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PURPOSE: The aim of this study was to explore the potential benefits of retinal pigment epithelium replacement therapy in patients with Bietti crystalline dystrophy (BCD) by assessing the disease pathology with the distinctive relationship between fundus autofluorescence (FAF) abnormality and visual field defect. METHODS: Sixteen eyes from 16 patients with BCD and 16 eyes from 16 patients with RHO-associated retinitis pigmentosa were included. Fundus autofluorescence, optical coherence tomography, and Goldmann perimetry results were retrospectively reviewed and assessed using image analyses. RESULTS: In patients with BCD, the FAF abnormality area was not correlated with the overall visual field defect area and median overall visual field defect area (57.5%) was smaller than FAF abnormality area (98.5%). By contrast, the ellipsoid zone width was significantly correlated with the central visual field area (r = 0.806, P < 0.001). In patients with RHO-associated retinitis pigmentosa, the FAF abnormality area and ellipsoid zone width were significantly correlated with the overall visual field defect area (r = 0.833, P < 0.001) and central visual field area (r = 0.887, P < 0.001), respectively. CONCLUSION: The FAF abnormality shown in patients with BCD involves retinal pigment epithelium degeneration without complete loss of photoreceptors or visual function. These results suggest that patients with BCD are good candidates for retinal pigment epithelium replacement therapy for preservation of residual visual function.
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Distrofias Hereditarias de la Córnea , Angiografía con Fluoresceína , Fondo de Ojo , Epitelio Pigmentado de la Retina , Tomografía de Coherencia Óptica , Agudeza Visual , Pruebas del Campo Visual , Campos Visuales , Humanos , Campos Visuales/fisiología , Femenino , Masculino , Estudios Retrospectivos , Persona de Mediana Edad , Tomografía de Coherencia Óptica/métodos , Distrofias Hereditarias de la Córnea/diagnóstico , Distrofias Hereditarias de la Córnea/fisiopatología , Angiografía con Fluoresceína/métodos , Adulto , Epitelio Pigmentado de la Retina/patología , Anciano , Agudeza Visual/fisiología , Enfermedades de la Retina/diagnóstico , Enfermedades de la Retina/fisiopatología , Trastornos de la Visión/fisiopatología , Trastornos de la Visión/diagnóstico , Imagen Óptica , Retinitis Pigmentosa/fisiopatología , Retinitis Pigmentosa/diagnóstico , Adulto JovenRESUMEN
Retinitis pigmentosa (RP) is the most common inherited retinal dystrophy and a major cause of blindness. RP is caused by several variants of multiple genes, and genetic diagnosis by identifying these variants is important for optimizing treatment and estimating patient prognosis. Next-generation sequencing (NGS), which is currently widely used for diagnosis, is considered useful but is known to have limitations in detecting copy number variations (CNVs). In this study, we re-evaluated CNVs in EYS, the main causative gene of RP, identified via NGS using multiplex ligation-dependent probe amplification (MLPA). CNVs were identified in NGS samples of eight patients. To identify potential CNVs, MLPA was also performed on samples from 42 patients who were undiagnosed by NGS but carried one of the five major pathogenic variants reported in Japanese EYS-RP cases. All suspected CNVs based on NGS data in the eight patients were confirmed via MLPA. CNVs were found in 2 of the 42 NGS-undiagnosed RP cases. Furthermore, results showed that 121 of the 661 patients with RP had EYS as the causative gene, and 8.3% (10/121 patients with EYS-RP) had CNVs. Although NGS using the CNV calling criteria utilized in this study failed to identify CNVs in two cases, no false-positive results were detected. Collectively, these findings suggest that NGS is useful for CNV detection during clinical diagnosis of RP.
