Systematic Review and Meta-analysis: Outcomes After Descemet Membrane Endothelial Keratoplasty Versus Ultrathin Descemet Stripping Automated Endothelial Keratoplasty.

PURPOSE: To compare the efficacy and safety of ultrathin Descemet stripping (automated) endothelial keratoplasty (UT-DS(A)EK) versus Descemet membrane endothelial keratoplasty (DMEK) for the treatment of Fuchs endothelial dystrophy (FED) and bullous keratopathy (BK). DESIGN: Systematic review and meta-analysis. METHODS: Literature containing DMEK and UT-DSAEK were searched in the Cochrane Database of Systematic Reviews, PubMed, EMBASE, LILACS, and through manual reference searching. Studies were included that measured the outcome of interventions-including best corrected visual acuity (BCVA), endothelial cell density (ECD), and postoperative complications, especially graft detachment with the need of re-bubbling, graft rejection, graft failure, and postoperative elevated intraocular pressure (IOP)-in patients with FED and BK. Included outcomes were pooled as standardized mean differences (SMD) or risk ratios (RR) using random effects models. Inter-study heterogeneity was assessed using the Q-test and I2 statistic. RESULTS: Seven (of 163) studies met all the inclusion and exclusion criteria. Meta-analysis showed a significantly better BCVA 12 months postoperatively, but an increased re-bubbling rate in eyes after DMEK compared with eyes after UT-DS(A)EK (BCVA: SMD = 0.50 [95% CI 0.27-0.74] and re-bubbling rate: RR = 0.33 [95% CI 0.16-0.67]). All other parameters did not differ significantly between both interventions, although estimates were imprecise (graft failure: RR = 0.65 [95% CI 0.18-2.30], graft rejection: RR = 1.40 [95% CI 0.27-7.30], and postoperative intraocular pressure elevation: RR = 1.14 [95% CI 0.60-2.18]). Postoperative SMDs of ECD could not be evaluated due to significant heterogeneity between studies. CONCLUSIONS: Although the improvement in BCVA was higher after UT-DS(A)EK than after conventional DS(A)EK, the BCVA after DMEK was still superior. The complication rates were comparable for both procedures, except for the higher rate of re-bubbling after DMEK.

Long-term Outcomes of Descemet Stripping Endothelial Keratoplasty: Ten-Year Graft Survival and Endothelial Cell Loss.

PURPOSE: To determine 10-year outcomes for graft and endothelial cell survival after Descemet stripping endothelial keratoplasty (DSEK), including risk factors for graft failure. DESIGN: Retrospective clinical cohort study. METHODS: Three hundred fifty-six consecutive DSEK grafts performed by 10 surgeons using a standardized protocol were analyzed. Primary outcomes were cumulative graft survival and percentage endothelial cell loss (ECL) from 6 months to 10 years; secondary outcomes included risk factors for graft failure, postoperative complications, visual outcomes, and central corneal thickness. RESULTS: Indications include Fuchs endothelial dystrophy (n = 209), bullous keratopathy (n = 88), and previous graft failure (n = 39). One hundred and four eyes (29%) had preoperative glaucoma. Cumulative graft survival rates of all eyes at 1, 3, 5, and 10 years were 97%, 90%, 85%, and 79%, respectively. Ten-year graft survival for Fuchs endothelial dystrophy was 92%. Mean ± SD percentage ECL of all grafts was 46.6% ± 17.3% at year 1, 54.9% ± 18.7% at year 3, 59.6% ± 17.4% at year 5, and 73.1% ± 9.7% at year 10. Cox regression identified preoperative glaucoma (hazard ratio [HR]: 8.41; 95% CI, 1.30-54.5; P = .026), including previous glaucoma surgery (HR: 3.63; 95% CI: 1.03-12.74; P = .04) and regrafts (HR: 5.29; 95% CI: 2.02-13.89; P = .001) as significant risk factors for graft failure. CONCLUSIONS: At 10 years, DSEK survival rate was 79% for all eyes, including complex grafts, and ECL was 73%. For Fuchs endothelial dystrophy, 10-year graft survival rate was 92%. Despite a mean 10-year endothelial cell count of only 692 cells/mm2, graft survival remained high with good vision. DSEK continues to be a viable treatment option, especially in complex eyes with comorbidity.

Dual corneal involvement by endothelial and epithelial corneal dystrophies in Steinert's disease: A case of triple dystrophy.

