Phacoemulsification, pinhole pupilloplasty, and pre-Descemet抯 endothelial keratoplasty for keratoconus with Fuchs� endothelial dystrophy

The comorbidity of keratoconus with Fuchs� endothelial dystrophy with cataract is a rare clinical combination. We present an amalgamation of surgical techniques to manage the above clinical conditions and its complications in single setting. The modified triple procedure, namely, the phacoemulsification, pinhole pupilloplasty, and pre-Descemet抯 endothelial keratoplasty (PDEK) in the order of description is followed in single sitting. Lens removal by phacoemulsification, correction of irregular astigmatism by pinhole pupilloplasty (pinhole optics), and exchanging the endothelial layer for PDEK forms the main segments of the triple procedure. This combination of techniques may decrease the risk of multiple surgeries and its related complications. Moreover, it will allow the patient for faster visual rehabilitation by improving the uncorrected visual acuity and visual quality.

Demographic profile and clinical characteristics of Fuchs’ endothelial corneal dystrophy in a tertiary eye care center

Purpose: This study was performed to determine the demographic profile and clinical characteristics in patients with Fuchs’ endothelial corneal dystrophy (FECD) reporting to a tertiary eye care center in India. It is a retrospective, single?center, observational study. Methods: The study included 280 patients (559 eyes) diagnosed with FECD presenting between January 2013 and December 2020. The data was collected from the electronic medical record system of the institute. Patient data included demographic features, clinical characteristics, investigations, and surgical interventions. Results: The mean age of the patients was 62 years. Late?onset FECD (95.7%) was more common than early?onset FECD (4.3%). Male: female ratio for late?onset FECD and early?onset FECD was 1:1.65 and 3:1, respectively. More than one?third of the patients had associated systemic history. Preexisting ocular diseases were seen in 5.9% of eyes. Blurring of vision was seen in 383 eyes (68.5%), 13 eyes (2.1%) had glare, and 163 eyes (29.2%) were asymptomatic. A total of 113 surgical interventions were done in 108 eyes (including repeat transplants). Only cataract surgery was done in 40 (7.2%) eyes, whereas penetrating keratoplasty, Descemet stripping endothelial keratoplasty, and Descemet membrane endothelial keratoplasty without or with cataract surgery (sequential or triple procedure) were done in 12 (2.1%), 47 (8.4%), and 14 (2.5%) eyes, respectively. Conclusion: Patients with FECD present mostly during the sixth decade. Posterior lamellar keratoplasty is the most common transplant procedure being performed on FECD patients

Inhibidores de la Rho quinasa y su rol en la disfunción endotelial corneal / Rho Kinase Inhibitors and their Role in Corneal Endothelial Dysfunction

El endotelio corneal es su capa más interna y, a pesar de ser una monocapa de células, es capaz de preservar la transparencia del tejido con dos funciones fundamentales: de barrera y de bomba endotelial sodio-potasio (Na-K). Las células endoteliales tienen muy poca capacidad de regeneración, por lo que cualquier lesión endotelial es compensada por la expansión y migración de las células residuales adyacentes. La disfunción endotelial corneal se caracteriza por un edema de la córnea que puede llevar hasta el transplante de este tejido. Nuevas terapias farmacológicas con inhibidores de Rho-Kinasa y terapias basadas en ingeniería tisular se han propuesto recientemente. Se realizó una búsqueda automatizada sobre los principales avances en estas terapias utilizando la plataforma Infomed, específicamente la Biblioteca Virtual de Salud. La información se resumió en el informe final. Concluimos que existe un progreso significativo en el entendimiento de la patogénesis, y en el desarrollo de los nuevos tratamientos(AU)

The corneal endothelium is its innermost layer and, despite being a monolayer of cells, is able to preserve tissue transparency with two fundamental functions: barrier and endothelial sodium-potassium (Na-K) pump. Endothelial cells have very little regenerative capacity, so any endothelial injury is compensated by the expansion and migration of adjacent residual cells. Corneal endothelial dysfunction is characterized by corneal edema that can lead to corneal tissue transplantation. New pharmacologic therapies with Rho kinase inhibitors and tissue engineering-based therapies have recently been proposed. An automated search on the main advances in these therapies was performed using the Infomed platform, specifically the Virtual Health Library. The information was summarized in the final report. We conclude that there is significant progress in the understanding of pathogenesis, and in the development of new treatments(AU)

Clinical profile and demographic distribution of Fuchs’ endothelial dystrophy: An electronic medical record–driven big data analytics from an eye care network in India