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Variaciones en el Número de Copia de ADN , Proteínas del Ojo , Secuenciación de Nucleótidos de Alto Rendimiento , Retinitis Pigmentosa , Humanos , Retinitis Pigmentosa/genética , Retinitis Pigmentosa/diagnóstico , Secuenciación de Nucleótidos de Alto Rendimiento/métodos , Femenino , Masculino , Proteínas del Ojo/genética , Persona de Mediana Edad , Adulto , Reacción en Cadena de la Polimerasa Multiplex/métodosRESUMEN
Clinical studies using suspensions or sheets of human pluripotent cell-derived retinal pigment epithelial cells (hiPSC-RPE) have been conducted globally for diseases such as age-related macular degeneration. Despite being minimally invasive, cell suspension transplantation faces challenges in targeted cell delivery and frequent cell leakage. Conversely, although the RPE sheet ensures targeted delivery with correct cell polarity, it requires invasive surgery, and graft preparation is time-consuming. We previously reported hiPSC-RPE strips as a form of quick cell aggregate that allows for reliable cell delivery to the target area with minimal invasiveness. In this study, we used a microsecond pulse laser to create a local RPE ablation model in cynomolgus monkey eyes. The hiPSC-RPE strips were transplanted into the RPE-ablated and intact sites. The hiPSC-RPE strip stably survived in all transplanted monkey eyes. The expansion area of the RPE from the engrafted strip was larger at the RPE injury site than at the intact site with no tumorigenic growth. Histological observation showed a monolayer expansion of the transplanted RPE cells with the expression of MERTK apically and collagen type 4 basally. The hiPSC-RPE strip is considered a beneficial transplantation option for RPE cell therapy.
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Células Madre Pluripotentes Inducidas , Macaca fascicularis , Epitelio Pigmentado de la Retina , Animales , Epitelio Pigmentado de la Retina/trasplante , Epitelio Pigmentado de la Retina/citología , Humanos , Células Madre Pluripotentes Inducidas/citología , Degeneración Macular/patologíaRESUMEN
This study aimed to investigate how the extent and central/peripheral location of the residual visual field (VF) in patients with late-stage inherited retinal diseases (IRDs) are related to retinal sensitivity detected using full-field stimulus testing (FST). We reviewed the results of Goldmann perimetry and FST from the medical records of patients with IRDs whose VF represents central (within 10°) and/or peripheral islands, or undetectable. In total, 19 patients (19 eyes) were analyzed in this study. The median value of residual VF area was 1.38%. The median values of rod and cone sensitivities were - 14.9 dB and 7.4 dB, respectively. Patients with only the peripheral island (- 33.9 dB) had better median rod sensitivity than other groups (only central, - 18.9 dB; both, - 3.6 dB). VF area significantly correlated with rod sensitivity (r = - 0.943, p = 0.005) in patients with only peripheral island, but not with cone sensitivity. Peripheral VF islands were significant contributors to FST results, especially rod sensitivity. With reduced or loss of central vision, the extent of residual peripheral VF significantly affected rod sensitivity, suggesting that FST can be useful in quantitatively estimating the overall remaining vision in patients with late-stage IRD.
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Degeneración Retiniana , Campos Visuales , Humanos , Pruebas del Campo Visual/métodos , Adaptación a la Oscuridad , RetinaRESUMEN
OBJECTIVE: Cystoid macular edema (CME) in retinitis pigmentosa (RP) is an important complication causing visual dysfunction. We investigated the effect of CME on photoreceptors in RP patients with previous or current CME, using an adaptive optics (AO) fundus camera. METHODS: We retrospectively observed the CME and ellipsoid zone (EZ) length (average of horizontal and vertical sections) by optical coherence tomography. The density and regularity of the arrangement of photoreceptor cells (Voronoi analysis) were examined at four points around 1.5° from superior to inferior and temporal to nasal. We also performed a multivariate analysis using CME duration, central macular thickness and transversal length of CME. RESULTS: We evaluated 18 patients with previous or current CME (18 eyes; age, 48.7 ± 15.6 years) and 24 patients without previous or current CME (24 eyes; age, 46.0 ± 14.5 years). There were no significant differences in age, logMAR visual acuity, or EZ length. In groups with and without CME, cell density was 11967 ± 3148 and 16239 ± 2935 cells/mm2, and sequence regularity was 85.5 ± 3.4% and 88.5 ± 2.8%, respectively; both parameters were significantly different. The correlation between photoreceptor density and age was more negative in group with CME. The CME group tended toward greater reductions in duration of CME. CONCLUSION: Complications of CME in RP patients may lead to a decrease in photoreceptor density and regularity. Additionally, a longer duration of CME may result in a greater reduction in photoreceptor density.