INTRODUCTION: A case of dual corneal involvement due to Fuchs endothelial corneal dystrophy and epithelial basement membrane corneal dystrophy in a patient with Steinert's myotonic dystrophy type 1 is described, and a literature review on the triple association is made. CASE DESCRIPTION: A 52-year-old male diagnosed with myotonic dystrophy type 1 presented due to progressive bilateral vision loss during the past year. A full ophthalmological evaluation was made, with biomicroscopy, funduscopy, anterior segment optical coherence tomography, and endothelial cell count using specular microscopy. Exploration revealed bilateral superior palpebral ptosis, visual acuity 0.5 in the right eye and 0.3 in the left eye, and with an intraocular pressure of 11 and 10 mmHg, respectively. Biomicroscopy revealed map-dot-fingerprint lesions characteristic of epithelial basement membrane corneal dystrophy in both eyes, as well as abundant endothelial guttae due to Fuchs endothelial corneal dystrophy (stage II) and bilateral nuclear and posterior subcapsular cataracts. Specular microscopy in turn showed cell loss and a destructured endothelial map. Finally, anterior segment optical coherence tomography revealed the accumulation of epithelial basement membrane and hyperreflective endothelial excrescences corresponding to guttae. CONCLUSION: The association of Fuchs endothelial corneal dystrophy with myotonic dystrophy has been described and explained by a common genetic basis in the expansion of a CTG trinucleotide repeat, though this is the first reported case of the triple association of Fuchs endothelial corneal dystrophy, epithelial basement membrane corneal dystrophy, and myotonic dystrophy type 1. New mutations or still unknown genetic alterations could possibly explain the triple association reported in our case.

Fuchs endothelial corneal dystrophy: The vicious cycle of Fuchs pathogenesis.

Fuchs endothelial corneal dystrophy (FECD) is the most common primary corneal endothelial dystrophy and the leading indication for corneal transplantation worldwide. FECD is characterized by the progressive decline of corneal endothelial cells (CECs) and the formation of extracellular matrix (ECM) excrescences in Descemet's membrane (DM), called guttae, that lead to corneal edema and loss of vision. FECD typically manifests in the fifth decades of life and has a greater incidence in women. FECD is a complex and heterogeneous genetic disease where interaction between genetic and environmental factors results in cellular apoptosis and aberrant ECM deposition. In this review, we will discuss a complex interplay of genetic, epigenetic, and exogenous factors in inciting oxidative stress, auto(mito)phagy, unfolded protein response, and mitochondrial dysfunction during CEC degeneration. Specifically, we explore the factors that influence cellular fate to undergo apoptosis, senescence, and endothelial-to-mesenchymal transition. These findings will highlight the importance of abnormal CEC-DM interactions in triggering the vicious cycle of FECD pathogenesis. We will also review clinical characteristics, diagnostic tools, and current medical and surgical management options for FECD patients. These new paradigms in FECD pathogenesis present an opportunity to develop novel therapeutics for the treatment of FECD.

Implicación ocular en pacientes con distrofia miotónica / Eye involvement in patients with myotonic dystrophy

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Ultraviolet A light induces DNA damage and estrogen-DNA adducts in Fuchs endothelial corneal dystrophy causing females to be more affected.

Fuchs endothelial corneal dystrophy (FECD) is a leading cause of corneal endothelial (CE) degeneration resulting in impaired visual acuity. It is a genetically complex and age-related disorder, with higher incidence in females. In this study, we established a nongenetic FECD animal model based on the physiologic outcome of CE susceptibility to oxidative stress by demonstrating that corneal exposure to ultraviolet A (UVA) recapitulates the morphological and molecular changes of FECD. Targeted irradiation of mouse corneas with UVA induced reactive oxygen species (ROS) production in the aqueous humor, and caused greater CE cell loss, including loss of ZO-1 junctional contacts and corneal edema, in female than male mice, characteristic of late-onset FECD. UVA irradiation caused greater mitochondrial DNA (mtDNA) and nuclear DNA (nDNA) damage in female mice, indicative of the sex-driven differential response of the CE to UVA, thus accounting for more severe phenotype in females. The sex-dependent effect of UVA was driven by the activation of estrogen-metabolizing enzyme CYP1B1 and formation of reactive estrogen metabolites and estrogen-DNA adducts in female but not male mice. Supplementation of N-acetylcysteine (NAC), a scavenger of reactive oxygen species (ROS), diminished the morphological and molecular changes induced by UVA in vivo. This study investigates the molecular mechanisms of environmental factors in FECD pathogenesis and demonstrates a strong link between UVA-induced estrogen metabolism and increased susceptibility of females for FECD development.