Purpose: To describe the demographics and clinical profile of Fuchs’ endothelial corneal dystrophy (FECD) in patients presenting to a multi?tiered ophthalmology hospital network in India. Methods: This cross?sectional hospital?based study included 3,082,727 new patients presenting between August 2010 and December 2021. Patients with a clinical diagnosis of FECD in at least one eye were included as cases. The data were collected using an electronic medical record system. Results: Overall, 2570 (0.08%) patients were diagnosed with FECD. The majority of the patients were female (65.53%) and were predominantly adults (99.92%). The most common age group at presentation was during the seventh decade of life with 867 patients (33.74%). The overall prevalence was higher in patients from a higher socioeconomic status (0.1%) presenting from the urban geography (0.09%) and in retired individuals (0.4%). About half of the 5,140 eyes had mild or no visual impairment (< 20/70) in 2643 eyes (51.42%) followed by moderate visual impairment (>20/70 to 20/200) in 708 eyes (13.77%). The average logMAR was 0.61 ± 0.81 at presentation. The most documented corneal signs were guttae (76.63%), corneal scar (23%) and stromal edema (21.73%). The most associated ocular comorbidity was cataract (47.32%) followed by glaucoma (5.39%). More than a tenth of the affected eyes required a surgical intervention of endothelial keratoplasty (15.58%). Conclusion: FECD more commonly affects females presenting during the seventh decade of life. Majority of the eyes had mild or no visual impairment and endothelial keratoplasty is warranted in a tenth of the affected eyes

Comportamiento de los pacientes con distrofia corneal endotelial de Fuchs en la provincia Camagüey

Introducción: La distrofia corneal endotelial de Fuchs se trata de un trastorno degenerativo específico, bilateral y progresivo del endotelio corneal, es la más frecuente pero no siempre es diagnosticada en sus etapas iniciales en las consultas de oftalmología general. Objetivo: Describir el comportamiento clínico de pacientes con distrofia corneal endotelial de Fuchs en la provincia Camagüey. Métodos: Se realizó un estudio observacional, descriptivo, transversal en el Centro Oftalmológico del Hospital Universitario Manuel Ascunce Domenech en la provincia Camagüey desde noviembre 2019 hasta junio 2021. El universo de estudio estuvo constituido por todos los pacientes que asistieron durante el periodo de estudio y la muestra la conformaron 19 pacientes (38 ojos) quienes cumplieron con los criterios de inclusión y exclusión. Las variables estudiadas fueron edad, sexo, color de la piel, agudeza visual con corrección, asociación con glaucoma, paquimetría, biomicroscopía del segmento anterior, microscopía endotelial, microscopía confocal, estadio de la enfermedad y tipo de tratamiento aplicado. Resultados: Predominaron los pacientes entre 40 y 59 años de edad, el sexo femenino y color blanco de la piel. Sobresalió la visión útil, los valores de paquimetría altos y asociados al glaucoma. Se constató la presencia de guttas, edema corneal, bajo conteo celular con polimorfismo y polimegatismo. El estadio 2 estuvo en 47,4 % y el tratamiento médico se aplicó en el 97,4 %. Conclusiones: La distrofia aparece con más frecuencia después de los 40 años de edad, en sexo femenino y color blanco de piel. Predominó la visión útil, valores altos de paquimetrías y asociación con glaucoma. En la biomicroscopía del segmento anterior predominaron las guttas y el edema estromal y la microscopía endotelial y confocal se caracterizaron en su mayoría por el bajo conteo celular, las guttas, polimorfismo y polimegatismo. Prevaleció el estadio 2 y el tratamiento médico.

Introduction: Fuchs endothelial corneal dystrophy is a specific, bilateral and progressive degenerative disorder of the corneal endothelium, it is the most frequent but it is not always diagnosed in its initial stages in general ophthalmology consultations. Objective: To describe the clinical behavior of patients with Fuchs endothelial corneal dystrophy in Camagüey province. Methods: A cross-sectional descriptive observational study was carried out at the Ophthalmological Center of the Manuel Ascunce Domenech University Hospital in Camagüey in the period from November 2019 to June 2021. The study universe consisted of all the patients who attended during the study period and the sample was made up of 19 patients (38 eyes) who met the inclusion and exclusion criteria. The variables studied were age, sex, skin color, corrected visual acuity, association with glaucoma, pachymetry, anterior segment biomicroscopy, endothelial microscopy, confocal microscopy, disease stage, and type of treatment applied. Results: Patients between 40 and 59 years of age, female sex, and white skin color predominated. Useful vision stood out, high pachymetry values and associated with glaucoma, the presence of guttas, corneal edema, low cell count with polymorphism, and polymegatism was confirmed. Stage 2 was 47.4% and medical treatment was applied in 97.4%. Conclusions: Dystrophy appears more frequently after 40 years of age, in females and white skin persons. Useful vision, high pachymetry values, and association with glaucoma prevailed. In the biomicroscopy of the anterior segment, guttas and stromal edema predominated, and endothelial and confocal microscopy were mostly characterized by low cell count, guttas, polymorphism, and polymegatism. Stage 2 and medical treatment prevailed.

