Relationship of Fuchs Endothelial Corneal Dystrophy Severity to Central Corneal Thickness

Kopplin, Laura J.; Przepyszny, Katie; Schmotzer, Brian; Rudo, Karen; Babineau, Denise C.; Patel, Sanjay; Verdier, David D.; Jurkūnas, Ūla V.; Iyengar, Sudha K.; Lass, Jonathan H.

doi:10.1001/archophthalmol.2011.1626

Cited by 41 publications

(24 citation statements)

References 29 publications

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“…Nevertheless, corneas in the early stages of Fuchs dystrophy appear to be thicker than normal, 7,31 suggesting that subclinical edema is present early in the disease because of early endothelial dysfunction. Thus, the anterior corneal abnormalities may be secondary to chronic subclinical corneal edema.…”

Section: Discussionmentioning

confidence: 99%

Corneal Abnormalities Early in the Course of Fuchs' Endothelial Dystrophy

et al. 2014

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Purpose Corneas with advanced Fuchs endothelial dystrophy that require endothelial keratoplasty manifest anterior corneal structural and cellular abnormalities that have been associated with visual deficits both before and after endothelial keratoplasty. In this study, we determined the onset of these abnormalities in the course of the disease. Design Cross-sectional study. Participants Sixty-three eyes (39 subjects) with a range of severity of Fuchs dystrophy, and 25 eyes (13 subjects) with normal corneas. Methods All corneas were examined by using slit-lamp biomicroscopy, ultrasonic pachymetry, and confocal microscopy. The clinical grade of Fuchs dystrophy was assessed according to the presence and extent of guttae and clinically evident edema, and was categorized as mild (grades 1–2), moderate (grades 3–4), or advanced (grades 5–6). Normal corneas were devoid of any central guttae (grade 0). Corneal backscatter (haze) was measured from the confocal image light intensity profile. Stromal cell density and number, and the presence of abnormal subepithelial cells, were determined from confocal images. Comparisons between groups were made by using generalized estimating equation models to account for any correlation between fellow eyes of the same subject. Main Outcome Measures Anterior corneal backscatter, stromal cell density and number, presence of subepithelial cells, central corneal thickness. Results Anterior corneal backscatter was 18–67% higher in moderate and advanced Fuchs dystrophy compared to normal (p≤0.003); a similar trend was noted in mild Fuchs dystrophy compared to normal (p=0.08). Stromal cell density and the absolute number of stromal cells in the anterior 10% of the stroma were approximately 20% and 27% lower, respectively, in Fuchs dystrophy (regardless of severity) compared to normal (p<0.001). Abnormal subepithelial cells were visible in 9%, 19%, and 30% of corneas with mild, moderate, and advanced Fuchs dystrophy, respectively. Only corneas with advanced Fuchs dystrophy were thicker than normal (p<0.001). Conclusions Anterior corneal cellular and structural abnormalities begin early in the course of Fuchs dystrophy, prior to the onset of clinically evident edema. The chronicity of these changes can explain their incomplete resolution after endothelial keratoplasty, and understanding the onset of these might help to determine the optimal time to intervene to achieve best outcomes.

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Section: Discussionmentioning

confidence: 99%

Corneal Abnormalities Early in the Course of Fuchs' Endothelial Dystrophy

et al. 2014

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“…Kopplin et al have shown that in eyes with Fuchs dystrophy without slit lamp evidence of corneal edema, increasing CT is associated with increasing guttae. 17 …”

Section: Discussionmentioning

confidence: 99%

“…While the association between increased intraocular pressure and increased corneal thickness has been well documented 17, 19 , the causal relationship appears to be complex, with mechanisms not well understood. Patients with ocular hypertension are more likely to have increased CT 20 , in contrast to normal tension glaucoma or primary open angle glaucoma patients who are more likely to have average or lower than average CT. 21 It is unclear which of our patients with elevated intraocular pressure readings within the first postoperative month may have had ocular hypertension, primary open angle glaucoma, angle closure, or were steroid responders.…”

