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PURPOSE: The present review will summarize FECD-associated genes and pathophysiology, diagnosis, current  therapeutic approaches, and future treatment perspectives. METHODS: Literature review. RESULTS: Fuchs' endothelial corneal dystrophy (FECD) is the most common bilateral corneal dystrophy and accounts for one-third of all corneal transplants performed in the US. FECD is caused by a combination of genetic and non-heritable factors, and there are two types: early-onset FECD, which affects individuals from an early age and is usually more severe, and late-onset FECD, which is more common and typically manifests around the age of 40. The hallmark findings of FECD include progressive loss of corneal endothelial cells and the formation of focal excrescences (guttae) on the Descemet membrane. These pathophysiological changes result in progressive endothelial dysfunction, leading to a decrease in visual acuity and blindness in later stages. The present review will summarize FECD-associated genes and pathophysiology, diagnosis, current therapeutic approaches, and future treatment perspectives. CONCLUSION: With the characterization and understanding of FECD-related genes and ongoing research into regenerative therapies for corneal endothelium, we can hope to see more significant improvements in the future in the management and care of the disease.

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PURPOSE: The aim of this study was to evaluate the efficacy of artificial intelligence-derived morphometric parameters in characterizing Fuchs corneal endothelial dystrophy (FECD) from specular microscopy images. METHODS: This cross-sectional study recruited patients diagnosed with FECD, who underwent ophthalmologic evaluations, including slit-lamp examinations and corneal endothelial assessments using specular microscopy. The modified Krachmer grading scale was used for clinical FECD classification. The images were processed using a convolutional neural network for segmentation and morphometric parameter estimation, including effective endothelial cell density, guttae area ratio, coefficient of variation of size, and hexagonality. A mixed-effects model was used to assess relationships between the FECD clinical classification and measured parameters. RESULTS: Of 52 patients (104 eyes) recruited, 76 eyes were analyzed because of the exclusion of 26 eyes for poor quality retroillumination photographs. The study revealed significant discrepancies between artificial intelligence-based and built-in microscope software cell density measurements (1322 ± 489 cells/mm 2 vs. 2216 ± 509 cells/mm 2 , P < 0.001). In the central region, guttae area ratio showed the strongest correlation with modified Krachmer grades (0.60, P < 0.001). In peripheral areas, only guttae area ratio in the inferior region exhibited a marginally significant positive correlation (0.29, P < 0.05). CONCLUSIONS: This study confirms the utility of CNNs for precise FECD evaluation through specular microscopy. Guttae area ratio emerges as a compelling morphometric parameter aligning closely with modified Krachmer clinical grading. These findings set the stage for future large-scale studies, with potential applications in the assessment of irreversible corneal edema risk after phacoemulsification in FECD patients, as well as in monitoring novel FECD therapies.

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BACKGROUND/PURPOSE: The purpose of this study was to analyze Descemet stripping endothelial keratoplasty (DSEK) outcomes and develop a nomogram to compute the probability of 3- and 5-year DSEK graft survival based on risk factors. STUDY DESIGN/METHODS: The medical records of 794 DSEK procedures between January 1, 2008, and August 1, 2019, were retrospectively reviewed to identify 37 variables. We also evaluated for the presence of corneal graft failure, defined as irreversible and visually significant graft edema, haze, or scarring. Variables were assessed by multivariable Cox models, and a nomogram was created to predict the probability of 3- and 5-year graft survival. RESULTS: Graft failure occurred in 80 transplants (10.1%). The strongest risk factors for graft failure included graft detachment [hazard ratio (HR) = 4.46; P < 0.001], prior glaucoma surgery (HR = 3.14; P = 0.001), and glaucoma (HR = 2.23; P = 0.018). A preoperative diagnosis of Fuchs dystrophy was associated with a decreased risk of graft failure (HR = 0.47; P = 0.005) compared with secondary corneal edema. Our nomogram has a concordance index of 0.75 (95% confidence interval, 0.69 to 0.81), which indicates that it may predict the probability of graft survival at 3 and 5 years with reasonable accuracy. We also analyzed graft rejection, which occurred in 39 cases (4.9%). The single risk factor found to be significantly associated with graft rejection was prior glaucoma surgery (HR = 2.87; P = 0.008). CONCLUSIONS: Our nomogram may accurately predict DSEK graft survival after 3 and 5 years based on 4 variables. This nomogram will empower surgeons to share useful data with patients and improve collective clinical decision-making.