Preoperative Risk Assessment for Progression to Descemet Membrane Endothelial Keratoplasty Following Cataract Surgery in Fuchs Endothelial Corneal Dystrophy.

PURPOSE: To identify preoperative corneal tomographic features that predict progression to endothelial keratoplasty (EK) following cataract surgery in Fuchs endothelial corneal dystrophy (FECD) and establish a regression model to identify high-risk patients. DESIGN: Prospective, observational cohort study. METHODS: Setting: Hospital Universitario Ramón y Cajal, Madrid, Spain. STUDY POPULATION: Sixty-eight patients (84 eyes) with FECD who underwent phacoemulsification. INTERVENTION: We assessed preoperative best-corrected visual acuity; ultrasound central corneal thickness; pachymetric, anterior chamber depth, and corneal backscatter variables using Scheimpflug imaging; and endothelial cell density. MAIN OUTCOME MEASURES: Progression to EK. RESULTS: A total of 33 eyes (39.3%) needed EK after phacoemulsification to rehabilitate vision. On multivariate analysis, anterior layer (AL) corneal backscatter between 0 and 2 mm from the apex and relative increase in central corneal thickness from the "relative pachymetry display" by the Pentacam were significant predictors of the risk of progression to EK. Using these 2 variables, a risk score (RISC score) was derived from the regression model (area under the curve = 0.973; best cutoff point with a specificity of 95% representing a sensitivity of 96%). Excluding corneal backscatter data from the multivariate regression model, corneal thickness at the pupil center by the Pentacam and relative increase in central corneal thickness were significant predictors and provided a modified risk score (RIPT score) with similar performance. CONCLUSION: Both scores demonstrated accuracy in predicting progression to EK using easily accessible preoperative data. This approach, which can be readily implemented by surgeons, allows for individualized risk assessment.

Association of the Gutta-Induced Microenvironment With Corneal Endothelial Cell Behavior and Demise in Fuchs Endothelial Corneal Dystrophy.

Importance: The number and size of guttae increase over time in Fuchs endothelial corneal dystrophy (FECD); however, the association between these physical parameters and disease pathogenesis is unclear. Objective: To determine the role of guttae in corneal endothelial cell function. Design, Settings, and Participants: In an in vitro model, cells from a human corneal endothelial cell line, HCENC-21T, were seeded on decellularized normal (n = 30) and FECD (n = 70) endothelial basement (Descemet) membranes (DMs). Normal human corneas were sent to our laboratory from 3 sources. The study took place at the Schepens Eye Research Institute, Massachusetts Eye and Ear, Boston, and was performed from September 2015 to July 2017. Normal DMs were obtained from 3 different tissue banks and FECD-DMs were obtained from patients undergoing endothelial keratoplasty in 2 departments. Main Outcomes and Measures: Endothelial cell shape, growth, and migration were assessed by live-cell imaging, and gene expression analysis as a function of guttae diameter was assessed by laser capture microscopy. Results: Mean (SD) age of normal-DMs donors was 65.6 (4.4) years (16 women [53%]), and mean (SD) age of FECD-DMs donors was 68.9 (10.6) years (43 women [61%]). Cells covered a greater area (mean [SD], 97.7% [8.5%]) with a greater mean (SD) number of cells (2083 [153] cells/mm2) on the normal DMs compared with the FECD DMs (72.8% [11%]; P = .02 and 1541 [221] cells/mm2 221/mm2; P = .01, respectively). Differences in endothelial cell growth over guttae were observed on FECD DMs depending on the guttae diameter. Guttae with a mean (SD) diameter of 10.5 (2.9) µm did not impede cell growth, whereas those with a diameter of 21.1 (4.9) µm were covered only by the cell cytoplasm. Guttae with the largest mean (SD) diameter, 31.8 (3.8) µm, were not covered by cells, which instead surrounded them in a rosette pattern. Moreover, cells adjacent to large guttae upregulated αSMA, N-cadherin, Snail1, and NOX4 genes compared with ones grown on normal DMs or small guttae. Furthermore, large guttae induced TUNEL-positive apoptosis in a rosette pattern, similar to ex vivo FECD specimens. Conclusions and Relevance: These findings highlight the important role of guttae in endothelial cell growth, migration, and survival. These data suggest that cell therapy procedures in FECD might be guided by the diameter of the host guttae if subsequent clinical studies confirm these laboratory findings.

