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Model predicts progression of primary cornea guttata to Fuchs' corneal dystrophy
Invest Ophthalmol Vis Sci. 2011;52(11):7888-7893.
A mathematical prediction model provided a reliable forecast of disease progression in Fuchs' corneal dystrophy, a large study found.
The development of cornea guttata and its progression to Fuchs' corneal dystrophy are largely unknown, the study authors said.
"A retrospective study with a large database and an adequate mathematical model can be used ... to predict the prognosis of patients with guttata cornea," they said. "Our mathematical model is a simple method for predicting the prognosis of patients with guttata cornea."
The study included 3,281 eyes of 1,971 patients classified according to disease severity: stage 0, no guttae; stage 1, guttata cornea without edema; stage 2, mild Fuchs' dystrophy; and stage 3, severe Fuchs' dystrophy. Patient age and endothelial cell density were also recorded.
The mathematical prediction model was based on endothelial cell density (ECD) of 3,600 cells/mm2 and the annual decrease rate of endothelial cell density from 5 years of age being constant within each disease stage but different between stages.
The model enabled the plotting of an age-ECD curve within each class of disease progression and an age-ECD curve of optimal ECD decrease.
Study results showed that the annual rate of ECD decrease was 0.44% in eyes with stage 0 disease, 0.81% in eyes with stage 1 disease, 2.65% in eyes with stage 2 disease and 3.08% in eyes with stage 3 disease.
The age-ECD loss curves of 1.4% per year and 2% per year delineated stage 1 disease into three subgroups: asymptomatic guttata cornea, borderline guttata cornea and pre-Fuchs' corneal dystrophy, the authors said.
Based on mathematical modeling, the authors propose that a subset of patients with primary guttata cornea or stage 1 Fuchs’ corneal dystrophy experience a rate of endothelial cell loss similar to that in normal eyes, whereas another subset experiences an accelerated rate of endothelial cell loss from early childhood onward. They further developed a classification system to distinguish these subsets based on endothelial cell density cut-off levels associated with decades of age. Interestingly, they measured endothelial cell densities of up to 1,000 cells/mm2 in corneas with stromal and epithelial edema and bullae. However, the automated endothelial cell count software that was utilized may have had difficulty accurately detecting the cell borders in the presence of significant guttae. This cross-sectional study was performed in a Japanese population, and results in Caucasians may differ given well-recognized differences in racial prevalence.
– Marianne O. Price, PhD
Executive
Director, Cornea Research Foundation of America, Indianapolis
Disclosure:
Dr. Price has no relevant financial disclosures.