Aplanamento corneano após DMEK associado a facoemulsificação em paciente com distrofia endotelial de Fuchs, ceratocone e catarata / Corneal flattening after simultaneous DMEK and phacoemulsification in patient with Fuchs endothelial dystrophy, keratoconus and cataract combined

Resumo Descrição de relato de caso de paciente com ceratocone (KC), Distrofia Endotelial de Fuchs (DEF) e catarata concomitantes com descompensação corneana submetido a Ceratoplastia Lamelar Posterior pela técnica Descemet's Membrane Endothelial Keratoplasty (DMEK) associado a facoemulsificação com implante de lente intraocular (LIO). Observou-se aplanamento corneano significativo no pós-operatório e acuidade visual final sem correção de 20/25 . Destaca-se a possibilidade do DMEK como alternativa à Ceratoplastia Penetrante (Penetrating Keratoplasty - PK) em casos de DEF e KC associados. Aplanamento corneano pode ocorrer neste grupo de pacientes, o que pode resultar em imprevisibilidade refracional no cálculo do poder da LIO ao se optar por facectomia combinada. Neste relato, apesar da modificação da curvatura corneana após cirurgia combinada de DMEK com facectomia, a acuidade visual final sem correção foi satisfatória, demonstrando a possibilidade de sucesso desta abordagem na coexistência de DEF, KC e Catarata. Entretanto, a possibilidade de mudança significativa na curvatura corneana deve ser considerada em pacientes com KC, edema de córnea secundário a DEF e catarata, na decisão de cirurgia simultânea ou em dois tempos.

Abstract This is a case report of a patient with concomitant Keratoconus (KC), Fuchs Endothelial Dystrophy (FED) and cataract with corneal decompensation submitted to Posterior Lamellar Corneal surgery (Descemet's Membrane Endothelial Keratoplasty - DMEK) associated with phacoemulsification with intraocular lens (IOL) implantation. Corneal flattening and uncorrected visual acuity of 20/25 was observed in the postoperative period. We reasure the viability of DMEK as an alternative to Penetrating Keratoplasty (PK) in cases of associated DEF and KC associated. Changes in corneal curvature may occur in this group of patients and lead to the possibility of refractive unpredictability in IOL calculation when performing a combined cataract surgery. Despite unexpected corneal flattening, satisfactory final visual acuity was achieved, demonstrating the possibility of success of this approach in the coexistence of the three conditions. Nonetheless, the possibility of corneal curvature changes should be considered in patients with KC and corneal decompensation due to FED in decision making, regarding simultaneous or sequential surgical approach.

Corneal endothelium in unilateral Fuchs heterochromic iridocyclitis

Purpose: To assess the corneal endothelium in patients with Fuchs heterochromic iridocyclitis (FHI) and compare it with the normal fellow eye. Methods: Retrospective, observational, cross-sectional study of 31 patients seen between Jan 2016 to Dec 2018, with clinical diagnosis of Fuchs heterochromic iridocyclitis, was performed. Specular microscopic examination was documented in both eyes. The affected eyes formed the study group and the fellow healthy eyes served as controls. Results: The mean age of the patients was 29.9 ± 8.2 years. The endothelial cell density (P = 0.0001) was significantly lower, whereas average cell size (P = 0.0001), coefficient of variation (P = 0.004), and maximum cell area (P = 0.01) were significantly higher in the affected eye compared to the control eye. In three patients, the affected eye showed guttae, while the healthy fellow eye revealed a normal specular mosaic. Conclusion: Specular microscopic analysis shows endothelial alterations in the affected eyes in FHI.

Comparative study of urine sediment elements with fully automated analyzer and the bright field microscope method using Sternheimer Malbin dye / Эрүүл мэндийн лаборатори

