Ocular findings in a Spanish cohort of myotonic dystrophy type 1.

PURPOSE: Myotonic dystrophy type 1 is the most common muscular dystrophy in adulthood, caused by a triplet repeat in chromosome 19q13.3. The present study investigates the frequency of the different ocular alterations in Spanish patients with DM1 and its relationship with the severity of the genetic alteration. METHODS: Cross-sectional and multicenter study was conducted on patients with genetically confirmed DM1. Ophthalmologic examinations included visual acuity assessment, manifest refraction, slit-lamp biomicroscopy, tonometry, ocular motility, corneal tomography, and macular and optic nerve optical coherence tomography. RESULTS: A total of 42 patients (84 eyes) were included. Mean age was 46.9 ± 13.4 (SD) years, and 57.1% were women. Fifteen patients had undergone cataract surgery in at least one eye (35.7%), and 13 (30.9%) had significant cataract. Mean intraocular pressure (IOP) was 10.5 ± 2.9 mmHg, and mean central corneal thickness (CCT) was 580.04 ± 48.61 µm. Half of the patients had significant ptosis, and 8 patients (9.75%) had undergone eyelid surgery. Macular abnormalities included retinal pigment epithelium alterations in 8 eyes of 6 patients, epiretinal membrane in 3 eyes, and lamellar hole in 2 eyes. A moderate correlation was found between IOP and ptosis with the number of triplet repeats. CONCLUSION: Early cataract onset, low IOP, thicker CCT, and ptosis were the most significant manifestations of DM in our sample. Correlation found between IOP and ptosis with CTG repeat could be interesting in order to improve diagnosis and medical care of these patients but should be confirmed in further studies.

A Risk Prediction Model for Endothelial Keratoplasty After Uncomplicated Cataract Surgery in Fuchs Endothelial Corneal Dystrophy.

PURPOSE: To develop a risk prediction model for endothelial keratoplasty (EK) after uncomplicated cataract surgery in Fuchs endothelial corneal dystrophy (FECD) using Scheimpflug imaging. DESIGN: Prospective, observational cohort study. METHODS: The study was conducted at the Ramón y Cajal University Hospital (Madrid, Spain) on 127 eyes from 93 consecutive patients with vision loss, FECD, and cataracts. OBSERVATION: We assessed the corneas using Scheimpflug imaging pachymetry and elevation maps for loss of regular isopachs, displacement of the thinnest point, and posterior surface depression according to the Mayo Clinic subclinical corneal edema classification. We also recorded other preoperative data. Primary Endpoints: The primary endpoint was the need for EK after uncomplicated phacoemulsification within 2 years (median duration, 18 months). We calculated the risk using hazard ratios and the Kaplan-Meier cumulative incidence risk. RESULTS: Forty-four participants required EK, and those eyes with 1, 2, or all 3 tomographic features had a hazard risk of 21.8, 57.2, and 76.5, respectively (P < .005), compared with those eyes with normal tomographic patterns. The best predictive model was based on the number of tomographic features simultaneously present in an eye and the central corneal thickness (CCT) at the pupillary center. We aimed to develop a risk score from 0 to 8. The cumulative risk for EK ranged from virtually 0 for risk scores <4 to almost 100% for those with a score of 8. CONCLUSIONS: The combination of CCT values and tomographic features can be employed to make valid predictions of the risk of requiring EK after phacoemulsification.

Causes of corneal transplant failure: a multicentric study.

PURPOSE: To identify the causes of failure of the different surgical corneal graft techniques: penetrating keratoplasty (PK), deep anterior lamellar keratoplasty (DALK), Descemet stripping automated endothelial keratoplasty (DSAEK) and Descemet membrane endothelial keratoplasty (DMEK). METHODS: This multicentric retrospective study enrolled a consecutive cohort of patients who had undergone any type of keratoplasty between 2001 and 2016. The clinical data were obtained from the patient's medical records, following ethical guidelines, permissions and data protection. The main outcome measured in the study was the cause of graft failure, defined as any irreversible loss of graft transparency capable of compromising vision. The main causes of graft failure were classified as follows: (A) primary graft failure (PGF), (B) immunological rejection, (C) non-rejection (which includes endothelial decompensation without rejection, IOP elevation/glaucoma, diseases of the ocular surface, recurrence of the primary disease, wound dehiscence/hypotonia and trauma, among others) and (D) specific causes of lamellar keratoplasty failure. A descriptive study of the obtained data was carried out. The distribution of the causes of failure was evaluated according to the type of corneal transplant. RESULTS: Our research included a cohort of 571 keratoplasty failures, of which 509 met the inclusion criteria. The analysis of the causes of the PK failure showed that immunological allograft rejection represented the main cause, with 28.2% of the failures, followed by surface diseases (17.8%) and endothelial decompensation without rejection (17.3%). For the PK re-grafts group, the main cause of failure was immunological allograft rejection (34.0%), followed by diseases of the ocular surface (18.5%). For the DALK group, the failures mainly occurred due to surface diseases such as limbal stem cell insufficiency, infectious keratitis, keratolysis or persistent epithelial defect (37.8%). However, the main reason for failure in the DSAEK group was endothelial decompensation without rejection (31.9%) while primary graft failure was the main cause of failure in the DMEK group (64.1%). CONCLUSION: The main reason for failure in PK was immunological allograft rejection, both in primary and secondary transplants. The leading causes for failure were diseases of the ocular surface in the DALK population, endothelial decompensation without rejection in DSAEK and primary graft failure in DMEK.

