Scleral lens induced short term corneal changes in eyes with Pellucid Marginal Degeneration.

PURPOSE: To investigate the short-term effect of scleral lens (SL) on corneal curvature and corneal oedema in Pellucid Marginal Degeneration (PMD) eyes. METHODS: Corneal anterior, posterior curvature and corneal thickness were measured in 14 eyes of 14 PMD participants with Schiempflug imaging at different corneal diameters and meridians at baseline and after 6 h of SL wear. RESULTS: There was a significant flattening (up to 0.26 mm) of the anterior corneal curvature noted in the inferotemporal quadrant (from 210 to 255 degree at 2 mm, 8 mm and 10 mm corneal diameter), inferonasal quadrant (from 285 to 345 degree at 6 mm and 8 mm corneal diameter), and inferiorly at 2 mm and 10 mm corneal diameter (p < 0.05). Similarly, posterior corneal curvature showed statistically significant steepening mostly in inferotemporal quadrants (from 195 to 255 degree from 4 mm to 8 mm corneal diameter) and inferonasally at 2 mm and 4 mm corneal diameter (p < 0.05). A statistically significant increase in the corneal thickness noted in different corneal diameters with corneal oedema ranging from 2.10 % to 4.00 % after 6 h of SL wear. A gradual increase in corneal oedema was noted form centre to periphery. The baseline central fluid reservoir thickness (FRT) was 341.07 ± 139.8 which reduced to 276.71 ± 114.32 µm after 6 h of lens wear. No significant correlation was noted between corneal oedema with different parameters like initial and final FRT, change in anterior and posterior corneal curvature, and lens thickness (p > 0.05). CONCLUSIONS: Short-term SL wear induced a clinically acceptable range of corneal oedema. A clinically significant flattening in anterior curvature and minimal steepening in posterior curvature were noted. Practitioners should be careful while measuring corneal parameters in PMD eyes wearing SL, as these alterations can provide false impression of disease progression.

Long-Term Follow-Up of Pediatric Excimer Laser-Assisted Penetrating Keratoplasty for Congenital Stromal Corneal Dystrophy.

PURPOSE: The purpose of this study was to highlight characteristic clinical and microscopic findings and report the long-term follow-up of pediatric excimer laser-assisted penetrating keratoplasty (excimer-PKP) for congenital stromal corneal dystrophy (CSCD). METHODS: A 2-year-old Greek child presented with CSCD at our department. Clinical examination showed bilateral flake-like whitish corneal opacities affecting the entire corneal stroma up to the limbus. Genetic testing identified a mutation of the decorin gene (c.962delA). The variant was not present in the parents and represented a de novo mutation. The uncorrected visual acuity was 20/100 in both eyes. Excimer-PKP (8.0/8.1 mm) was performed on the right eye at the age of 2.5 years and on the left eye at the age of 3 years. Postoperatively, alternating occlusion treatment was performed. RESULTS: The light microscopic examination demonstrated a disorganized extracellular matrix of the corneal stroma characterized by a prominent irregular arrangement of stromal collagen lamellae with large interlamellar clefts containing ground substance, highlighted by periodic acid-Schiff- and Alcian blue-positive reaction detecting acid mucopolysaccharides. Electron microscopy showed disorganization and caliber variation of collagen lamellae and thin filaments within an electron-lucent ground substance. The postoperative course was unremarkable. Both grafts remained completely clear 14 years postoperatively. Corneal tomography showed moderate regular astigmatism with normal corneal thickness. The corrected distance visual acuity was 20/25 in both eyes. CONCLUSIONS: Excimer-PKP for CSCD might be associated with excellent long-term results and a good prognosis, particularly when the primary surgery is performed at a very young age. However, this requires close postoperative follow-up examinations by an experienced pediatric ophthalmologist to avoid severe amblyopia.

Clinical outcomes of epithelial basement membrane dystrophy after keratorefractive lenticule extraction.