Introduction@#The traditional microscopic method is to visually count the elements in the urine, but it is difficult to distinguish between the cells because they are not stained. Sternheimer Malbin staining, on the other hand, contains a variety of dyes that help to distinguish elements in urine sediment, improve the differentiation between cell nuclei and cytoplasm, provide more information about cell shape and image, and make it easier to differentiate kidney disease. @*Objective@#To study the results of the reading of a fully automatic urine sediment analyzer of compared with the Sternheimer Malbin stained bright field microscope method.@*Research materials and methods@#In this study included 150 people who served the MJTH of the MNUMS received permission to participate in the research. The urine sample collected in accordance with the standard operating instructions was counted by a fully automated analyzer and stained with Sternheimer Malbin dye and counted red cells (RBC), white blood cells (WBC), epithelial cells (EC), and renal epithelium (RTEC) under a microscope using a Fuchs-Rosenthal chamber.@*Results@#23.3% (n=35) of the respondents were male, 76.6% (n=115) were female, and the average age was 44.3±11.6. There 16.6% (25)/9.3% (14) of the RBCs were counted in excess of the reference volume when analyzed under an microscope stained with an automated urine sediment analyzer and Sternheimer-Malbin dye. For each WBC method, 45.4% (68)/41 (61)% and EC 24.7% (37)/23.3% (35) were counted above the reference volume. 90% (135)/32% (48) of the total samples were counted in excess of the RTEC reference volume. Comparing the performance of the automatic urine sediment analyzer with the light microscope method, the sensitivity and specificity were RBC-99.8%/99.1%, WBC-99.3%/99.6%, EC-99.7%/99.2, and RTEC-99.1%/99.2%. False-positive and false-negative results were rated for each RBC-99.9%/99.1%, WBC-99.3%/99.6%, EC 99.8%/99.2%, and RTEC-99.7%/99.9%, respectively. The positive likelihood ratio was RBC, WBC, RTEC 1.0, or the test was useless, while the negative likelihood ratio was RBC was very different, WBC was slightly different, EC was very different, and RTEC was very different. Positive and negative predictive value indicators RBC-99.3%/99.4%, WBC-99.4%/99.4%, EC-99.4%/99.5, RTEC-99.2%/99.1%, optimality for RBC, WBC, EC 99.4%, RTEC -99.1%.@*Conclusion@#</br> 1. The results of an automated urine sediment analyzer and a bright field microscope stained by Sternheimer Malbin were similar for red blood cells, white blood cells, and epithelial cells, but different for renal tubular epithelial cells. </br> 2. The resuls UF-5000 analyzer and bright field microscope analysis using Sternheimer Malbin dye were comparable.

Research progress of descemetorhexis without endothelial keratoplasty promoting corneal endothelial regeneration / 国际眼科杂志(Guoji Yanke Zazhi)

@#Endothelial dysfunctionis traditionally considered irreversible, and endothelial keratoplasty(EK)is almost the only treatment available. Recently, however, a surgery called descemetorhexis without endothelial keratoplasty(DWEK)can regenerate the central corneal endothelial cells in patients with Fuchs endothelial corneal dystrophy(FECD), and local Rho-associated kinase inhibitor can enhance its efficacy.

Astigmatismo y aberraciones corneales en ojos con Iridociclitis Heterocrómica de Fuchs / Corneal astigmatism and aberrations in patients with Fuchs heterochromic iridocyclitis

Introducción: La Iridociclitis Heterocrómica de Fuchs (IHF) es la causa del 2 a 7% de las uveítis anteriores, el astigmatismo corneal como condición de la IHF y como posible causa de la disminución de la visión, ha sido una entidad muy poco estudiada hasta el momento, tiene una gran importancia debido a que por la anisometropía que podría generar, puede llevar a alteraciones de la agudeza visual. Objetivo: Evaluar la existencia de astigmatismo corneal y/o aberraciones corneales inherentes a los ojos con Iridociclitis Heterocrómica de Fuchs. Diseño del estudio: Estudio transversal, analítico, de fuentes primarias. Método: Se realizó muestreo por conveniencia, en el cual se incluyeron 44 ojos de 22 pacientes con diagnóstico de IHF unilateral o bilateral. Para cada caso, se realizó tomografía corneal (Pentacam® AXL - OCULUS) y aberrometria (OPD-Scan III® - NIDEK) y se compararon ojos enfermos con los ojos sanos. Resultados: Se encontró una mediana de 1.35 (RIC: 0.50 ­ 1.70) para el astigmatismo de la cara anterior de los ojos enfermos .vs. 0.8 (RIC: 0.7 ­ 1.1) para el astigmatismo de la cara anterior de los ojos sanos (p= 0.135). Se identificó un astigmatismo en cara anterior >1.00 D en el 61.5% de los ojos enfermos .vs. 27.8% de los ojos sanos (p= 0.027) con un RR de 2.15. No se encontró una diferencia estadísticamente significativa entre los ojos enfermos y los ojos sanos para las aberraciones analizadas. Conclusión: El presente estudio no demuestra la existencia de astigmatismo o aberraciones inherentes a la IHF, pero evidencia una tendencia de mayor astigmatismo en los ojos con IHF, que posiblemente podría lograr significancia estadística con una muestra mayor.

