrics are provided in the Table, which contextualizes these results within the broader systematic review.

Figure 3.

Summary diagram presenting the various AI approaches used in the management of FECD. The diagram categorizes the AI algorithms employed in different clinical contexts, including the assessment of corneal endothelium, evaluation of corneal edema, and prognostication of post-corneal transplantation graft detachment and rejection. This overview highlights the application of AI methods within clinical workflow for managing FECD.

Figure 4.

Slit-scanning in vivo confocal microscopy images of the corneal endothelium. (A) Healthy control cornea. Image of a normal corneal endothelium shows a uniform hexagonal cell pattern with consistent cell density and no visible guttae or abnormalities (white solid arrowhead). (B) Early-stage FECD. Image shows isolated guttae (white dashed arrowhead), characterized by dark, round bodies with central white hyperreflectivity, indicating early endothelial cell distress. (C) Late-stage FECD. Increased confluency of guttae (white dashed arrowhead), illustrating a more extensive presence of these structures (dashed-line highlighted area) with evidence of endothelial cell loss.

Figure 5.

Cross-sectional AS-OCT images of the cornea in FECD. (A) Early-stage FECD. AS-OCT image of the cornea shows no clinical signs of corneal edema. The corneal thickness remains within the normal range, and no significant epithelial or stromal changes are visible. (B) Late-stage FECD. AS-OCT image shows increased central corneal thickness, epithelial bullae (fluid-filled cystic spaces), and increased pixel intensity (backscatter) in the stroma, indicating corneal decompensation and edema.

Figure 6.

(A, B) Cross-sectional AS-OCT images of the cornea showing a small partial graft detachment after DMEK (A) and a more extensive graft detachment (B).