PURPOSE: To determine the clinical outcomes of keratorefractive lenticule extraction (KLEx) in eyes with epithelial basement membrane dystrophy (EBMD) that developed after surgery. SETTING: Onnuri Smile Eye Clinic, Seoul, South Korea. DESIGN: Retrospective single-center study. METHODS: This study reviewed the medical records of 26 eyes of 16 patients who exhibited signs of EBMD after KLEx. Postoperatively, corneal findings and visual outcomes were evaluated. Statistical analyses were also performed on eyes without corneal complications after KLEx. RESULTS: Signs of EBMD were first observed within 1 week postoperatively in 22 eyes (84.6%): 6 eyes (23.1%) showed epithelial defects, 10 eyes (38.5%) showed subepithelial corneal opacity, 5 eyes (19.2%) showed diffuse lamellar keratitis, and 1 eye (3.9%) showed epithelial ingrowth. Symptoms such as pain, while corneal lesions were present, occurred in 21 eyes (80.8%). At 3 months postoperatively, 21 eyes (80.8%) showed no specific findings on slitlamp microscopy after medical treatment, 2 eyes (7.7%) showed subepithelial corneal opacity, 2 eyes (7.7%) showed corneal scar, and 1 eye (3.9%) showed epithelial ingrowth. Uncorrected distance visual acuity was 20/25 or better in 24 eyes (92.3%), and spherical equivalent was within ±1.0 D in 20 eyes (76.9%). The efficacy index of the EBMD group did not significantly differ from the control group, while the safety index was significantly lower. CONCLUSIONS: Manifestations of EBMD can occur after KLEx and can affect clinical outcomes. Most cases showed favorable results with appropriate nonsurgical treatment; however, some cases had long-term complications such as corneal scarring.

Analysis of Radial Peripapillary Capillary Density in Patients with Bietti Crystalline Dystrophy by Optical Coherence Tomography Angiography.

OBJECTIVE: We wanted to investigate the radial peripapillary capillary (RPC) network in patients with Bietti crystalline dystrophy (BCD). METHODS: We compared RPC densities in the disk and different peripapillary regions, obtained using optical coherence tomography angiography in 22 patients with BCD (37 eyes) and 22 healthy subjects (37 eyes). The BCD group was then divided into Stage 2 and Stage 3 subgroups based on Yuzawa staging, comparing the RPC densities of the two. RESULTS: The disk area RPC density was 38.8% ± 6.3% in the BCD group and 49.2% ± 6.1% in the control group ( P < 0.001), and peripapillary region RPC density was significantly lower in the BCD group than in the control group (49.1% ± 4.7% and 54.1% ± 3.0%, respectively, P < 0.001). There were no significant RPC density differences between the tempo quadrant and inside disk of Stages 2 and 3 subgroups; the other areas showed a significantly lower RPC density in Stage 3 than in Stage 2 BCD. CONCLUSION: The BCD group RPC density was significantly lower than the control group. The reduction of RPC density in the tempo quadrant occurred mainly in the Stage 1 BCD. In contrast, the reduction of RPC density in superior, inferior, and nasal quadrants occurred mainly in Stage 2.

CHORIORETINAL ATROPHY IN VITREORETINAL LYMPHOMA: Risk Factors and Visual Outcomes.

PURPOSE: To investigate the frequency, risk factors, and functional prognosis of chorioretinal atrophy (CRA) in vitreoretinal lymphoma (VRL). METHODS: This was a retrospective cohort study of consecutive patients with vitreoretinal lymphoma. The demographic, clinical, and retinal features and the treatment modalities of each patient were collected. The charts and the multimodal imaging at each visit were reviewed. The risk factors associated with CRA were investigated with a mixed-model Cox regression. RESULTS: Of the 79 eyes of 40 patients included, 41 eyes (52%) had CRA; 27 and 14 eyes had focal and diffuse CRA, respectively. The rate of vitreoretinal lymphoma lesions in the macula was similar between focal and diffuse CRA (96% vs. 93%). The eyes with CRA had worse best-corrected visual acuity (P = 0.006) than eyes with no CRA; diffuse atrophy had the worst best-corrected visual acuity (P < 0.001). The presence of retinal infiltrates (hazard ratio = 3.75, 95% confidence interval [CI] 1.46-9.59, P = 0.006) and vertical hyperreflective lesions (hazard ratio= 4.13 95% CI 1.14-14.93, P = 0.03) on optical coherence tomography and macular involvement (hazard ratio = 6.59, 95% CI 1.41-30.53, P = 0.02) were associated with a higher risk of CRA. CONCLUSION: Vitreoretinal lymphoma presenting with retinal infiltrates and macular involvement carried a higher risk of CRA. Risk factors for CRA should be identified for the potential of severe visual loss. Prompt diagnosis of vitreoretinal lymphoma may allow better control of the disease.