Background: Fuchs heterochromic iridocyclitis (FHI) represents 2 to 7% of cases of anterior uveitis. To date, few studies have addressed corneal astigmatism as an inherent condition and as possible cause of decreased visual acuity in FHI patients. This may be relevant since the generated anisometropia may lead to alterations in visual acuity. Objective: To evaluate the presence of corneal astigmatism and/or corneal aberrations in patients with FHI. Study design: Analytic cross sectional study. Method: A convenient sampling was performed. The sample included 44 eyes of 22 patients with unilateral or bilateral FHI. All participants were studied by corneal tomography (Pentacam® AXL ­ OCULUS) and aberrometry (OPDScan III® - NIDEK); measurements of diseased and healthy eyes were compared. Results: Twenty-two patients were enrolled in this study. The mean age at diagnosis was 46.9 +/- 12 years, and 40.9% were men. FHI eyes exhibited a median astigmatism of the anterior surface of 1.35D (IQR: 0.50 ­ 1.70), compared to 0.8D (IQR: 0.7 ­ 1.1) observed in healthy eyes (p= 0.135). Anterior surface astigmatism >1.00 diopters (D) was found in 61.5% of diseased eyes compared to 27.8% of healthy eyes, with a risk ratio of 2.15. Regarding corneal aberrations, no statistically significant differences were identified between diseased and healthy eyes for the analyzed aberrations. Conclusion: While our current data does not show statistically signifi cant differences between groups that support that corneal astigmatism or aberrations are inherent to FHI-aff ected eyes, our study does show a trend that suggests increased presence of clinically relevant astigmatism in eyes with FHI. Statistical significance may be achieved with a larger sample size.

Descemet membrane endothelial keratoplasty in multifocal pseudophakic eyes / Ceratoplastia endotelial da membrana de Descemet em olhos pseudofácicos multifocais

ABSTRACT Purpose: This report describes the use of Descemet membrane endothelial keratoplasty for the management of endothelial decompensation after multifocal intraocular lens implantation. Methods: In this retrospective study, we reviewed and assessed the surgical outcomes of 9 patients (9 eyes) who underwent Descemet membrane endothelial keratoplasty after multifocal intraocular lens implantation. Results: Corneal edema occurred due to Fuchs endothelial corneal dystrophy (n=3), pseudophakic bullous keratopathy (n=3), Descemet's membrane detachment (n=2), and toxic anterior segment syndrome (n=1). The Descemet membrane endothelial keratoplasty surgeries were uneventful in all eyes, but rebubbling procedures were necessary in 2 eyes. One month after the surgery, all the corneas were clear. After 6 months, excluding 1 eye with amblyopia, the mean distance corrected visual acuity was 0.10 logMAR, with all eyes achieving 0.18 logMAR or better. Conclusions: This is the first report of Descemet membrane endothelial keratoplasty after multifocal intraocular lens implantation, and it suggests that good results can be achieved without multifocal intraocular lens exchange.

RESUMO Objetivo: Descrever o uso da ceratoplastia endotelial da membrana de Descemet para manejar descompensação endotelial após implante de lente intraocular multifocal. Métodos: Neste estudo retrospectivo, foram revisados e avaliados os resultados cirúrgicos de 9 olhos de 9 pacientes que foram submetidos a ceratoplastia endotelial da membrana de Descemet para manejar descompensação endotelial após implante de lente intraocular multifocal. Resultados: Descompensação endotelial ocorreu por distrofia endotelial de Fuchs (n=3), ceratopatia bolhosa do pseudofácico (n=3), descolamento da membrana de Descemet (n=2) e síndrome tóxica do segmento anterior (n=1). No ato per operatório de todos os olhos não houve intercorrência, com injeção de ar sendo necessário em dois olhos no pós-operatório por descolamento parcial do enxerto. Um mês após a cirurgia, todas as córneas estavam claras. Após seis meses, excluindo um olho com ambliopia, a acuidade visual média corrigida para longe foi de 0,10 logMAR, com todos os olhos atingindo 0,18 logMAR ou melhor. Conclusões: Este é o primeiro relato de ceratoplastia endotelial da membrana de Descemet após implante de lente intraocular multifocal, sugerindo que bons resultados podem ser alcançados sem a troca da lente intraocular multifocal.

Outcomes of phacoemulsification in patients with Fuchs' heterochromic iridocyclitis / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To evaluate the clinical outcomes of phacoemulsification in patients with Fuchs' heterochromic iridocyclitis(FHIC). <p>METHODS: In this noncomparative case series,18 eyes of 18 patients clinically diagnosed with FHIC and cataract were studied. All of them received phacoemulsification combined with intraocular lens implantation.Preoperative and postoperative visual acuity, intraocular pressure, corneal endothelial cells density were measured. Intraoperative and postoperative complications were evaluated. <p>RESULTS: The mean follow-up period was 9.8±3.3mo. The only intraoperative complication was peripheral iris bleeding(17%). Compared with preoperative, the best corrected visual acuity improved 6mo postoperatively(0.98±0.85 <i>vs</i> 0.08±0.14, <i>P</i><0.001). There was no significant difference in intraocular pressure between preoperative and postoperative(15.11±2.63mmHg <i>vs</i> 14.94±2.49mmHg, <i>P</i>>0.05).The mean endothelial cells counting loss rate postoperatively was 10%±6%. Fibrin formation in the anterior chamber occurred in 2 eyes postoperatively. During follow-up, 1 eye developed anterior capsule contraction, and 4 eyes developed posterior capsule opacification. <p>CONCLUSION: Phacoemulsification is a safe procedure with good visual outcomes in patients with FHIC and cataract.