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Donor to host transmission of infectious agents is a well-recognized entity, more commonly related to Endothelial Keratoplasty (EK) than to Penetrating Keratoplasty (PK), that involves complications ranging from interface keratitis to endophthalmitis. A systematic review of the literature was conducted to identify the published articles until December 2020 reporting cases of endophthalmitis secondary to corneal graft contamination in posterior lamellar keratoplasties (DSAEK and DMEK) along with donor characteristics, microbiological profile, treatment and outcomes. Each identified article was assessed to meet donor to host infection criteria, defined as a post-procedural infection in which the same microbiological agent was identified in both the donor corneoscleral rim or preservation medium and receptor ocular tissue. From 23 research articles, eight reports of endophthalmitis in nine patients following DSAEK or DMEK secondary to donor to host infection fulfilled the inclusion criteria. The majority were male and the median age was 72.0 (45.0-81.0) years old. Indications of surgery were primarily pseudophakic bullous keratopathy and Fuchs dystrophy. A fungal pathogen was identified in eight of nine patients. All the cases underwent surgical management with lenticule removal or endothelial plaque aspiration. The final corrected distance visual acuity (CDVA) in all cases was 20/200 or better. Endophthalmitis after an EK procedure is a rare complication whose outcome depends on the aggressive and precocious treatment. Identification of early signs of interface keratitis and lenticule removal seems mandatory in patients undergoing DSAEK or DMEK to prevent further involvement of the globe.

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PURPOSE: To describe the demographic characteristics and clinical course of Fuchs endothelial corneal dystrophy (FECD) in a Mexican-mestizo population. METHODS: A retrospective observational and longitudinal study was performed in consecutive patients with the clinical diagnosis of Fuchs endothelial corneal dystrophy seen at our institution. Initial and last follow-up best-corrected visual acuity, slit-lamp findings, and specular microscopy endothelial morphometric parameters were analyzed. RESULTS: One hundred and two eyes belonging to 51 patients were included in the analysis. Median age at the time of diagnosis was 69 years (range, 25-87 years) with a female-to-male ratio of 3.3:1. Visual loss (40%) followed by glare (13.3%) and fluctuating matutine vision loss (13.3%) was the most common complaints at presentation. Regarding FECD staging, 65 (63.7%) were classified as stage-I FECD, 21 (20.6%) stage-II, and 15 (14.7%) as stage-III. A high percentage of eyes (44.1%) presented visual impairment ( ≤ 20/50) at presentation, and the presence of isolated corneal guttata was the most common stage of presentation (64%) at slit-lamp examination. While fifty-nine (57.8%) eyes did not require any medical or surgical management, 17 (16.7%) eyes were managed with hypertonic saline eyedrops alone or in combination with bandage contact lens, and 18 (17.6%) required corneal transplantation. Penetrating keratoplasty alone (8 eyes, 44.4%), or in combination with cataract extraction and intraocular lens implantation (3 eyes, 16.7%), was the most frequent surgical technique performed. CONCLUSION: Demographical characteristics of Fuchs dystrophy regarding age at presentation, gender distribution, and clinical stage at the time of diagnosis did not differ significantly from other international reports. Almost 20% of these patients will require keratoplasty during the disease, emphasizing the need for safer and more reproducible keratoplasty techniques.