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Edema Macular , Retinitis Pigmentosa , Humanos , Adulto , Persona de Mediana Edad , Edema Macular/complicaciones , Estudios Retrospectivos , Retinitis Pigmentosa/complicaciones , Retinitis Pigmentosa/diagnóstico por imagen , Fóvea Central , Tomografía de Coherencia Óptica/métodos , Células FotorreceptorasRESUMEN
Transplantation of induced pluripotent stem cell (iPSC)-derived retinal organoids into retinal disease animal models has yielded promising results, and several clinical trials on iPSC-derived retinal pigment epithelial cell transplantation have confirmed its safety. In this study, we performed allogeneic iPSC-derived retinal organoid sheet transplantation in two subjects with advanced retinitis pigmentosa (jRCTa050200027). The primary endpoint was the survival and safety of the transplanted retinal organoid sheets in the first year post-transplantation. The secondary endpoints were the safety of the transplantation procedure and visual function evaluation. The grafts survived in a stable condition for 2 years, and the retinal thickness increased at the transplant site without serious adverse events in both subjects. Changes in visual function were less progressive than those of the untreated eye during the follow-up. Allogeneic iPSC-derived retinal organoid sheet transplantation is a potential therapeutic approach, and the treatment's safety and efficacy for visual function should be investigated further.
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Células Madre Pluripotentes Inducidas , Retinitis Pigmentosa , Animales , Humanos , Retina , Retinitis Pigmentosa/terapia , Visión Ocular , OrganoidesRESUMEN
Purpose: Novel therapeutic options, such as regenerative medicine and gene therapy, are now emerging as viable treatment options for patients with severe visual impairments, such as retinitis pigmentosa (RP). Gradable assessment of patients' visual function is essential to consider treatment options and to evaluate treatment outcomes; however, evaluation of visual function in patients with advanced low vision is often challenging because of patients' poor and sometimes unpredictable responses. In this study, we attempted to accurately assess visual capabilities and disease stage in patients with RP with a visual acuity (VA) of ≤ 0.01. Design: Retrospective analysis of visual function indicators, including VA, retinal thickness, full-field stimulus testing (FST), and chromatic pupillometry. Subjects: Overall, 43 patients (84 eyes) with advanced RP with a VA of ≤ 0.01 visited Kobe City Eye Hospital from 2019 to 2021. Methods: Hierarchical (multilevel) Bayesian modeling was used to estimate individual eye's pupil response and FST threshold, taking into account the ambiguity and randomness often observed in patients with ultralow vision. Using the estimated ability obtained from each test, the correlation between each test and retinal thickness was further analyzed to make a comprehensive assessment of the data. Main Outcome Measures: Visual acuity, retinal thickness, FST threshold, and pupil diameter change to different light stimuli. Results: Full-field stimulus testing and pupillometry measurements were moderately correlated with VA but exhibited a wide range of values within the same VA groups. Full-field stimulus testing was not correlated with central retinal thickness at counting fingers/hand motion VA range and seemed to reflect overall remaining photoreceptor function, including peripheral retina. Pupillometry may be able to distinguish between different levels of inner retinal function. Conclusions: The combination of pupillometry and FST allowed for graded evaluation of visual function within patients grouped in the same VA groups in patients with advanced RP with ultralow vision. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.