Section: Discussionmentioning

confidence: 99%

Corneal Thickness as a Predictor of Corneal Transplant Outcome

Verdier

Sugar

Baratz

et al. 2013

Cornea

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Purpose Assess corneal thickness (CT) and correlation with graft outcome after penetrating keratoplasty in the Cornea Donor Study. Methods 887 subjects with a corneal transplant for a moderate risk condition (principally Fuchs or pseudophakic corneal edema) had post-operative CT measurements throughout a 5 year follow up time. Relationships between baseline (recipient, donor, and operative) factors and CT were explored. Proportional hazards models were used to assess association between CT and graft failure. Relationship between CT and cell density was assessed with a longitudinal repeated measures model and Spearman correlation estimates. Results Higher longitudinal CT measurements were associated with diagnosis of pseudophakic or aphakic corneal edema (P<0.001), intraocular pressure > 25mmHg during the first post-operative month (P=0.003), white (non-Hispanic) donor race (P=0.002) and respiratory causes of donor death (P<0.001). Among those without graft failure within the first post-operative year, the 5-year cumulative incidence (±95% CI) of graft failure was 5% ± 5% in those with a 1-year CT ≤500μm, 5% ± 3% for CT 501 – 550μm, 7% ± 4% for CT 551 – 600μm and 20% ± 11% for CT >600μm. In multivariate analysis, both 1 year CT and cell density were associated with subsequent graft failure (P=0.002 and 0.009). CT increase was modestly associated with endothelial cell loss during follow up (r=-0.29). Conclusion During the first 5 years following penetrating keratoplasty, CT can serve as a predictor of graft survival. However, CT is not a substitute for cell density measurement as both measures were independently predictive of graft failure.

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“…Patient information (age, sex, affected eye, guttata score, pachymetry, past medical history) was collected with permission from electronic medical documentation of clinical examinations. Guttata score [67] and pachymetry [68] were taken from the most recent office visit prior to the procedure. Patients selected had no previous consumption of immunomodulatory medications and medications with known effects on epigenetic mechanisms.…”

Section: Methodsmentioning

confidence: 99%

Comprehensive characterization of DNA methylation changes in Fuchs endothelial corneal dystrophy

et al. 2017

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Transparency of the human cornea is necessary for vision. Fuchs Endothelial Corneal Dystrophy (FECD) is a bilateral, heritable degeneration of the corneal endothelium, and a leading indication for corneal transplantation in developed countries. While the early onset, and rarer, form of FECD has been linked to COL8A2 mutations, the more common, late onset form of FECD has genetic mutations linked to only a minority of cases. Epigenetic modifications that occur in FECD are unknown. Here, we report on and compare the DNA methylation landscape of normal human corneal endothelial (CE) tissue and CE from FECD patients using the Illumina Infinium HumanMethylation450 (HM450) DNA methylation array. We show that DNA methylation profiles are distinct between control and FECD samples. Differentially methylated probes (10,961) were identified in the FECD samples compared with the control samples, with the majority of probes being hypermethylated in the FECD samples. Genes containing differentially methylated sites were disproportionately annotated to ontological categories involving cytoskeletal organization, ion transport, hematopoetic cell differentiation, and cellular metabolism. Our results suggest that altered DNA methylation patterns may contribute to loss of corneal transparency in FECD through a global accumulation of sporadic DNA methylation changes in genes critical to basic CE biological processes.

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Relationship of Fuchs Endothelial Corneal Dystrophy Severity to Central Corneal Thickness

Cited by 41 publications

References 29 publications

Corneal Abnormalities Early in the Course of Fuchs' Endothelial Dystrophy

Corneal Abnormalities Early in the Course of Fuchs' Endothelial Dystrophy

Corneal Thickness as a Predictor of Corneal Transplant Outcome

Comprehensive characterization of DNA methylation changes in Fuchs endothelial corneal dystrophy

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