Peripheral Hypertrophic Subepithelial Corneal Degeneration.

PURPOSE: The purpose of this study was to report a large series of patients with peripheral hypertrophic subepithelial corneal degeneration (PHSCD) and differentiate the condition from Salzmann nodular degeneration (SND). METHODS: We retrospectively reviewed the charts of 49 patients diagnosed with PHSCD and reported their clinical, refractive, and topographic/tomographic findings. RESULTS: Most of the eyes were white and quiet. Minimal variable injection was present in a few eyes usually in the presence of pseudopterygium. Typical corneal involvement consists of peripheral circumferential-elevated whitish subepithelial opacities with fine superficial vessels along the limbus and linear deposits of iron in the epithelium along the central edge of the opacification. The typical topographic/tomographic findings consist of flattening directly over the corneal opacification with central flattening aligning with the axis of the opacification. In all subjects, the mean refractive astigmatism was significantly less than the mean topographic/tomographic Sim K astigmatism. Thirty-five eyes underwent surgical excision. The surgical eyes demonstrated significantly less astigmatism and better best-spectacle corrected visual acuity than pre-op. Moreover, all the eyes that underwent surgery for discomfort experienced significant improvement in their symptoms. Histopathology of the keratectomy specimens demonstrated paucicellular subepithelial fibrosis with overlying epithelium that was variable in caliber. CONCLUSIONS: PHSCD is distinct from SND, primarily occurring in middle-aged women, bilateral and fairly symmetric with larger more peripheral opacities than SND, and absence of inflammatory signs and symptoms.

Analysis of Radial Peripapillary Capillary Density in Patients with Bietti Crystalline Dystrophy by Optical Coherence Tomography Angiography / 生物医学与环境科学（英文）

OBJECTIVE@#We wanted to investigate the radial peripapillary capillary (RPC) network in patients with Bietti crystalline dystrophy (BCD).@*METHODS@#We compared RPC densities in the disk and different peripapillary regions, obtained using optical coherence tomography angiography in 22 patients with BCD (37 eyes) and 22 healthy subjects (37 eyes). The BCD group was then divided into Stage 2 and Stage 3 subgroups based on Yuzawa staging, comparing the RPC densities of the two.@*RESULTS@#The disk area RPC density was 38.8% ± 6.3% in the BCD group and 49.2% ± 6.1% in the control group ( P < 0.001), and peripapillary region RPC density was significantly lower in the BCD group than in the control group (49.1% ± 4.7% and 54.1% ± 3.0%, respectively, P < 0.001). There were no significant RPC density differences between the tempo quadrant and inside disk of Stages 2 and 3 subgroups; the other areas showed a significantly lower RPC density in Stage 3 than in Stage 2 BCD.@*CONCLUSION@#The BCD group RPC density was significantly lower than the control group. The reduction of RPC density in the tempo quadrant occurred mainly in the Stage 1 BCD. In contrast, the reduction of RPC density in superior, inferior, and nasal quadrants occurred mainly in Stage 2.

Partial-Thickness Intrastromal Lamellar Keratoplasty for Corneal Pellucid Marginal Degeneration.