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PURPOSE: To present an alternative technique (Pachy-DSEK) for the manual preparation of thin endothelial lamellae in Descemet stripping endothelial keratoplasty (DSEK), as well as to evaluate its visual and anatomic outcomes. METHODS: A retrospective chart review was conducted in 15 cases who underwent DSEK at a private eye clinic in Brazil (INOB, Brasília) from June 2017 to December 2019. All patients had ocular comorbidities and relative contraindications to Descemet membrane endothelial keratoplasty (DMEK). All endothelial lamellae were manually prepared by using a standardized technique. Best corrected visual acuity (BCVA), tomographic parameters and graft's thickness were evaluated preoperatively and at 6 months. Endothelial cell counts were evaluated preoperatively and at 12 to 24 months. RESULTS: During preparation there was one case (6%) of peripheral tear and no tissue was lost. At 6 months, the median BCVA improved from 1.60 to 0.40 logMAR (p = 0.0009). There was no significant change in anterior (p = 0.507) and posterior astigmatism (p = 0.483), anterior (p = 0.683) and posterior mean keratometry (p = 0.767), and total corneal power (p = 0.952). The median central graft thickness at 6 months was 80 µm. Ultrathin grafts (<130 µm) were achieved in 80% of cases. At 12 to 24 months endothelial cell count decreased significantly. Graft's detachment occurred in two cases (13%) and endothelial rejection in one case (6%). CONCLUSION: By using ultrasonic pachymetry intra-operatively and standardizing graft preparation, most manually dissected endothelial lamellae were ultrathin. Pachy-DSEK was safe and effective for treating endothelial disfunction in eyes with ocular comorbidities. It may be a cost-effective alternative to automated dissection methods.

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ABSTRACT Description of a patient with Fuchs endothelial dystrophy submitted to a corneal transplant, performed by Descemet membrane endothelial keratoplasty, which evolved with sudden, paroxysmal pain in the frontotemporal region, postoperatively. Due to the ophthalmologic picture of the patient, the attending physician believed in possible rejection of the graft, neglecting the complaint of pain. Even after a successful second transplant, performed due to primary failure, disabling pain persisted and the physician did not manage it. After years of investigation, consulting with several specialists, it was concluded the patient presented trigeminal neuralgia that had not been treated since the surgical procedure. In addition, it led to several psychosocial consequences. Therefore, it is essential to be aware trigeminal neuralgia is a possible outcome of corneal transplantation, and its symptoms should not be neglected by the attending physician, thus contributing to better management for transplanted patients.

RESUMO Descrição do relato de caso de uma paciente com distrofia endotelial de Fuchs submetida a transplante de córnea, realizado pela técnica DMEK, que evoluiu com quadro de dor súbita, paroxística, em região frontotemporal, no pós-operatório. Devido ao quadro oftalmológico da paciente, o médico assistente acreditava em possível rejeição do enxerto, negligenciando a dor. Mesmo após sucesso do segundo transplante, realizado devido à falência primária, as dores incapacitantes persistiam, e nenhuma conduta, por parte do médico, foi realizada. Após anos de investigação, mediante consultas com diversos especialistas, concluiu-se que a paciente apresentava um quadro de neuralgia do nervo trigêmeo que não tinha sido tratada desde a realização do procedimento cirúrgico. Além disso, apresentava uma série de consequências psicossociais. Portanto, torna-se imprescindível entender que a neuralgia do nervo trigêmeo é um possível desfecho do transplante de córnea, e seus sintomas não devem ser negligenciados por parte do médico assistente, contribuindo para melhores condutas para os pacientes transplantados.

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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.