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PURPOSE: To describe a novel case of bilateral rapidly progressive retinopathy after immunotherapy with pembrolizumab for metastatic urothelial carcinoma. METHODS: Case report. RESULTS: A 64-year-old man undergoing pembrolizumab immunotherapy was referred to our hospital because of bilateral acute vision loss. His best-corrected visual acuity was 20/30 in the right eye and 20/320 in the left eye, and a visual field test revealed central and paracentral scotomas in the right eye and central scotoma in the left eye. We suspected immune-related retinopathy based on the progressive photoreceptor damage with abnormal electroretinogram findings, absence of overt intraocular inflammation, and presence of malignancy. Cessation of pembrolizumab and steroid pulse therapy followed by decreasing oral prednisolone improved visual function and photoreceptor damage, although there was recurrence after pembrolizumab was restarted. CONCLUSION: We reported a case of rapidly progressive retinopathy that may have been triggered by pembrolizumab immunotherapy for metastatic urothelial carcinoma. High-dose corticosteroid pulse therapy was effective in reversing photoreceptor damage.
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Carcinoma de Células Transicionales , Enfermedades de la Retina , Neoplasias de la Vejiga Urinaria , Masculino , Humanos , Persona de Mediana Edad , Enfermedades de la Retina/inducido químicamente , Ceguera , Escotoma , Inmunoterapia/efectos adversosRESUMEN
PURPOSE: To identify the genotypic and phenotypic characteristics of rhodopsin (RHO)-associated retinitis pigmentosa (RP) in the Japanese population. STUDY DESIGN: Cross-sectional, single-center study METHODS: The medical records of 1336 patients with RP who underwent genetic testing at our clinic between November 2008 and September 2021 were reviewed, and patients with RHO variants were included. The patients were divided into class A and class B to assess genotype-phenotype correlations based on previous reports. The clinical findings, including best-corrected visual acuity (BCVA), OCT parameters (ellipsoid zone [EZ] width and central retinal thickness [CRT]), and presence of macular degeneration, of the 2 groups were compared. RESULTS: The study included 28 patients diagnosed with RHO-associated RP (class A, 19; class B, 9). The BCVA was significantly worse in class A patients than in class B patients (P = 0.045). Superior EZ width was significantly shorter in class A than in class B patients (P = 0.016). Class A patients tended to have thinner CRT and shorter inferior EZ width than those of class B patients, although this difference was not significant. Macular degeneration was observed in 61.5% of class A and 12.5% of class B patients, demonstrating that macular degeneration can be a common complication in class A variants. CONCLUSION: Patients with class A variants presented with a severer form of RP than that of patients with class B variants in the Japanese population. These results suggest that the phenotype of RHO-associated RP is linked to the location of the variants and that such a genotype-phenotype correlation is less affected by ethnicities with different genetic backgrounds.
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Degeneración Macular , Retinitis Pigmentosa , Humanos , Rodopsina/genética , Estudios Transversales , Pueblos del Este de Asia , Tomografía de Coherencia Óptica/métodos , Retinitis Pigmentosa/diagnóstico , Retinitis Pigmentosa/genética , Fenotipo , GenotipoRESUMEN
PURPOSE: To investigate the long-term outcomes of cataract surgery for glaucoma management in patients with primary angle-closure disease (PACD). STUDY DESIGN: Retrospective case series. METHODS: We reviewed the medical records of 87 eyes of 87 patients with PACD who underwent uncomplicated cataract surgery alone at the Kobe City Medical Center General Hospital. Only patients with a minimum follow-up of 10 years were included. The patients were divided into PACD spectrum categories: primary angle-closure glaucoma (PACG), primary-angle closure (PAC), and primary angle-closure suspect (PACS). The treatment outcomes were compared among the 3 groups. Intraocular pressure (IOP), number of glaucoma eye drops, requirement of additional glaucoma treatment, visual field progression, and progression to glaucoma during the follow-up period were evaluated. RESULTS: Among the 87 patients, 39 had PACG; 26, PAC; and 22, PACS. Ten years after surgery, the IOP had significantly decreased from baseline in all 3 groups. The rate of requirement of additional glaucoma treatment during the follow-up period was significantly higher in the PACG group than in the other groups. Almost half of the patients with PACG required additional glaucoma treatment; of those patients, six (15.4%) underwent glaucoma surgery. Three patients (11.5%) with PAC required additional glaucoma medication. Visual field progression was observed in 28.1% of the patients with PACG. In 1 patient with PAC, the condition progressed to PACG, but there was no such progression in any of the patients with PACS. CONCLUSIONS: We confirmed that cataract surgery had a long-term (> 10 years) effect on IOP reduction in eyes with PACD. Early intervention with cataract surgery may be preferable for glaucoma management in patients with PACD.