PURPOSE: Pellucid marginal degeneration (PMD) is a bilateral corneal ectatic disorder, characterized by thinning of the inferior cornea. Numerous surgical procedures have been described in the literature, and there is a lack of consensus regarding the best available surgical management. Recently, full-thickness intrastromal lamellar keratoplasty (ILK) was described as an option for treating PMD and emerged as a promising technique. However, ILK results in an irregular surface, leading to a significant residual against-therule (ATR) astigmatism. METHODS: This study describes a variation of the ILK technique, using a partial-thickness graft to treat 2 cases of PMD. RESULTS: At the final follow-up, both patients improved visual acuity and had their mean simulated keratometry flattened, although a residual ATR astigmatism remained. CONCLUSIONS: The ILK technique modification might lead to a more regular thickness pattern, although it did not seem efficient in reducing the ATR astigmatism.

Different mRNA expression patterns in keratoglobus and pellucid marginal degeneration keratocytes.

PURPOSE: Alike keratoconus (KC), keratoglobus (KG) and pellucid marginal degeneration (PMD) belong to ectatic corneal diseases. While there are numerous studies on keratoconus pathophysiology, there is no exact knowledge on genetic and pathophysiological background of KG and PMD, so far. It is not yet clarified, whether KG and PMD are independent clinical entities or represent different stages of the same disease. Our purpose was to investigate key parameters concerning collagen synthesis, intracellular LOX expression and inflammation in corneal stromal cells of KG and PMD subjects, in vitro. METHODS: Normal human keratocytes of corneas from the LIONS Cornea Bank Saar-Lor-Lux, Trier/Westpfalz and human keratocytes of KG and PMD patients were isolated and cultured as keratocytes. To examine Collagen I and V (Col I, Col V), heat shock protein 47 (Hsp47), Lysyl Oxidase (LOX), nuclear factor kappa B (NF-κB) mRNA and protein expression in all cell types, quantitative PCR and Western blot analysis has been performed. RESULTS: Col5A1 mRNA expression was significantly lower in KG and PMD keratocytes and LOX mRNA expression was significantly higher in KG-keratocytes, compared to controls. Col1A1, Hsp47 and NF-κB mRNA expression and the analyzed protein expressions did not differ from controls, in KG or PMD. CONCLUSIONS: Col5A1 mRNA expression is decreased in KG and PMD and LOX mRNA expression is increased in KG. Therefore, the pathophysiology of KG and PMD differs from KC and these seem to be from KC independent entities. The explanation of the peripheral corneal thinning in KG and PMD must be investigated in further studies.

Simplified, Readily Available Method for the Treatment of Band Keratopathy With Ethylenediaminetetraacetic Acid.

PURPOSE: To compare 3 methods for creating ethylenediaminetetraacetic acid (EDTA) solution using readily available Vacutainer tubes for the treatment of band keratopathy. METHODS: All 3 protocols used commercially available Vacutainer blood collection tubes coated with K2EDTA. An osmometer was used to measure and compare the concentration of EDTA created using 3 different protocols. The time required for preparation of the solution was measured and compared to evaluate its efficiency for everyday clinical use. In addition, volume of EDTA solution obtained was measured for method 1. The most promising protocol for clinical use was then used for treatment of a series of patients. RESULTS: Average osmolarity was 532, 285, and 422 for methods 1, 2, and 3, respectively (ANOVA P < 0.01, all Tukey honestly significant difference P < 0.01). For the respective mixtures, average concentration was 65, 35, and 52 mg/mL, and average time to create solution was 189, 38, and 83 seconds (ANOVA P < 0.01, all Tukey honestly significant difference P < 0.01). The most promising, method 3, was found to be safe and effective in removing calcium from the corneal stroma in a series of 5 patients with 6 eyes treated. It also yielded 25% more solution for clinical use than method 1. CONCLUSIONS: Method 3 using a single 10-mL Vacutainer tube with 18 mg of K2-EDTA had the best balance of effective concentration of EDTA, time to preparation, and simplicity of methodology, when compared with previously published methods 1 and 2. It also yielded a greater final volume of solution.

Crystals deposits in the anterior and posterior lens cortex in Bietti corneo-retinal dystrophy.