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PURPOSE: We report a simplified Descemet's membrane endothelial keratoplasty (DMEK) technique that involves safe and effective preparation and introduction, correct orientation, and easy unfolding of the donor graft inside the recipient anterior chamber. METHODS: In this retrospective study, we assessed the surgical outcomes of 26 eyes of 23 consecutive patients (mean age, 61.2 ± 11.4 yr; range, 39-82 yr) with Fuchs endothelial corneal dystrophy (n=19) or bullous keratopathy (n=7) who underwent the Samba technique, a simplified DMEK method, at the Sorocaba Ophthalmology Hospital, Sorocaba Eye Bank, Sorocaba, Brazil, between August 2011 and July 2012. RESULTS: Of the 26 operated eyes, only two (7.7%) experienced partial graft detachment requiring rebubbling, and in those eyes, the graft was reattached successfully with one air bubble. There were no cases of primary graft failure, tissue loss, or pupillary block. All patients with good visual potential achieved a best-corrected visual acuity of 20/30 or better at 6 months, and 82.6% achieved a best-corrected visual acuity of 20/30 or better 1 month postoperatively. CONCLUSION: In this retrospective study, the Samba technique, a simplified DMEK procedure, was safe and effective, with an acceptably low rebubbling rate and no incidence of primary graft failure or pupillary block. Moreover, rapid and nearly complete visual recovery was achieved. This simplified DMEK technique can be adopted by corneal surgeons worldwide as a primary treatment for endothelial dysfunction with a less steep learning curve and low rate of postoperative complications.

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ABSTRACT Purpose: We report a simplified Descemet's membrane endothelial keratoplasty (DMEK) technique that involves safe and effective preparation and introduction, correct orientation, and easy unfolding of the donor graft inside the recipient anterior chamber. Methods: In this retrospective study, we assessed the surgical outcomes of 26 eyes of 23 consecutive patients (mean age, 61.2 ± 11.4 yr; range, 39-82 yr) with Fuchs endothelial corneal dystrophy (n=19) or bullous keratopathy (n=7) who underwent the Samba technique, a simplified DMEK method, at the Sorocaba Ophthalmology Hospital, Sorocaba Eye Bank, Sorocaba, Brazil, between August 2011 and July 2012. Results: Of the 26 operated eyes, only two (7.7%) experienced partial graft detachment requiring rebubbling, and in those eyes, the graft was reattached successfully with one air bubble. There were no cases of primary graft failure, tissue loss, or pupillary block. All patients with good visual potential achieved a best-corrected visual acuity of 20/30 or better at 6 months, and 82.6% achieved a best-corrected visual acuity of 20/30 or better 1 month postoperatively. Conclusion: In this retrospective study, the Samba technique, a simplified DMEK procedure, was safe and effective, with an acceptably low rebubbling rate and no incidence of primary graft failure or pupillary block. Moreover, rapid and nearly complete visual recovery was achieved. This simplified DMEK technique can be adopted by corneal surgeons worldwide as a primary treatment for endothelial dysfunction with a less steep learning curve and low rate of postoperative complications.

RESUMO Objetivo: Relatar uma técnica simplificada de ceratoplastia endotelial da membrana de Descemet (DMEK) que envolve a preparação e a introdução seguras e eficazes, a orientação correta e o fácil desdobramento do enxerto doador dentro da câmara anterior receptora. Métodos: Neste estudo retrospectivo, foram revisados e avaliados os resultados cirúrgicos de 26 olhos de 23 pacientes consecutivos (idade média: 61,2 ± 11,4 anos, intervalo: 39 a 82 anos) com distrofia corneana endotelial de Fuchs (n=19) ou ceratopatia bolhosa (N=7) submetidos à técnica "Samba", método de DMEK simplificado, no Hospital Oftalmológico de Sorocaba, Banco de Olhos de Sorocaba, Sorocaba, Brasil, entre agosto de 2011 e julho de 2012. Resultados: Dos 26 olhos operados, apenas 2 olhos (7,7%) apresentaram descolamento parcial do enxerto que necessitou de nova injeção de ar na câmara anterior "re-bubble", e nesses olhos o enxerto foi posicionado com sucesso com o procedimento de "re-bubble". Nenhum dos 26 olhos apresentaram falência primária do enxerto ou perda de tecido, ou bloqueio pupilar. Todos os pacientes com bom potencial visual obtiveram a acuidade visual melhor corrigida de 20/30 ou melhor e 82,6% tinham acuidade visual melhor corrigida de 20/30 ou melhor com 1 mês de cirurgia. Conclusão: Neste estudo retrospectivo, a técnica de Samba, um procedimento de DMEK simplificado, mostrou-se segura e eficaz, com uma taxa de "re-bubble" aceitavelmente baixa e nenhuma incidência de falência primária ou complicação com bloqueio pupilar. Além disso, a recuperação visual rápida e completa foi rapidamente alcançada. Esta técnica DMEK simplificada pode ser adotada por cirurgiões de córnea em todo o mundo como um tratamento primário para disfunção endotelial com uma curva de aprendizado rápida e baixa taxa de complicações pós-operatórias.