Cataract surgery in patients with Fuchs endothelial corneal dystrophy and high myopia / 上海交通大学学报（医学版）

Objective · To evaluate the effect of phacoemulsification combining with intraocular lens (IOL) implantation in cataract patients with Fuchs endothelial corneal dystrophy (FECD) and high myopia. Methods · Five patients (9 eyes) diagnosed with FECD and high myopia were collected retrospectively at Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine from January 2015 to February 2016. All the 9 eyes underwent phacoemulsification combining with IOL implantation. Visual acuity, intraocular pressure, corneal endothelial cell density (CD) and central corneal thickness (CCT) were evaluated. Paired t-test was performed to evaluate the differences. Results · 88.89% (8/9) of the vision of the patients was improved, the best corrected visual acuity improved at least 2 lines. The intraocular pressure had no statistically significant difference before and after the operation (t=-1.066, P=0.318). The preoperative and postoperative CDs were (2184.25±196.44) cells/mm2 and (1910.0±83.41) cells/mm2 (t=2.876,P=0.064). The preoperative and postoperative CCTs were (546.83±33.74) μm and (566.67±24.66) μm (t=1.971, P=0.106). The loss rate for endothelial cells was (12.10±7.84)%. Only one patient was observed corneal edema and the CD could not be determined, so he underwent penetrating keratoplasty.Conclusion · Phacoemulsification combining with IOL implantation is safe and effective for patients with FECD and high myopia. Preoperative evaluation of corneal endothelium is necessary. Some measures should be taken into consideration to reduce the loss of endothelial cells during in surgery.

Cataract surgery in patients with Fuchs endothelial corneal dystrophy and high myopia / 上海交通大学学报（医学版）

Objective · To evaluate the effect of phacoemulsification combining with intraocular lens (IOL) implantation in cataract patients with Fuchs endothelial corneal dystrophy (FECD) and high myopia. Methods · Five patients (9 eyes) diagnosed with FECD and high myopia were collected retrospectively at Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine from January 2015 to February 2016. All the 9 eyes underwent phacoemulsification combining with IOL implantation. Visual acuity, intraocular pressure, corneal endothelial cell density (CD) and central corneal thickness (CCT) were evaluated. Paired t-test was performed to evaluate the differences. Results · 88.89% (8/9) of the vision of the patients was improved, the best corrected visual acuity improved at least 2 lines. The intraocular pressure had no statistically significant difference before and after the operation (t=-1.066, P=0.318). The preoperative and postoperative CDs were (2184.25±196.44) cells/mm2 and (1910.0±83.41) cells/mm2 (t=2.876,P=0.064). The preoperative and postoperative CCTs were (546.83±33.74) μm and (566.67±24.66) μm (t=1.971, P=0.106). The loss rate for endothelial cells was (12.10±7.84)%. Only one patient was observed corneal edema and the CD could not be determined, so he underwent penetrating keratoplasty.Conclusion · Phacoemulsification combining with IOL implantation is safe and effective for patients with FECD and high myopia. Preoperative evaluation of corneal endothelium is necessary. Some measures should be taken into consideration to reduce the loss of endothelial cells during in surgery.

Clinical Characteristics and Prognosis of Fuchs Dystrophy According to COL8A2 Gene Mutation Status

PURPOSE: To compare the clinical characteristics and prognosis of Fuchs dystrophy patients according to COL8A2 gene mutation status. METHODS: Eighty-one patients (162 eyes) initially diagnosed with Fuchs dystrophy from 1996 to 2015 were divided into two groups according to COL8A2 gene mutation status. Retrospective analysis was performed comparing gender, age at diagnosis, presence of family history, diabetes mellitus, symptoms of blurred vision in the morning, changes in central corneal thickness and endothelial cell density with time, need for corneal transplantation, and pre-operative corneal status in the two groups. RESULTS: Of the 81 patients, 12 were shown to harbor a COL8A2 gene mutation. Individuals with mutation were significantly associated with presence of family history, diabetes mellitus, and blurred vision in the morning (p = 0.021, p = 0.024, p = 0.044, respectively). They also had significantly thicker central cornea and lower endothelial cell density at the time of diagnosis (p = 0.020, p = 0.005, respectively). The differences in central corneal thickness and endothelial cell density between the two eyes in one patient were significantly smaller in patients with gene mutation (p = 0.043, p = 0.022, respectively). Over a 5-year follow-up period, 60.0% of eyes in patients with gene mutation and 19.2% of eyes in patients without gene mutation underwent corneal transplantation, a significant difference between the two groups (p = 0.014). CONCLUSIONS: By testing for COL8A2 gene mutation, early binocular disease progression and the possible need for corneal transplantation in the future can be predicted among patients diagnosed with Fuchs dystrophy.

Clinical spectrum, diagnostic criteria, and polymerase chain reaction of aqueous humor in viral and toxoplasma detection in Fuchs’ uveitis syndrome.