BACKGROUND: Whereas crystals deposit in the retina, the cornea and limbus in Bietty corneo-retinal dystrophy (BCD) is now well established and documented, only two published cases report their findings in the lens and no cases deep in the lens cortex. MATERIAL AND METHODS: Four consecutive adult patients from three different unrelated families presenting lens crystals associated with advanced genetically confirmed BCD were enrolled with advanced disease and long follow up (>12 years). Demographics, visual acuity, slit lamp biomicroscopy, lens and posterior pole photography, optical coherence tomography (OCT), autofluorescence, and screening for CYP4V2 type of mutation were performed. The setting was Jules Gonin Eye Hospital, Switzerland, between 1.1 2013 and 1.11. 2019. RESULTS: All patients were European women. The ages ranged from 40 to 81 years. Best Snellen visual acuity ranged from light perception to 1.0. All patients presented with limbus and retinal crystals deposit that disappeared over time and the development of severe chorioretinal atrophy. With long-term follow up, multiple crystal-like deposits appeared in the anterior, posterior lens capsule and cortex. All patients, but one, had homozygous or compound heterozygous mutations in CYP4V2 gene. CONCLUSIONS: To the best of our knowledge, there are no published cases of crystal deposits in the cortex of the lens of patients diagnosed with BCD associated with CYP4V2 gene mutation. This could be a feature of advanced BCD, and their presence in the lens cortex questions the hypothesis of floating deposits from posterior pole although their exact etiology remains to be determined.

North Carolina Macular Dystrophy: Phenotypic Variability and Computational Analysis of Disease-Associated Noncoding Variants.

Purpose: North Carolina macular dystrophy (NCMD) is an autosomal dominant, congenital disorder affecting the central retina. Here, we report clinical and genetic findings in three families segregating NCMD and use epigenomic datasets from human tissues to gain insights into the effect of NCMD-implicated variants. Methods: Clinical assessment and genetic testing were performed. Publicly available transcriptomic and epigenomic datasets were analyzed and the activity-by-contact method for scoring enhancer elements and linking them to target genes was used. Results: A previously described, heterozygous, noncoding variant upstream of the PRDM13 gene was detected in all six affected study participants (chr6:100,040,987G>C [GRCh37/hg19]). Interfamilial and intrafamilial variability were observed; the visual acuity ranged from 0.0 to 1.6 LogMAR and fundoscopic findings ranged from visually insignificant, confluent, drusen-like macular deposits to coloboma-like macular lesions. Variable degrees of peripheral retinal spots (which were easily detected on widefield retinal imaging) were observed in all study subjects. Notably, a 6-year-old patient developed choroidal neovascularization and required treatment with intravitreal bevacizumab injections. Computational analysis of the five single nucleotide variants that have been implicated in NCMD revealed that these noncoding changes lie within two putative enhancer elements; these elements are predicted to interact with PRDM13 in the developing human retina. PRDM13 was found to be expressed in the fetal retina, with greatest expression in the amacrine precursor cell population. Conclusions: We provide further evidence supporting the role of PRDM13 dysregulation in the pathogenesis of NCMD and highlight the usefulness of widefield retinal imaging in individuals suspected to have this condition.

Surgical outcomes of re-excimer laser phototherapeutic keratectomy (re-PTK).

To assess the surgical outcomes of re-excimer laser phototherapeutic keratectomy (re-PTK) for recurrent disease after initial PTK. Retrospective cohort study with historical comparison group. This study involved 56 patients who underwent re-PTK (mean follow-up period: 47.9 ± 36.2 months) at the Baptist Eye Institute, Kyoto, Japan. In all subjects, corrected-distance visual acuity (CDVA) before and after re-PTK was compared. Postoperative recurrence of corneal lesion with a decrease of CDVA of two lines or more was compared with postoperative best CDVA deemed as a significant relapse. The Kaplan-Meier method was used to compare the recurrence rate post-re-PTK with that after the initial PTK. The disease classification in the 78 eyes was heterozygous-type GCD (hetero-GCD, n = 47 eyes), homozygous-type GCD (homo-GCD, n = 13 eyes), band keratopathy (n = 7 eyes), lattice corneal dystrophy (n = 6 eyes), and other (n = 5 eyes). After re-PTK, homo-GCD recurred statistically significantly earlier than hetero-GCD (P = 0.0042). No significant difference was found in the recurrence rate for all diseases between post-re-PTK and post initial PTK (P > 0.05). Surgical outcomes after re-PTK were nearly equal to those after initial-PTK.