Descemet Membrane Endothelial Keratoplasty for Epithelial Downgrowth After Clear Corneal Cataract Surgery.

OBJECTIVES: To describe the first case of Descemet membrane endothelial keratoplasty (DMEK) combined with argon laser photocoagulation and intracameral 5-fluorouracil (5-FU) injection in a patient with epithelial downgrowth after cataract surgery. METHODS: Case report and review of the literature. RESULTS: A 77-year-old female underwent uneventful cataract surgery using a clear corneal incision in her left eye. Six months after surgery, an epithelial cell sheet began to cover the posterior cornea and then spread to the anterior chamber structures, leading to anterior synechia and corectopia at 2 years. Argon laser photocoagulation was used to remove epithelial downgrowth on the iris surface. However, retrocorneal epithelial downgrowth gradually progressed, resulting in bullous keratopathy. DMEK with intracameral 5-FU injection was performed to remove epithelial tissue and replace the diseased endothelium with healthy endothelium. One year after surgery, her vision improved to 20/50 and the endothelial cell count was 1,643 cells/mm in her left eye. There were no further complications or recurrence of epithelial downgrowth. CONCLUSIONS: DMEK with preoperative argon laser and intraoperative 5-FU is a useful option for the treatment of diffuse epithelial downgrowths.

[Fuchs endothelial corneal dystrophy]. / Éndotelial'naia distrofiia rogovitsy Fuksa.

Fuchs endothelial dystrophy is a severe disease characterized by slowly progressing bilateral asymmetric corneal edema usually seen in elderly patients. The primary purpose of treatment is to minimize edema-related symptoms, such as ocular discomfort and visual acuity loss. Conservative therapy is symptomatic and has a short-term positive effect that does not lead to full functional rehabilitation of the patient, while endothelial keratoplasty is pathogenetically oriented. Intentional replacement of pathologically altered corneal layers has several advantages: preservation of corneal architectonics, rapid recovery of visual function, 'closed eye' surgery with corneal tunnel approach, and independence from expensive equipment. However, principle indications and contraindications for various modifications of endothelial keratoplasty in the presence of comorbidity are still to be defined. Further improvement of the methods and their broader implementation into clinical practice are the most pressing and promising issues of corneal transplantation.

Interface Fluid Syndrome After Laser In Situ Keratomileusis (LASIK) Because of Fuchs Endothelial Dystrophy Reversed by Descemet Membrane Endothelial Keratoplasty (DMEK).

PURPOSE: To describe a case that developed "interface fluid syndrome" after previous laser in situ keratomileusis (LASIK) because of Fuchs endothelial dystrophy (FED), which was reversed by Descemet membrane endothelial keratoplasty (DMEK). METHODS: A 58-year-old male patient presented with bilateral visual impairment owing to FED and visually significant cataract. Cataract surgery was carried out in both eyes followed by DMEK in his left eye. RESULTS: After cataract surgery, visual acuity did not improve sufficiently because corneal thickness increased and a fine cleft with interface fluid developed between the LASIK-flap and the residual stromal bed. After uneventful DMEK in his left eye, the fluid resolved within a week and visual acuity improved rapidly. CONCLUSIONS: This case demonstrates that "interface fluid syndrome" after LASIK caused by concomitant endothelial dysfunction may be reversed by DMEK allowing fast visual recovery.

Descemet stripping automated endothelial keratoplasty in Fuchs' corneal endothelial dystrophy: anterior segment optical coherence tomography and in vivo confocal microscopy analysis.