Aim: The aim of this study is to describe the clinical features and diagnostic criteria of Fuchs’ uveitis (FU) and to determine whether it has an association with virus and toxoplasma in the aqueous humor during cataract surgery. Setting and Design: This is a prospective, case–control study. Materials and Methods: Patients with FU (n = 25), anterior uveitis (n = 15), and no uveitis (normal) (n = 50) were included based on predefined inclusion and exclusion criteria for all three groups. Polymerase chain reaction (PCR) of aqueous humor and serum for rubella, herpes simplex virus (HSV), cytomegalovirus (CMV), varicella‑zoster virus (VZV), and toxoplasma was done using conventional uniplex PCR. Statistical Analysis: It was done using SPSS software using Chi‑square test for categorical variables, and P < 0.05 was considered statistically significant. Results: Ninety patients were enrolled in the study in three groups, comparable for age, gender, and laterality of ocular involvement. All patients had diffuse keratic precipitates in FU group (P = 0001) with none having posterior synechiae (P = 0.046) which was statistically significant when compared to anterior uveitis patients. Iris nodules were noted in one case in both groups. Serum and aqueous PCR was negative for detection of VZV, CMV, toxoplasma, and rubella in all groups. PCR for HSV was positive in one patient in “normal” group but was not statistically significant. Conclusion: Our study shows that diagnosis of FU is mainly clinical. There appears to be no role of aqueous humor testing for viruses by PCR to aid in etiological diagnosis.

Mechanism of DJ -1 against oxidative stress and its roles in the pathogenesis of age -related macular degeneration and Fuchs endothelial corneal dystrophy / 国际眼科杂志(Guoji Yanke Zazhi)

?DJ-1 has been reported to act as aredox-activated chaperone and sensor of oxidative stress participated in a variety of activities in cellular, playing an important role in resisting oxidative stress, regulating signaling pathways and gene transcription, and maintaining mitochondria dynamic balance. DJ -1 is closely related to the occurrence and development of various diseases. Recently, the effect of DJ-1 in eye diseases has drawn more attention, and researchers have found its significant role of resistance to oxidative stress in the pathogenesis of Fuchs endothelial corneal dystrophy ( FECD) and age-related macular degeneration ( AMD ) .This review will state the mechanism of DJ-1 against oxidative stress and its role in the development of eye diseases.

Revisión con aguja tras implante de válvula de Ahmed en la ciclitis heterocrómica de Fuchs / Needle revision after Ahmed valve implantation in the management of Fuch´s heterochromic cyclitis

La ciclitis heterocrómica de Fuchs es una uveítis crónica que puede ser asintomática por años o expresar solo la heterocromía antes que aparezca cualquier otro signo. El glaucoma se considera una de las complicaciones más difíciles de tratar, y requiere cirugía en múltiples ocasiones. Los dispositivos de drenaje están siendo cada vez más utilizados como alternativa de tratamiento quirúrgico en estos casos. Asiste a la consulta médica una paciente de 36 años de edad, con antecedentes de uveítis crónica unilateral del ojo izquierdo asociado a catarata y glaucoma descompensado, a pesar del tratamiento médico. Se presenta con 50 VAR de visión y presión intraocular de 32 mmHg. Se realizó cirugía combinada: facoemulsificación e implante de válvula Ahmed modelo S2 con mitomicina C (0,2 mg/mL) durante cinco minutos. Se diagnostica ampolla de filtración encapsulada en la octava semana. Se realiza revisión con aguja y subconjuntival de 1 mg de bevacizumab (avastin) subtenoniano en área de la filtrante. La inyección se repite días alternos hasta completar tres dosis según protocolo institucional. Se logran cifras de presión intraocular de 17 mmHg y agudeza visual mejor corregida de 95 VAR a los 18 meses posoperatorios(AU)

Fuch´s heterochromic cyclitis is chronic uveitis that may be asymptomatic for years or may express heretochromia just before the onset of any other sign. Glaucoma is considered one of the most difficult complications to be treated and requires multiple surgeries. Drainage devices are increasingly used as a surgical treatment option in these cases. Here is the case of a 36 years-old woman with a history of unilateral chronic uveitis in her left eye associated to cataract and decompensate glaucoma despite the medical therapy. She presented with 50 VAR visual acuity and 32 mmHg intraocular pressure. She underwent combined surgery based on phacoemulsification and S2 model Ahmed valve implantation with mitomycin C (0,2 mg/mL) for 5 minutes. The diagnosis was encapsulated filtering bleb at the 8th week of operation. Subconjuctival needle revision with one mg of subtenial bevacizumab (avastin) in the bleb area was performed. Injection was administered every other day till completion of three doses according to the institutional protocol. Eighteen months after the surgery, the intraocular pressure was 17nnHg and the best corrected visual acuity reached 95 VAR best corrected visual acuity(AU)