Characteristics, Behaviors, and Awareness of Contact Lens Wearers Purchasing Lenses Over the Internet.

OBJECTIVES: To describe the characteristics and hygiene habits of contact lens (CL) wearers who acquire CLs over the Internet, as well as their awareness of modifiable risk factors (RFs). METHODS: A web-based survey was conducted among clients of an online CL sales platform during 6 months. Demographic data, CL hygiene-related awareness, and compliance were collected and analyzed. RESULTS: The questionnaire was completed by 1,264 CL wearers: the average age was 40.8 years; most were women (71.1%), had myopia (76.4%), and wore monthly disposable CLs (63.6%). The frequency of eye examination was significantly lower among those buying the CL exclusively online (15.8% vs. 6.6%, P<0.001) and those who had begun CL use on their own (17.5% vs. 8.8%, P<0.001). Initiation to CL wear without the intervention of an eye care practitioner was more frequent in wearers with less experience. Common risk behaviors included water exposure (68.1% in swimming and 64.9% in the shower), use of the case (61.9%) and CL (65.1%) beyond the recommended replacement time, and reuse of the maintenance solution (31.9%). Ignorance of recommended hygiene increased the probability of risky behavior. CONCLUSION: There were no differences in compliance or awareness of risks between those purchasing exclusively online and other CL wearers. However, the growing frequency of self-taught initiation in CL wear among less-experienced online customers and online exclusive purchase was associated with a decrease in eye examination frequency and therefore worse compliance and increased risks. This finding underlines the importance of publicizing correct hygiene habits by all possible means so as to increase compliance.

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.

Keratometric, Pachymetric, and Surface Elevation Characterization of Corneas With Fuchs Endothelial Corneal Dystrophy Treated With DMEK.

PURPOSE: To characterize pachymetric, keratometric, and surface elevation parameters in pseudophakic eyes with Fuchs dystrophy that underwent Descemet membrane endothelial keratoplasty (DMEK) and to compare these characteristics with those in normal pseudophakic eyes. METHODS: Postoperative corneal tomography with a median follow-up of 20 months was evaluated using the Pentacam tomographer on 46 DMEK eyes. Results were compared with data from an age-matched cohort of 46 normal eyes. Parameters included curvature analysis, keratometry values, corneal thickness (CT), pachymetric progression index, Ambrósio relational thickness, posterior elevation, and D-index values. Bonferroni correction was used for multiple comparisons. RESULTS: CT at the apex (mean difference 41 µm; 95% confidence interval 28-54) and CT at the thinnest point (46 µm; 33-60) were statistically significantly lower in the DMEK group (P < 0.001), whereas pachymetric progression index average (0.47; 0.32-0.62), Ambrósio relational thickness maximum (172; 126-228), IHD (0.096; 0.005-0.014), and the global D-index (1.03; 0.51-1.56) were significantly higher in eyes with DMEK (P < 0.001). The mean CT along the vertical and horizontal meridians was significantly lower in the DMEK group within the central 5 mm. A quarter and one half of the DMEK eyes had a CT of less than 500 µm at the apex or at the thinnest point, respectively. CONCLUSIONS: Resolution of corneal edema after DMEK was associated with a thinner-than-normal central cornea, steeper pachymetric progression from the thinnest point to the periphery, and some high-end outliers for posterior elevation values. Caution should be exerted in planning corneal refractive surgery for residual refractive errors.

Repeatability and Intersession Reproducibility of Pentacam Corneal Thickness Maps in Fuchs Dystrophy and Endothelial Keratoplasty.