BACKGROUND: To evaluate the in vivo corneal changes using in vivo confocal microscopy (IVCM) and anterior segment optical coherence tomography (AS-OCT) in patients with Fuchs' dystrophy who underwent Descemet stripping automated endothelial keratoplasty (DSAEK) and the relationship between these changes and the postoperative visual recovery up to 1-year follow-up. METHODS: Before DSAEK and 1 day, 3, 6 and 12 months after surgery 31 patients (39 pseudophakic eyes) underwent a complete ophthalmological evaluation including best corrected visual acuity (BCVA), IVCM (subepithelial haze, interface haze, graft thickness) and AS-OCT (graft thickness). RESULTS: Graft thickness measurements by AS-OCT were strongly correlated to those obtained using IVCM at every follow-up stage (intraclass correlation coefficient = 0.95 to 0.97 between 3 and 12 months, P < 0.001 for all coefficients). No correlation between BCVA and graft thickness measured by AS-OCT at any follow-up stage was found, while at 3 and 6 postoperative months the correlations between BCVA and preoperative subepithelial haze (r = 0.61, P < 0.001 and r = 0.46, P = 0.002), interface haze (r = 0.51, P < 0.001 and r = 0.46, P = 0.003), postoperative subepithelial haze (r = 0.43, P = 0.004 and r = 0.39, P = 0.001) were significant. CONCLUSIONS: The study confirmed corneal subepithelial haze and interface haze as important factors limiting visual acuity after DSAEK, while graft thickness was not related to BCVA.

Fuchs endothelial corneal dystrophy in patients with myotonic dystrophy: a case series.

PURPOSE: The aim was to report 4 cases of Fuchs endothelial corneal dystrophy (FECD) in patients with an established diagnosis of myotonic dystrophy (DM) and suggest a mechanism for their association based on the known molecular genetics and potential pathophysiological parallels of DM and FECD. METHODS: We reviewed all available medical records and pathology slides for the 4 reported cases from the Department of Ophthalmology at Oregon Health and Science University's Casey Eye Institute and Devers Eye Institute at the Legacy Good Samaritan Medical Center in Portland, OR. RESULTS: Four patients were found to have DM and bilateral corneal guttae, consistent with the diagnosis of FECD. All the identified patients were female and were aged between 34 and 63, and 2 patients were related (mother and daughter). The corneal specimens from 2 of the 4 patients who had undergone a corneal transplant were pathologically confirmed to be consistent with the diagnosis of FECD. CONCLUSIONS: To our knowledge, FECD has not been previously reported in association with DM. Because both diseases are somewhat prevalent in the United States, it is possible that their coexistence is merely a coincidence in these patients. However, recent studies into the pathogenesis of each disease have shown more parallels between FECD and DM, suggesting the possibility of a noncoincidental association. Potential mutual pathogenic mechanisms may involve altered protein expression causing the deregulation of ion homeostasis, an unstable intronic trinucleotide repeat expansion, or activation of the unfolded protein response and oxidative stress pathways.

[Complications in endothelial keratoplasty performed by cornea fellows]. / Complicações em transplantes endoteliais realizados por estagiários de córnea.

PURPOSE: To report early complications in endothelial keratoplasty performed by cornea fellows. METHODS: Retrospective study of endothelial keratoplasty performed by second-year cornea fellows. Described the early complications and its treatments. RESULTS: Thirty four endothelial keratoplasty were performed by cornea fellows, from July 2007 to August 2008. From this, 29 were Descemet stripping endothelial keratoplasty and 5 Descemet stripping automated endothelial keratoplasty; 14 combined with phacoemulsification and 1 with extracapsular cataract surgery. The main indication was Fuchs' dystrophy (18 cases), followed by bullous keratopathy (11 cases), primary failure after endothelial keratoplasty (4 cases) and congenital hereditary endothelial dystrophy (1 case). Main surgery complication was donor button detachment in 8 eyes (23.5%), followed by primary graft failure in 7 eyes (20.6%) and acute glaucoma after pupillary block in 1 eye (2.9%). CONCLUSIONS: The complication rate in this series, which were the first ever in a group of six cornea fellows, was high. This shows the steep learning curve for this surgical procedure. The assistance of an experienced surgeon could help to achieve a less steep learning curve with a lower complication rate.

Complicações em transplantes endoteliais realizados por estagiários de córnea / Complications in endothelial keratoplasty performed by cornea fellows