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Extracción de Catarata , Catarata , Glaucoma de Ángulo Cerrado , Glaucoma , Humanos , Catarata/complicaciones , Glaucoma/complicaciones , Glaucoma de Ángulo Cerrado/complicaciones , Glaucoma de Ángulo Cerrado/cirugía , Presión Intraocular , Estudios RetrospectivosRESUMEN
OBJECTIVE: To predict the visual prognosis of cataract surgery in patients with retinitis pigmentosa by measuring ellipsoid zone (EZ) width using spectral-domain optical coherence tomography. METHODS: This retrospective study included patients with retinitis pigmentosa who underwent uncomplicated cataract surgery between December 2017 and June 2020. Preoperative best-corrected visual acuity (BCVA) and the best postoperative BCVA during follow-up were collected. EZ width was measured on preoperative cross-sectional optical coherence tomography images along the horizontal/vertical meridian through the fovea. RESULTS: Thirty-eight eyes of 38 patients (22 female; mean [±standard deviation] age, 62.1 ± 11.8 years) were included. The median preoperative logarithm of the minimum angle of resolution BCVA of 0.52 (range, 0.00-3.00) significantly improved to 0.07 (range, -0.18-3.00) after surgery (P < 0.001). On preoperative spectral-domain optical coherence tomography images, the median horizontal, vertical, and average EZ widths were 783 (range, 0-9837), 761 (range, 0-10 250), and 769 (range, 0-10 043) µm, respectively. Postoperative BCVA significantly correlated with the horizontal (r = -0.784, P < 0.001), vertical (r = -0.777, P < 0.001), and average EZ widths (r = -0.777, P < 0.001). The area under the receiver operating characteristic curve for the ability of the horizontal, vertical, and average EZ widths to discriminate eyes with and without postoperative BCVA ≤ 0.3 was 0.971, 0.960, and 0.963, respectively, with best cut-off values of 513, 608, and 515 µm, respectively. CONCLUSIONS: EZ width measurement can help predict the visual prognosis of cataract surgery in patients with retinitis pigmentosa. A preferable visual acuity prognosis can be expected in patients with an EZ width of approximately 600 µm.
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Opacificación Capsular , Retinitis Pigmentosa , Humanos , Femenino , Persona de Mediana Edad , Anciano , Estudios Retrospectivos , Estudios Transversales , Retinitis Pigmentosa/complicaciones , Retinitis Pigmentosa/diagnóstico , Retina , Pronóstico , Tomografía de Coherencia Óptica/métodosRESUMEN
We investigated the potential of ChatGPT in the ophthalmological field in the Japanese language using board examinations for specialists in the Japanese Ophthalmology Society. We tested GPT-3.5 and GPT-4-based ChatGPT on five sets of past board examination problems in July 2023. Japanese text was used as the prompt adopting two strategies: zero- and few-shot prompting. We compared the correct answer rate of ChatGPT with that of actual examinees, and the performance characteristics in 10 subspecialties were assessed. ChatGPT-3.5 and ChatGPT-4 correctly answered 112 (22.4%) and 229 (45.8%) out of 500 questions with simple zero-shot prompting, respectively, and ChatGPT-4 correctly answered 231 (46.2%) questions with few-shot prompting. The correct answer rates of ChatGPT-3.5 were approximately two to three times lower than those of the actual examinees for each examination set (p = 0.001). However, the correct answer rates for ChatGPT-4 were close to approximately 70% of those of the examinees. ChatGPT-4 had the highest correct answer rate (71.4% with zero-shot prompting and 61.9% with few-shot prompting) in "blepharoplasty, orbit, and ocular oncology," and the lowest answer rate (30.0% with zero-shot prompting and 23.3% with few-shot prompting) in "pediatric ophthalmology." We concluded that ChatGPT could be one of the advanced technologies for practical tools in Japanese ophthalmology.