Descemet Membrane Endothelial Keratoplasty Outcomes between Young and Old Graft Recipients.

Purpose: To evaluated Descemet's membrane endothelial keratoplasty (DMEK) outcomes in young and old graft recipients.Materials and Methods: Data of 164 surgeries with a median age of 76 years (interquartile range 14 years) undergoing DMEK surgery between 2016 and 2018 was reviewed. Complications, graft survival, and visual acuity gain were compared between subjects in the 25th percentile (young recipients; aged 70 years and less, n = 21) and 75th percentile (old recipients; aged 85 years and over, n = 27) over the 2-year follow-up.Results: Young recipients had a lower rate of pre-operative glaucoma (14.3% vs. 51.9%, p = .014) and pseudophakic bullous keratopathy (9.5% vs. 59.3%, p < .001), and a higher rate of Fuchs endothelial dystrophy (57.1% vs. 14.8%, p = .002) and combined cataract extraction at the time of DMEK surgery (52.4% vs. 7.4%, p = .001) when compared to old recipients. Complications (primary graft failure, pupillary block, cystoid macular edema or infectious keratitis) were independent of graft recipient age. Descemet's membrane detachment requiring re-bubbling was observed more often in young compared to the old recipients (42.9% vs. 14.8%, p = .049). Visual acuity gain between the groups remained comparable up to 1-year, whereas at 2-years old recipients showed significantly declined visual acuity gains compared to the young recipients (0.14 ± 0.68 vs. 0.74 ± 0.49, p = .012). Graft recipients aged over 85 years had a considerably higher graft failure rate over the 24-months (40.7% vs. 4.8%, p = .006) and shorter graft survival time (p = .002; log-rank) when compared to the recipients aged under 70 years. After adjusting for potential confounders such as Fuchs endothelial dystrophy, pseudophakic bullous keratopathy and glaucoma, the recipients aged over 85 remained at higher risk for graft failure (HR = 17.278, 95% CI = 1.787-167.1, p = .014).Conclusions: In aged DMEK recipients, regardless of the low incidence of early postoperative complications, the rate of postoperative graft failure was significantly higher and graft survival shorter than in younger recipients.

Predictors of Vision-Related Quality of Life After Endothelial Keratoplasty in the Descemet Endothelial Thickness Comparison Trials.

PURPOSE: To determine which baseline characteristics are associated with vision-related quality of life (QOL) after endothelial keratoplasty. METHODS: A patient- and outcome-masked randomized clinical trial. Consecutive patients presenting with isolated endothelial disease requiring endothelial keratoplasty at Oregon Health Sciences University and Stanford University. Study eyes randomized to one of the 2 types of endothelial keratoplasty were given the national eye institute vision function questionnaire 25 at baseline and 12 months. In this prespecified secondary outcome, we assessed the role of recipient and donor characteristics as predictors of the 12-month national eye institute vision function questionnaire 25 change score. RESULTS: The mean vision function questionnaire (VFQ) score was 74 (SD 14) at baseline and increased to 82 (SD 12) at 3 months and 87 (SD 10) at 12 months. We were unable to find a difference in vision-related QOL in study participants who underwent Descemet membrane endothelial keratoplasty compared with ultrathin Descemet stripping endothelial keratoplasty [coef -0.98, 95% confidence interval (CI) -9.27 to 7.31; P = 0.82]. In multivariable analysis, patients who underwent endothelial keratoplasty in 2 eyes had approximately 8 points higher VFQ at 12 months after the second eye than those who had only one eye enrolled (95% CI 0.10-15.72; P = 0.047). Posterior densitometry produced an approximately 1-point increase each in the VFQ change score for each 1 grayscale unit increase at baseline (95% CI 0.26-1.81; P = 0.009). Although the sample size was small, a diagnosis of pseudophakic bullous keratopathy had approximately 19-points more improvement on average compared with Fuchs (95% CI 7.68-30.00; P = 0.001). CONCLUSIONS: Bilateral endothelial keratoplasty resulted in higher vision-related QOL compared with unilateral. Baseline densitometry, objective measure of corneal haze that predicts vision-related QOL, may play a role in monitoring disease progression.