Corneal Endothelial Cell Loss after Tube Shunt Surgery in Fuch's Heterochromic Iridocyclitis

PURPOSE: To report a case of corneal failure after implantation of the Ahmed glaucoma valve occurring in a patient diagnosed with Fuchs' heterochromic iridocyclitis. CASE SUMMARY: A 53-year-old male who complained of ocular pain and suddenly decreased visual acuity in his right eye visited our clinic. His visual acuity was 0.15 and intraocular pressure (IOP) was 55 mm Hg. The slit-lamp examination revealed edematous cornea, fine round or stellate keratic precipitates connected with fine filaments on the endothelium and depigmentation of the iris. The corneal endothelial cell density was 2,958 cells/mm2. There was no specific finding in his left eye. The IOP did not improve with medical treatment, therefore, an Ahmed glaucoma valve was implanted in his right eye. At every follow-up exam the tube was well positioned and the IOP was maintained between 8 and 13 mm Hg. Eight months postoperatively, the patient complained of decreased visual acuity and the cell density was decreased to 1,408 cells/mm2. Posterior subcapsular cataract opacity was observed as well as progression of depigmentation and distortion of the iris. Seventeen months after the surgery, the cell density was 700 cells/mm2. On follow-up examination, his visual acuity was decreased to FC10 cm with the cataract progressing, therefore cataract surgery was performed. One month postoperatively, his vision improved to 0.1. However, the visual acuity deteriorated due to progression of the corneal edema and penetrating keratoplasty was performed. CONCLUSIONS: Aggravation of the corneal complication after Ahmed glaucoma valve implantation should be considered in patients with Fuchs' heterochromic iridocyclitis-induced glaucoma.

Características clínicas de la distrofia corneal endotelial de Fuchs / Clinical characteristics of Fuchs' endothelial corneal dystrophy in families under study

OBJETIVOS: caracterizar clínicamente la distrofia corneal endotelial de Fuchs en las familias estudiadas. MÉTODOS: se realizó un estudio observacional, descriptivo y transversal en familias con distrofia de Fuchs, en el Instituto Cubano de Oftalmología "Ramón Pando Ferrer", del año 2008 al 2011. Se operacionalizaron variables como: estadio clínico de la enfermedad, amplitud de cámara anterior y ángulo iridocorneal, presión intraocular, longitud axial, paquimetría, densidad celular endotelial, pleomorfismo y polimegatismo. Se resumieron las variables utilizando frecuencias absolutas y relativas porcentuales. Para la relación entre densidad celular y paquimetría se hizo un análisis de correlación bivariado no paramétrico. RESULTADOS: los pacientes enfermos se encontraban en su mayoría en el estadio I y II, con 50 y 60 % para los recién diagnosticados y los ya conocidos respectivamente. La amplitud de la cámara anterior fue normal en el 51,9 %; el ángulo iridocorneal estrecho en el 55,6 %, la presión intraocular normal en el 66,7 % y una hipermetropía axial en el 55,6 % de los casos con distrofia corneal. No se encontró asociación significativa entre paquimetría y densidad celular, pero sí pleomorfismo y polimegatismo en el 100 % de los enfermos y en más del 50 % de los descendientes directos. CONCLUSIONES: las variables analizadas demuestran ser importantes a la hora de caracterizar la enfermedad, además de señalar daños endoteliales en descendientes no enfermos, aspecto a tener en cuenta para su seguimiento.

OBJECTIVE: to describe the clinical characteristics of Fuchs' endothelial corneal dystrophy in the families under study. METHODS: observational, descriptive and cross-sectional study of families with Fuchs' corneal dystrophy diagnosed at "Ramón Pando Ferrer" Cuban Institute of Ophthalmology from 2008 to 2011. The study variables were clinical stage of the disease, depth of anterior chamber and iridocorneal angle, intraocular pressure, axial length, corneal thickness, endothelial cell density, pleomorphism and polymegatism. The variables were summarized using percentage absolute and relative frequencies. For assessing the ratio of the cell density and pachymetry, a nonparametric bivariate correlation analysis was made. RESULTS: the patients with Fuchs' endothelial corneal dystrophy were mostly in the clinical stages I and II, accounting for 50 % of the newly diagnosed patients and 60 % of the previously known ones. The amplitude of the anterior chamber was normal in 51,9 %, the iridocorneal angle was narrow in 55,6 %. Intraocular pressure was normal in 66,7 % and axial hypermetropia was found in 55,6 % of cases with corneal dystrophy. No significant association was found between the corneal thickness and cell density, but it was between pleomorphism and polyimegathism in 100 % of patients and in more than 50 % of the direct descendants. CONCLUSIONS: the variables proved to be important in characterizing the disease as well as in pointing endothelial damages among non-sick descendants. This aspect should be considered on the follow-up period.