PURPOSE: To assess intrasession repeatability and intersession reproducibility of Pentacam corneal thickness maps in patients with Fuchs endothelial corneal dystrophy (FECD) before and after endothelial transplantation. METHODS: In this observational diagnostic evaluation, 20 healthy subjects along with 81 consecutive patients were examined. Patients were classified into 4 groups: FECD without corneal edema, FECD with corneal edema, Descemet stripping automated endothelial keratoplasty, and Descemet membrane endothelial keratoplasty. Three consecutive scans of each eye were obtained at 2 different sessions. Raw pachymetry data were used to calculate average values of 4 concentric annular zones, which were also divided into 8 sectors. Repeatability and reproducibility coefficients (CR), coefficient of variation (CV), intraclass correlation coefficient, and 95% limits of agreement were calculated. RESULTS: The intrasession repeatability CV was ≤1% in the central 6 mm for all groups, with an intraclass correlation coefficient ≥0.97. It was better at the central zone than the periphery in all groups. Intersession reproducibility tended to be worse in the central area than the periphery in FECD without edema (CR ≤ 24.37; CV ≤ 1.48) and FECD with edema (CR ≤ 36.74; CV ≤ 2.03), whereas it was better in the central area in healthy eyes (CR ≤ 20.11; CV ≤ 1.32) and improved after Descemet stripping automated endothelial keratoplasty (CR ≤ 21.93; CV ≤ 1.31) and Descemet membrane endothelial keratoplasty (CR ≤ 30.83; CV ≤ 1.94). CONCLUSIONS: Pentacam corneal thickness maps showed good repeatability and intersession reproducibility in virgin and grafted corneas with FECD, which makes it a valid tool for monitoring these patients. Central areas showed the highest variability between sessions in diseased groups.

Squamous Cell Neoplasm of the Ocular Surface in a Patient With Psoriasis Treated With Tumor Necrosis Factor-Alpha Inhibitors.

PURPOSE: To report a case of conjunctival squamous cell carcinoma in a patient with psoriasis treated with tumor necrosis factor-alpha inhibitors. METHODS: Case report and review of the literature. RESULTS: A 44-year-old white woman presented to our hospital 1 year after developing a rapidly growing nodular lesion with papillomatous characteristics on the bulbar conjunctiva. The patient had a medical history of psoriasis since childhood and psoriatic arthritis since 2009. She had received several immunosuppressive medications such as leflunomide, adalimumab, methotrexate, and etanercept. The lesion appeared a few months after commencing treatment with etanercept and methotrexate. A histological examination revealed a squamous intraepithelial neoplasm of the conjunctiva that was negative for human papillomavirus infection. CONCLUSIONS: Several case reports have described squamous cell carcinoma in different parts of the body in patients taking tumor necrosis factor-alpha inhibitors. Squamous cell neoplasm of the ocular surface should be considered during differential diagnosis of new conjunctival lesions in such patients.

Comparison of toric intraocular lenses and peripheral corneal relaxing incisions to treat astigmatism during cataract surgery.

PURPOSE: To evaluate and compare toric intraocular lens (IOL) implantation and spherical IOL implantation with peripheral corneal relaxing incisions to manage astigmatism during phacoemulsification. SETTING: Ophthalmology Service, Hospital Ramón y Cajal, Madrid, Spain. DESIGN: Prospective randomized comparative case series. METHODS: Eyes with cataract and corneal astigmatism (1.00 to 3.00 diopters [D]) had toric IOL implantation or peripheral corneal relaxing incisions. Outcome measures were visual outcomes, slitlamp assessment, digital toric IOL axis determination, spectacle need, and patient satisfaction. RESULTS: Three months postoperatively, the mean uncorrected distance visual acuity (UDVA) was 0.13 ± 0.10 (SD) in the toric IOL group and 0.19 ± 0.12 in the relaxing incisions group; the UDVA was better than 0.20 in 75% of eyes and 60% of eyes, respectively. Refractive cylinder decreased significantly in both groups, with a mean residual refractive astigmatism of 0.61 ± 0.41 D in the toric IOL group and 1.32 ± 0.60 D in the relaxing incisions group (P<.01). The mean toric IOL rotation was 3.65 ± 2.96 degrees, with no significant differences between slitlamp and digital photograph measurements. There was a trend toward better mesopic contrast sensitivity with glare in the toric IOL group. There were no differences in VF-14 or patient satisfaction results; 15% of patients in the toric IOL group and 45% in the relaxing-incision group required distance spectacles postoperatively. CONCLUSION: Although refractive astigmatism decreased in both groups, toric IOL implantation was more effective and predictable, resulting in greater spectacle independence.