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Transplantation of embryonic/induced pluripotent stem cell-derived retina (ESC/iPSC-retina) restores host retinal ganglion cell light responses in end-stage retinal degeneration models with host-graft synapse formation. We studied the immunological features of iPSC-retina transplantation using major histocompatibility complex (MHC)-homozygote monkey iPSC-retinas in monkeys with laser-induced retinal degeneration in MHC-matched and -mismatched transplantation. MHC-mismatched transplantation without immune suppression showed no evident clinical signs of rejection and histologically showed graft maturation without lymphocytic infiltration, although immunological tests using peripheral blood monocytes suggested subclinical rejection in three of four MHC-mismatched monkeys. Although extensive photoreceptor rosette formation was observed on histology, evaluation of functional integration using mouse models such as mouse ESC-retina (C57BL/6) transplanted into rd1(C3H/HeJ, MHC-mismatched model) elicited light responses in the host retinal ganglion cells after transplantation but with less responsiveness than that in rd1-2J mice (C57BL/6, MHC-matched model). These results suggest the reasonable use of ESC/iPSC-retina in MHC-mismatched transplantation, albeit with caution.
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Células Madre Pluripotentes Inducidas , Degeneración Retiniana , Ratones , Animales , Células Madre Pluripotentes Inducidas/patología , Degeneración Retiniana/patología , Ratones Endogámicos C57BL , Ratones Endogámicos C3H , Retina/patología , Primates , Complejo Mayor de Histocompatibilidad , Haplorrinos , Antígenos de HistocompatibilidadRESUMEN
This study aimed to evaluate the distribution of retinal pigment epithelium (RPE) melanin in patients with retinitis pigmentosa (RP) using entropy measurements by custom-made polarization-sensitive optical coherence tomography (PS-OCT) images, and compare entropy with the intensity of short-wavelength (SW) and near-infrared (NIR) autofluorescence (AF). We retrospectively reviewed the retinal images, including PS-OCT, SW-AF, and NIR-AF of patients with RP who had a hyperautofluorescent ring on AF. A total of 12 eyes of 12 patients (8 women and 4 men; mean age: 37.9 years) were included. There was a strong positive correlation between entropy value and NIR-AF intensity (r = 0.626, p < 0.001), and there was a very weak negative correlation between entropy value and SW-AF (r = - 0.197, p = 0.001). The mean values of the entropy in the foveal, temporal (2 mm from the fovea), and nasal (2 mm from the fovea) sections were 0.41 (± 0.09), 0.29 (± 0.08), and 0.26 (± 0.08), respectively. The entropy was significantly higher in the foveal section than in the temporal and nasal sections (p = 0.002 and p = 0.003, respectively). There was no significant difference between the entropies values for the temporal and nasal sections (p = 0.157). Age, logMAR best-corrected visual acuity, ellipsoid zone width, and central retinal thickness were not correlated with foveal entropy. We presented RPE melanin imaging in patients with RP using PS-OCT for the first time. PS-OCT can be a useful tool for monitoring patients with RP.
