Visual Function in Pseudophakic Eyes with Fuchs' Endothelial Corneal Dystrophy.

PURPOSE: To assess vision in pseudophakic eyes with Fuchs' endothelial corneal dystrophy (FECD) before the onset of clinically detectable corneal edema. DESIGN: Cross-sectional study. METHODS: Sixty-one otherwise healthy pseudophakic eyes of 38 subjects with FECD (without clinically detectable edema) and 17 otherwise healthy pseudophakic eyes of 9 subjects with normal corneas. Subjects underwent clinical examination to determine the morphologic distribution of guttae (severity grading). Standardized best-corrected high-contract and low-contrast (photopic and mesopic) visual acuity (HCVA, LCVA) and straylight were measured. Scheimpflug tomography posterior elevation and pachymetry maps were interpreted for 3 tomographic features of subclinical edema: loss of regular isopachs, displacement of the thinnest point of the cornea, and presence of posterior surface depression. RESULTS: In FECD without tomographic features of edema (ie, normal tomography patterns), HCVA, LCVA, and straylight did not differ from that of eyes with normal corneas (P ≥ .09); these eyes encompassed the full range of severity grading of guttae. In FECD with all 3 tomographic features of edema, the same parameters were worse compared with eyes with normal corneas (P ≤ .02). In FECD with 1 or 2 tomographic abnormalities, mesopic LCVA (P = .04) and straylight (P = .003) were worse compared with eyes with normal corneas. CONCLUSIONS: Impairment of vision was associated with the presence of tomographic edema in eyes with FECD. When tomography patterns were normal in FECD (ie, guttae were present without tomographic edema), visual acuity and straylight were normal, and therefore corneal surgical intervention would not typically be indicated to improve vision.

Repeatability of Scheimpflug Tomography for Assessing Fuchs Endothelial Corneal Dystrophy.

PURPOSE: To determine if agreement between subjective interpretations of Scheimpflug tomography maps of corneas with Fuchs endothelial corneal dystrophy (FECD) is affected by daily and hourly changes in corneal edema. DESIGN: Reliability analysis. METHODS: Scheimpflug imaging pachymetry and posterior elevation maps of corneas with a range of severity of FECD were evaluated in a randomized manner for the presence of 3 tomographic features of edema. Agreement between interpretations of 1 masked observer was assessed (percentage, and κ-statistic with 95% confidence interval) for images taken within minutes on the same day, for images taken at a similar time on a different day, and for images taken over the course of a morning. Intra- and interobserver agreement was also assessed. RESULTS: Interpretations for individual tomographic features agreed for ≥88% of images (κ ≥ 0.75) taken within minutes on the same day; complete disagreement (ie, disagreement for all 3 tomographic features in an image) occurred in ≤3% of images. Interpretations agreed for ≥77% of images (κ ≥ 0.52) taken at a similar time on a different day; complete disagreement did not occur. Interpretations agreed for ≥81% of images (κ ≥ 0.61) taken over the course of a morning; complete disagreement occurred in ≤6% of images. Intraobserver agreement was ≥93% (κ ≥ 0.83) and interobserver agreement was ≥93% (κ ≥ 0.66); complete disagreement did not occur. CONCLUSIONS: Subjective interpretation of Scheimpflug images in FECD is highly repeatable for disease classification. Although small variations in interpretations resulted from pathophysiologic changes in corneal hydration and other factors, clinically significant disagreements in interpretation were uncommon and therefore unlikely to affect clinical decision-making.

Predicting the Prognosis of Fuchs Endothelial Corneal Dystrophy by Using Scheimpflug Tomography.

PURPOSE: To determine if Scheimpflug tomography pachymetry map and posterior elevation map patterns, central corneal thickness (CCT), and corneal backscatter can predict the prognosis of Fuchs endothelial corneal dystrophy (FECD). DESIGN: Cross-sectional study with follow-up of outcomes. PARTICIPANTS: Ninety-six eyes (56 subjects) with a range of severity of FECD. METHODS: Corneas were graded by cornea specialists according to the area and confluence of guttae and the presence of clinically definite edema. Masked and randomized Scheimpflug imaging pachymetry map and posterior elevation map patterns were assessed by 1 observer for loss of regular isopachs, displacement of the thinnest point of the cornea, and the presence of posterior surface depression. The prognosis of eyes over a 5-year (median) follow-up period was determined based on FECD progression (new onset of clinically definite edema or ≥5% increase in CCT) or intervention by endothelial keratoplasty. Cumulative probabilities of progression and intervention were estimated from survival analyses, with risk factors determined by using Cox proportional hazards models. MAIN OUTCOME MEASURES: Pachymetry map and posterior elevation map patterns, corneal backscatter, and CCT (ultrasonic pachymetry). RESULTS: In univariate analyses, loss of regular isopachs (hazard ratio [HR], 18.00) displacement of the thinnest point (HR, 11.53), focal posterior surface depression (HR, 10.21), and anterior corneal backscatter (HR, 1.22, per 1-grayscale unit increment), were risk factors for progression or intervention (P < 0.001), whereas CCT (HR, 1.30, per 25-µm increment) was not (P = 0.15). In multivariate analyses, loss of regular isopachs (HR, 11.57; P < 0.001) and displacement of the thinnest point (HR, 5.61; P = 0.02) were independent and clinically important risk factors for progression and intervention. The 5-year cumulative risk of disease progression and intervention was 7%, 48%, and 89% when none, 1 or 2, and all 3 pachymetry map and posterior elevation map parameters were present, respectively (P <0.001). The 4-year cumulative risk of disease progression and intervention after uncomplicated cataract surgery was 0%, 50%, and 75% when none, 1 or 2, and all 3 pachymetry map and posterior elevation map parameters were present, respectively (P < 0.001). CONCLUSIONS: Three Scheimpflug tomography pachymetry map and posterior elevation map patterns can predict FECD prognosis independent of CCT. The risk of FECD progression and intervention, including after uncomplicated cataract surgery, increases according to the number of parameters present.