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PURPOSE: To compare outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) and Descemet membrane endothelial keratoplasty (DMEK) when an experienced DSAEK surgeon transitions to DMEK while following 2 published endothelial keratoplasty surgical techniques. METHODS: This is a retrospective review of 200 eyes of 132 patients with Fuchs corneal dystrophy that underwent endothelial keratoplasty performed by one surgeon. Published, standardized techniques were followed for both DSAEK and DMEK. Postoperative complications were recorded. Best spectacle-corrected visual acuity (BSCVA), intraocular pressure, and central endothelial cell loss (ECL) were evaluated at 6 months postoperatively. RESULTS: There were no intraoperative complications. One DSAEK and 5 DMEK grafts developed graft detachment requiring rebubbling (P = 0.097). No iatrogenic primary graft failures occurred in the DSAEK group compared with one in the DMEK group. No pupil block episodes occurred in the DSAEK group compared with one in the DMEK group. Posterior synechiae formation occurred 15 times in the DMEK group and did not occur in the DSAEK group (P < 0.001). At 6 months, BSCVA was better in the DMEK group than in the DSAEK group (20/24; logMAR = 0.0844-20/32; logMAR = 0.2063) (P < 0.001). More eyes reached 20/20 or better BSCVA in the DMEK group compared with DSAEK (54.5%-13%) (P < 0.011). At 6 months, ECL was higher in the DMEK group than in the DSAEK group (31.9%-19.9%) (P < 0.001). CONCLUSIONS: Complications can be minimized and excellent outcomes can be achieved, without a steep learning curve, when an experienced DSAEK surgeon transitions to DMEK following a standardized technique. Six-month vision outcomes are better in the DMEK group; however, the rate of a newly described complication, posterior synechiae formation, and 6-month ECL are higher in the DMEK group than in the DSAEK group.

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PURPOSE: To report a case of bilateral Urrets-Zavalia syndrome (UZS) after Descemet stripping automated endothelial keratoplasty (DSAEK). METHODS: Case report. RESULTS: A 61-year-old patient with Fuchs endothelial dystrophy initially developed UZS in the left eye after DSAEK. Thirteen months later, she underwent combined cataract surgery and DSAEK in the right eye, and postoperatively she was noted to have elevated intraocular pressure and a fixed and dilated pupil on the first postoperative day. Both pupils showed partial improvement in reactivity over the course of weeks to months. This is the first case of bilateral UZS after DSAEK surgery and in a patient with Fuchs dystrophy. CONCLUSIONS: UZS may occur bilaterally after DSAEK. Because elevated intraocular pressure has been shown to be a risk factor for this condition, this should be monitored closely during the postoperative period.