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Retinitis Pigmentosa , Tomografía de Coherencia Óptica , Adulto , Femenino , Angiografía con Fluoresceína/métodos , Humanos , Masculino , Melaninas , Epitelio Pigmentado de la Retina/diagnóstico por imagen , Retinitis Pigmentosa/diagnóstico por imagen , Estudios Retrospectivos , Tomografía de Coherencia Óptica/métodosRESUMEN
AIM: To investigate the one-year outcomes and factors that influence the results of microhook ab interno trabeculotomy (µLOT). METHODS: The medical records of consecutive patients with open angle glaucoma who underwent µLOT (including combination of µLOT and cataract surgery) between February 2018 and July 2019 were retrospectively reviewed. Surgical success was defined as the following: an intraocular pressure (IOP)≤21 mm Hg or IOP≤preoperative IOP with a reduced number of glaucoma eye drops, without additional glaucoma surgery, and assessed using Kaplan-Meier survival analysis. A multivariate Cox proportional-hazards regression model was used to investigate the factors associated with surgical failure. RESULTS: The 59 eyes of 59 patients comprising 28 eyes with primary open angle glaucoma (POAG) and 31 with secondary open angle glaucoma (SOAG) were included. The mean IOP and number of glaucoma eye drops significantly decreased from 25.3±7.2 mm Hg and 3.9±1.1, preoperatively to 16.1±4.4 mm Hg (P<0.01) and 2.1±1.8 (P<0.01), respectively, 12mo postoperatively, with a cumulative success rate of 63.1%. The one-year success rate was significantly higher in POAG eyes than in SOAG eyes (80.0% vs 48.0%; P=0.011, log-rank test). Multivariate analyses revealed SOAG [P=0.017, adjusted hazard ratio (aHR): 3.468, 95%CI: 1.246-9.654] and the postoperative IOP spike (IOP>25 mm Hg within 2wk post-surgery; P<0.001, aHR: 5.382, 95%CI: 2.113-13.707) as independent factors associated with surgical failure. CONCLUSION: The µLOT is a good treatment option for POAG eyes. However, the postoperative course should be carefully followed in cases with postoperative IOP spike.
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Purpose: To compare the accuracy of intraoperative aberrometry (IA) for predicting postoperative refraction between eyes with emmetropia and myopia targets. Patients and Methods: This retrospective analysis included patients with axial myopia (axial length ≥ 25.0 mm) who underwent uncomplicated phacoemulsification cataract surgery with IA to achieve emmetropia (plano to -0.5 D) or intentional myopia (-2.5 D to -5.0 D). Preoperative ocular biometry was performed in all eyes using an IOLMaster. Refractive prediction errors in IA were compared between eyes with emmetropia and myopia targets. Refractive prediction errors in IA for both groups were also compared with those predicted by intraocular lens power calculation formulas including the SRK/T, Holladay 1, Hoffer Q, Holladay 2, Haigis, and Barrett Universal II formulas. Results: Thirty-nine eyes of 39 patients with a target of emmetropia and 22 eyes of 22 patients with a target of intentional myopia were included in the final analysis. The mean numerical error was significantly different from zero (myopic trend) in myopia-targeted eyes (-0.37 ± 0.54 D, one-sample t-test, P = 0.004, 95% confidence interval: -0.61 to -0.14), while it was close to zero in emmetropia-targeted eyes. The mean absolute error was significantly smaller in emmetropia-targeted eyes (0.28 ± 0.27 D) than in myopia-targeted eyes (0.51 ± 0.41 D, P = 0.01). IA was revealed as the most accurate method for predicting postoperative refraction in eyes with emmetropia target, whereas Barrett Universal II formula was found to be the most accurate for eyes with myopia target. Conclusion: In patients with axial myopia, the performance of IA was altered when targeting intentional myopia compared with emmetropia. Myopic shift in the refractive outcome should be considered when IA is used to target myopia.
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BACKGROUND: This study aimed to identify the features of ocular biometry in patients with EYS-related retinitis pigmentosa using IOLMaster 700. METHODS: We retrospectively reviewed the medical records of patients with retinitis pigmentosa. Patients with records of the following were included: (1) ocular biometry measurements using the IOLMaster 700 and (2) genetic diagnostic tests. Axial length, keratometry, anterior chamber depth, aqueous depth, lens thickness, central corneal thickness (CCT), and corneal diameter (white to white) measurements were extracted. Based on keratometry measurements, (1) standard keratometric astigmatism, (2) posterior corneal astigmatism, and (3) total corneal astigmatism were obtained. Demographics and biometric parameters were compared between patients with EYS-related retinitis pigmentosa and other patients with retinitis pigmentosa. RESULTS: A total of 86 eyes of 44 patients (23 females and 21 males; mean age: 47.7 years) with retinitis pigmentosa were included. Of these, 18 were identified as having EYS variants. CCT was significantly thinner (P < 0.001) and the posterior corneal curvature at the steepest meridian was significantly smaller (P = 0.024) in patients with EYS-related retinitis pigmentosa than in other patients with retinitis pigmentosa. The magnitudes of all corneal astigmatism measurements was higher in patients with EYS-related RP, although these differences were not statistically significant. CONCLUSION: Patients with EYS-related retinitis pigmentosa had unique features in ocular biometry, such as thinner central corneal thickness and smaller posterior corneal curvature radius at the steepest meridian compared with other patients with retinitis pigmentosa. The findings suggest that patients with retinitis pigmentosa have different ocular dimension features among the different causative genes.