Role of Anterior Segment OCT for Descemet Membrane Stripping During Descemet Membrane Endothelial Keratoplasty in Eyes With Congenital Hereditary Endothelial Dystrophy.

PURPOSE: To report the utility of preoperative anterior segment optical coherence tomography (ASOCT) in decision-making for stripping of the Descemet membrane (DM) during Descemet membrane endothelial keratoplasty (DMEK) in eyes with congenital hereditary endothelial dystrophy (CHED). METHODS: ASOCT was performed in eyes with CHED undergoing DMEK. Host DM was retained if the DM appearance was normal on ASOCT. Outcomes of DMEK with or without DM stripping (non DM stripping [DMEK]) were analyzed and compared regarding graft adhesion, graft clarity, visual acuity, and complications. RESULTS: Twelve eyes of 8 patients were included in the series. Of these, 6 eyes (mean age, 6 ± 2.6 years, range 3-8 years) underwent nDMEK and 6 eyes (mean age, 23.6 ± 10.2 years, range 13-39 years) underwent standard DMEK. There were no intraoperative complications in either group. During the early postoperative period, one eye in the nDMEK group had donor detachment that was successfully managed by repeat air injection. The mean preoperative and postoperative corrected visual acuities were 1.15 and 0.37 logarithm of the minimum angle of resolution (LogMAR) in the nDMEK group, and 0.95 and 0.25 LogMAR in the DMEK group (P 0.39 and 0.06). Average endothelial cell counts were 1826 ± 318 cell/mm in the nDMEK group (32.3% loss), and 1708 ± 271 cells/mm in the DMEK group (33.6% loss) at the last follow-up. CONCLUSIONS: Preoperative anterior segment OCT is useful in decision-making regarding stripping of host DM during DMEK in eyes with CHED. The outcomes of nDMEK were similar to DMEK in this small series.

RETINAL AND CHOROIDAL BLOOD PERFUSION IN PATIENTS WITH BIETTI CRYSTALLINE DYSTROPHY.

PURPOSE: To compare changes of chorioretinal blood perfusion between Bietti crystalline dystrophy (BCD) and typical retinitis pigmentosa and perform a staging and a longitudinal analysis of chorioretinal perfusion in BCD. METHODS: Twenty-eight patients with BCD (56 eyes), 28 patients with typical retinitis pigmentosa (56 eyes), and 28 healthy subjects (56 eyes) were enrolled. Macular structural parameters and subfoveal choroidal thickness were measured using optical coherence tomography. Retinal vessel and perfusion densities were calculated using optical coherence tomography angiography. Choroidal blood perfusion was assessed through indocyanine green angiography. The results of the BCD group were compared with those of the retinitis pigmentosa and control groups and followed by a staging and a longitudinal analysis of BCD. RESULTS: Macular structural and perfusion parameters were decreased less in the BCD group than those in the retinitis pigmentosa group. Subfoveal choroidal thickness was significantly thinner in the BCD group, with a remarkable choroidal perfusion deficit using indocyanine green angiography. The staging analysis revealed damage of both retinal and choroidal perfusion in BCD; however, the longitudinal analysis showed the impairment of choroidal perfusion outweighed retinal. CONCLUSION: Both retinal and choroidal blood perfusion are impaired in BCD, but choroidal perfusion deficit caused by CYP4V2 mutations may play a more vital pathologic role.

Pediatric Descemet Membrane Endothelial Keratoplasty.