OBJETIVO: Reportar complicações precoces numa série de transplantes endoteliais realizados por estagiários de córnea. MÉTODOS: Revisão retrospectiva dos transplantes endoteliais realizados por estagiários de córnea do segundo ano. Descritas as complicações precoces e respectivo tratamento. RESULTADOS: Foram realizados 34 transplantes endoteliais pelos estagiários de córnea entre julho de 2007 e agosto de 2008. Destes, 29 foram realizados pela técnica de "Descemet stripping endothelial keratoplasty" e 5 "Descemet stripping automated endothelial keratoplasty". Foram realizadas 15 cirurgias combinadas, 14 associadas a facoemulsificação com implante de lente intraocular e 1 associada a facectomia com implante de lente intraocular. A principal indicação foi por distrofia de Fuchs com 18 casos, 11 foram indicados por ceratopatia bolhosa após cirurgia de catarata, 4 por falência primária após transplante endotelial e 1 por distrofia endotelial congênita hereditária. A complicação mais encontrada foi descolamentos do botão doado em 8 olhos (23,5%). Falência primária foi encontrado em 7 olhos (20,6%). Glaucoma agudo por bloqueio pupilar foi encontrado em 1 paciente (2,9%). CONCLUSÕES: O alto número de complicações encontradas nestas cirurgias, que foram as primeiras de um grupo de 6 estagiários de córnea, mostra a longa curva de aprendizado para este procedimento cirúrgico. A orientação das primeiras cirurgias por cirurgiões mais experientes pode diminuir a curva de aprendizado e a taxa de complicações.

PURPOSE: To report early complications in endothelial keratoplasty performed by cornea fellows. METHODS: Retrospective study of endothelial keratoplasty performed by second-year cornea fellows. Described the early complications and its treatments. RESULTS: Thirty four endothelial keratoplasty were performed by cornea fellows, from July 2007 to August 2008. From this, 29 were Descemet stripping endothelial keratoplasty and 5 Descemet stripping automated endothelial keratoplasty; 14 combined with phacoemulsification and 1 with extracapsular cataract surgery. The main indication was Fuchs' dystrophy (18 cases), followed by bullous keratopathy (11 cases), primary failure after endothelial keratoplasty (4 cases) and congenital hereditary endothelial dystrophy (1 case). Main surgery complication was donor button detachment in 8 eyes (23.5%), followed by primary graft failure in 7 eyes (20.6%) and acute glaucoma after pupillary block in 1 eye (2.9%). CONCLUSIONS: The complication rate in this series, which were the first ever in a group of six cornea fellows, was high. This shows the steep learning curve for this surgical procedure. The assistance of an experienced surgeon could help to achieve a less steep learning curve with a lower complication rate.

Association of smoking and other risk factors with Fuchs' endothelial corneal dystrophy severity and corneal thickness.

PURPOSE: We investigated effects of smoking and other risk factors on the development of advanced Fuchs' endothelial corneal dystrophy (FECD) and on central corneal thickness (CCT). METHODS: Eyes from Caucasian probands, affected and unaffected family members, and unrelated controls matched for age from the FECD Genetics Multi-Center Study (n = 2044 subjects) were examined. Univariate and multivariate models, adjusted for family correlations, were used to determine the effect of smoking, sex, diabetes, and age on FECD case/control status and CCT. RESULTS: In a multivariate model, sex and smoking were associated significantly with advanced FECD (grades 4-6) development (P = 0.016 and P = 0.047, respectively). Female sex increased odds by 34%. Smoking increased odds by 30%. In a multivariate model, diabetes was associated with an increase of 9.1 µm in average CCT (P = 0.021). Female sex was associated significantly with a decrease in average CCT by 6.9 µm (P = 0.015). Smoking had no significant effect on CCT in any model. As shown previously, advanced FECD was associated with large increases in CCT (31.4-94.2 µm). CONCLUSIONS: Smoking was associated with an increased risk of advanced FECD and self-reported diabetes was associated with increased CCT. Further study of the impact of smoking and diabetes on FECD development and changes in corneal thickness is warranted.

Long-term corneal clarity after spontaneous repair of an iatrogenic descemetorhexis in a patient with Fuchs dystrophy.

PURPOSE: To report a case of long-term corneal clarity after spontaneous resolution of corneal edema associated with iatrogenic endothelial trauma in a patient with Fuchs dystrophy. METHODS: Retrospective case report. RESULTS: An 84-year-old woman with Fuchs dystrophy experienced an iatrogenic descemetorhexis during complicated phacoemulsification. Despite marked early postoperative corneal edema, she experienced spontaneous clearing of her cornea within 4 months with eventual reendothelialization of the central defect. She maintained excellent visual acuity for 16 years with no evidence of recurrent corneal edema. CONCLUSIONS: Self-repair of a descemetorhexis and long-term corneal clarity may occur in eyes with underlying endothelial dystrophy.