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A Yellow-headed Caracara (Milvago chimachima) was submitted to the Ophthalmology Service of the Federal University of Bahia with a corneal abnormality. During ophthalmic evaluation the right cornea was stained positively with fluorescein; a blurred bullous lesion, with irregular surface, compatible with the diagnosis of bullous keratopathy was found. This is a rare condition in a bird which was treated successfully with a modified third eyelid flap associated with antibiotic and hyaluronic acid eye drops. The adopted therapeutic proved to be simple to implement and viable for repair of the bullous keratopathy in the Yellow-headed caracara.(AU)

Um gavião carrapateiro (Milvago chimachima) foi encaminhado ao Serviço de Oftalmologia Veterinária da Universidade Federal da Bahia com alteração em córnea. Durante a avaliação oftálmica a prova da fluoresceína foi positiva no olho direito e foi observado lesão bolhosa de aspecto irregular, compatível com o diagnóstico de ceratopatia bolhosa. Esta é uma alteração rara em aves e no presente caso foi tratada através de "flap" modificado de terceira pálpebra associado a utilização de colírio antibiótico e ácido hialurônico. A terapêutica adotada foi de fácil implementação e viável no reparo da ceratopatia bolhosa em gavião carrapateiro.(AU)

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A Yellow-headed Caracara (Milvago chimachima) was submitted to the Ophthalmology Service of the Federal University of Bahia with a corneal abnormality. During ophthalmic evaluation the right cornea was stained positively with fluorescein; a blurred bullous lesion, with irregular surface, compatible with the diagnosis of bullous keratopathy was found. This is a rare condition in a bird which was treated successfully with a modified third eyelid flap associated with antibiotic and hyaluronic acid eye drops. The adopted therapeutic proved to be simple to implement and viable for repair of the bullous keratopathy in the Yellow-headed caracara.(AU)

Um gavião carrapateiro (Milvago chimachima) foi encaminhado ao Serviço de Oftalmologia Veterinária da Universidade Federal da Bahia com alteração em córnea. Durante a avaliação oftálmica a prova da fluoresceína foi positiva no olho direito e foi observado lesão bolhosa de aspecto irregular, compatível com o diagnóstico de ceratopatia bolhosa. Esta é uma alteração rara em aves e no presente caso foi tratada através de flap modificado de terceira pálpebra associado a utilização de colírio antibiótico e ácido hialurônico. A terapêutica adotada foi de fácil implementação e viável no reparo da ceratopatia bolhosa em gavião carrapateiro.(AU)

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RESUMO O DSAEK (Descemet stripping automated endotelial Keratoplasty) é um dos procedimentos de escolha para tratamento das doenças que acometem o endotélio corneano. Apesar do sucesso terapêutico, o procedimento pode induzir uma hipermetropia residual. Em média a refração se estabiliza em um perído que varia de 6 a 12 meses após o transplante. O objetivo desse relato é descrever a evolução dessa opção terapêutica utilizada em um paciente de 54 anos portador de Distrofia de Fuchs. O paciente foi submetido ao transplante de córnea (DSAEK) e a remoção do cristalino no mesmo tempo cirúrgico o que resultou em hipermetropia residual. Após 3 anos de acompanhamento apresentava-se com a melhor visão corrigida de 20/20 (Snellen) no olho operado com uma refração de +3,25 -1,00 (5º). Optou-se por realização de LASIK (Laser assisted in situ Keratomileusis) hipermetrópico, obtendo um resultado visual satisfatório.

ABSTRACT DSAEK (Descemet stripping automated endothelial keratoplasty) is one of the options for corneal endothelium disease, which in some patients can result in a residual hyperopia after the procedure. Usually 6 to 12 months after corneal transplantation refraction is already stable. This report describes a therapeutic option used in a 54 years old patient with Fuchs' endothelial dystrophy submitted to cataract and corneal transplant that resulted in residual hyperopia, three years after the procedure the best corrected vision was 20/20 with a refraction of +3.25 -1.00 (5 º) treated with Hyperopic - LASIK (Laser-assisted In Situ Keratomileusis) with satisfactory visual result.

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