PURPOSE: To report the technique and outcome of Descemet membrane endothelial keratoplasty (DMEK) in pediatric patients older than 6 years of age. DESIGN: Institutional interventional retrospective case series. METHODS: This study included 5 eyes of patients less than 15 years of age with endothelial dysfunction who underwent DMEK. Three eyes had Descemet stripping done of the same size as the donor graft. Two eyes underwent non-Descemet stripping endothelial keratoplasty. Attachment of DMEK scroll and improvement in corneal clarity, vision, pachymetry, and intraoperative or postoperative complication was noted. We defined primary graft failure as nonclearing corneal edema despite a well-attached lenticule on anterior segment optical coherence tomography. RESULTS: A total of 5 eyes of 5 children (all male) with a mean (± standard deviation) age of 9.2 ± 3.42 years underwent DMEK. The mean preoperative visual acuity of 1.93 ± 0.25 logMAR units improved postoperatively to 0.98 ± 0.29 (95% confidence interval, P = .03). Anatomic success (well-attached scroll with a more transparent cornea with a decrease in pachymetry) was seen in 4 of 5 eyes (80%). One eye had a primary donor failure. Two out of the 5 eyes (40%) required rebubbling. The mean pachymetry in eyes with successful procedure reduced from 1094.5 ± 101.5 µm to 619.25 ± 150.3 µm (P = .03). CONCLUSIONS: Although DMEK in pediatric eyes is challenging, the early results are encouraging, and it is a viable option in pediatric patients with endothelial failure.

Exacerbation of Granular Corneal Dystrophy Type 2 After Small Incision Lenticule Extraction.

PURPOSE: To report the outcome of unilateral small incision lenticule extraction (SMILE) in a patient with granular corneal dystrophy type 2 (GCD2). METHODS: Slit-lamp photography and Fourier domain optical coherence tomography were used to document the clinical course and appearance of the corneas in a patient with genetically determined GCD2 who underwent unilateral SMILE in the right eye. RESULTS: Slit-lamp examination of a 23-year-old woman revealed 2 faint opacities at the surgical interface approximately 2 months after the SMILE procedure had been performed on her right eye. Nine and 3 typical GCD2 deposits located immediately beneath the Bowman layer were observed in the right and left corneas, respectively. Over time, the deposits at the interface increased in size, density, and number in the right eye. Fourier domain optical coherence tomography performed 33 months after the SMILE procedure revealed deposits at the SMILE interface that were distinct from those located immediately beneath the Bowman layer. The severity of disease exacerbation was less in this patient than what is typically observed in others who have undergone laser-assisted in situ keratomileusis or photorefractive keratectomy. CONCLUSIONS: SMILE is contraindicated in patients with GCD2, as are other corneal refractive surgical procedures. This case highlights the importance of genetic testing before the performance of refractive corneal procedures-especially for patients with corneal opacities on preoperative slit-lamp examination or a family history of corneal disease compatible with that of a corneal dystrophy.

TOPICAL DORZOLAMIDE FOR CYSTOID MACULAR EDEMA IN BIETTI CRYSTALLINE RETINAL DYSTROPHY.

PURPOSE: To report a case of Bietti crystalline retinal dystrophy with cystoid macular edema (CME) that was successfully treated with topical carbonic anhydrase inhibitor. METHODS: A 35-year-old otherwise healthy woman, with a known case of Bietti crystalline retinal dystrophy, presented with progressive visual impairment in her right eye for 3 months. The best-corrected visual acuity was 20/50 in the right eye and 20/25 in the left eye. On the basis of the multimodal imaging findings, the patient was diagnosed with Bietti crystalline retinal dystrophy with unilateral CME. Carbonic anhydrase inhibitor therapy twice a day was initiated. RESULTS: Three months later, visual acuity improved to 20/25 in the right eye, and CME had resolved based on spectral domain ocular coherence tomography findings, although the CME reoccurred after discontinuation of the drug. Three months after resuming the therapy, the best-corrected visual acuity improved back to 20/25. CONCLUSION: Cystoid macular edema is one of the main causes of central visual worsening in patients with Bietti crystalline retinal dystrophy. This complication may be resolved with topical carbonic anhydrase inhibitor, resulting in improved anatomical and visual outcomes.