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Retinitis Pigmentosa , Tomografía de Coherencia Óptica , Longitud Axial del Ojo/anatomía & histología , Longitud Axial del Ojo/diagnóstico por imagen , Biometría , Proteínas del Ojo , Femenino , Humanos , Japón/epidemiología , Masculino , Persona de Mediana Edad , Retinitis Pigmentosa/diagnóstico , Retinitis Pigmentosa/genética , Estudios RetrospectivosRESUMEN
The retinal pigment epithelium (RPE) is essential for the survival and function of retinal photoreceptor cells. RPE dysfunction causes various retinal diseases including age-related macular degeneration (AMD). Clinical studies on ES/iPS cell-derived RPE transplantation for RPE dysfunction-triggered diseases are currently underway. Quantification of the diseased RPE area is important to evaluate disease progression or the therapeutic effect of RPE transplantation. However, there are no standard protocols. To address this issue, we developed a 2-step software that enables objective and efficient quantification of RPE-disease area changes by analyzing the early-phase hyperfluorescent area in fluorescein angiography (FA) images. We extracted the Abnormal region. This extraction was based on deep learning-based discrimination. We scored the binarized extracted area using an automated program. Our program's performance for the same eye from the serial image captures was within 3.1 ± 7.8% error. In progressive AMD, the trend was consistent with human assessment, even when FA images from two different visits were compared. This method was applicable to quantifying RPE-disease area changes over time, evaluating iPSC-RPE transplantation images, and a disease other than AMD. Our program may contribute to the assessment of the clinical course of RPE-disease areas in routine clinics and reduce the workload of researchers.
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Degeneración MacularRESUMEN
PURPOSE: To compare the accuracy of the intraocular lens (IOL) power calculation formulas for predicting the postoperative refraction in eyes with a target of emmetropia or intentional myopia. PATIENTS AND METHODS: This is a retrospective study conducted at Kobe City Eye Hospital, Kobe, Japan. Fifty eyes of 50 patients with axial myopia who underwent uncomplicated phacoemulsification and single-type IOL implantation for a target of emmetropia (plano to -0.5 D) or intentional myopia (-2.0 D to -3.0 D) were selected. Preoperative ocular biometry was performed using IOLMaster700 in all eyes. Refractive prediction errors of 6 IOL formulas integrated into IOLMaster700 were compared between eyes with a target of emmetropia and intentional myopia. RESULTS: The mean numerical errors of SRK/T (Sanders, Retzlaff, and Kraft/theoretical), Holladay 1, Hoffer Q, and Holladay 2 significantly differed between the two groups (p < 0.001, p = 0.008, 0.02, and 0.007, respectively). The values for mean numerical errors in eyes with a target of intentional myopia were smaller, showing relatively myopic outcome, as compared with those in eyes with a target of emmetropia. In eyes with a target of emmetropia, the mean numerical errors of Holladay 1 (p < 0.001, 95% confidence interval [CI]: 0.32 to 0.63), Hoffer Q (p = 0.001, 95% CI: 0.12 to 0.42), and Barrett Universal II (p = 0.007, 95% CI: 0.06 to 0.35) were significantly different from zero (hyperopic trend). Furthermore, in eyes with a target of intentional myopia, the mean numerical error of SRK/T (p = 0.001, 95% CI: -0.61 to -0.17) and Holladay 2 (p = 0.023, 95% CI: -0.43 to -0.04) were significantly different from zero (myopic trend). CONCLUSION: In patients with axial myopia, some IOL formulas may show a myopic trend in the refractive outcome when targeting intentional myopia as compared to emmetropia.