Trigeminal neuralgia after corneal transplantation - a case report / Neuralgia do trigêmeo pós-transplante de córnea - relato de caso

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.

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.

Descemet's membrane endothelial keratoplasty with a simplified technique and low complication rate: the samba technique / Ceratoplastia endotelial da membrana de Descemet com técnica simplificada e baixa taxa de complicação: a técnica de samba

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.

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.

Bullous keratopathy in a yellow-headed caracara (Milvago chimachima) treated with a modified third eyelid flap / Ceratopatia bolhosa em caracará (Milvago chimachima) tratado com flap de terceira pálpebra modificado

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)

LASIK hipermetrópico pós-DSAEK (Descement Stripping Automated Endotelial Keratoplasty) / LASIK for hyperopia correction pos DSAEK(Descement Stripping Automated Endotelial Keratoplasty)

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.

Cirugía de catarata en el paciente con fragilidad endotelial. Revisión de la literatura y aproximación para evaluación del riesgo / Cataract surgery in the patient with endothelial fragility. Review of the literature and approach for risk assessment

Propósito: Revisar Los factores de riesgo preoperatorios y quirúrgicos más importantes de daño corneal en pacientes que requieren cirugía de catarata y arrojar una herramienta para estratificar el riesgo y personalizar el abordaje quirúrgico. Diseño: Revisión de la literatura. Métodos: Se realizó una revisión de la literatura utilizando los siguientes términos DeCS: "fragilidad endotelial", "patología corneal" y "riesgo endotelial en cirugía de catarata" en los buscadores PubMed, Scopus, SciELO, Cochrane Library y Google Scholar. Además se utilizaron palabras clave como "endotelio corneal", "Distrofia endotelial de Fuchs", "Queratopatia bulosa pseudofaquica" y "descompensación corneal". Resultados: Un total de 39 artículos de 1978-2017 fueron revisados. Los mayores factores de riesgo de daño endotelial fueron identificados con énfasis en la distrofia endotelial de Fuchs. Con base en parámetros cuantitativos y cualitativos de la función endotelial, la dureza de la catarata y la profundidad de la cámara anterior, se diseñó la Matriz de Evaluación de Riesgo Endotelial para evaluar y asignar un puntaje a cada caso. Dependiendo del puntaje, el cirujano puede elegir entre opciones quirúrgicas como: procedimiento triple, técnicas libres de ultrasonido o facoemulsificación con protección endotelial. Conclusiones: La intersección entre fragilidad endotelial, dureza de la catarata y trauma endotelial en cirugía determina el riesgo de descompensación corneal. La valoración preoperatoria del riesgo usando la Matriz de Evaluación de Riesgo Endotelial tiene el potencial de ayudar en la selección del paciente y de la técnica quirúrgica.

Purpose: To review the most important pre-operative and surgical risk factors for endothelial damage in patients undergoing cataract surgery and to provide a tool for risk stratification and guidelines to customize the surgical approach. Design: Review of the literature. Methods: We performed a review of the literature using the following MeSH terms: "endothelial fragility", "corneal pathology" and "endothelial risk in cataract surgery" in PubMed, Scopus, SciELO, Cochrane Library and Google Scholar. In addition, keywords such as "corneal endothelium", "Fuchs' endothelial dystrophy", "pseudophakic bullous keratopathy" and "corneal decompensation" were used. Results: A total of 39 articles from 1978-2017 were reviewed. The major systemic, ocular and intraoperative factors causative of endothelial cell damage were identified, emphasizing on Fuchs' endothelial dystrophy. Based on selected qualitative and quantitative parameters of endothelial function, nuclear cataract density and anterior chamber depth, the Endothelial Risk Assessment Matrix was designed to evaluate each case and assign a particular score. Depending on the score, the surgeon can decide between options such as triple procedure, zero- ultrasound techniques or phacoemulsification with endothelial protection. Conclusions: The intersection of endothelial fragility, cataract hardness and intraoperative endothelial trauma determines the risk of corneal decompensation. Preoperative evaluation of endothelial risk by scoring with the Endothelial Risk Assessment Matrix has the potential to aid in patient selection and surgical customization.

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.

Estudio generacional en familias con distrofia corneal endotelial de Fuchs / Generational study conducted in families with Fuchs' endotelial corneal dystrophy

OBJETIVOS: identificar la presencia de rasgos clínicos de distrofia corneal endotelial de Fuchs en familiares de pacientes con este diagnóstico. MÉTODOS: se realizó un estudio observacional, descriptivo y transversal de 15 familias trigeneracionales de pacientes con distrofia corneal endotelial de Fuchs, captados en consulta de córnea del Instituto cubano de Oftalmología "Ramón Pando Ferrer" del año 2008 al 2011. La muestra quedó constituida por un total de 78 pacientes. Se utilizaron planillas de recolección de datos para estudiar las siguientes variables de interés: gutas corneales según grados, clasificación clínica, edad, sexo y grado de parentesco según árbol genealógico familiar. Se resumieron las variables cualitativas utilizando frecuencias absolutas y relativas porcentuales. RESULTADOS: del total de descendientes directos, el 44,1 % presentó gutas en su córnea. El 34,6 % de los pacientes estudiados presentaron la enfermedad y el 23,1 % córnea guttata. La distrofia y la córnea guttata se constataron más en el sexo femenino, con mayor incidencia en mayores de 60 años para los primeros. Los descendientes del primer nivel evidenciaron más afectación corneal que los de segundo nivel, con el 75,9 y el 23,5 % respectivamente. Todas las familias presentaron miembros afectados en su descendencia. CONCLUSIONES: la mayor parte de las familias de pacientes con distrofia corneal endotelial de Fuchs presentan cambios endoteliales en las córneas, por lo que estas deben ser incluidas en el estudio con fines diagnósticos y preventivos.

OBJECTIVE: to identify clinical features of Fuchs' endothelial corneal dystrophy in relatives of patients with this diagnosis. METHODS: bservational, descriptive and transversal study of 15 three- generation families of those patients with Fuchs' endothelial corneal dystrophy who went to the cornea consultation of the "Ramón Pando Ferrer", Cuban Institute of Ophthalmology from 2008 to 2011. The sample was made up of 78 patients. Data collection forms were used to study the following variables of interest: Cornea Guttata by degree, clinical classification, age, sex and degree of relationship as family tree. Qualitative variables were summarized using percentage absolute and relative frequencies. RESULTS: of the total number of direct descendants, 44,1 % had cornea guttata; 34,6 % of the patients presented with Fuchs' endothelial corneal dystrophy and 23,1 % of them had cornea guttata. Dystrophy and cornea guttata were found more frequently in females, with the highest incidence of the former in patients over 60 years. First degree descendants showed more corneal involvement than the second degree ones (75,9 and 23,5 %, respectively). All the families had offspring affected by the disease. CONCLUSIONS: most of the members of the families suffered corneal endothelial changes, so they should be included in the study for diagnostic and preventive purposes.

Relevância da biomecânica da córnea no glaucoma / Relevancy of corneal biomechanical in glaucoma

Relato de caso de uma paciente, com córnea espessa, por distrofia de Fuchs em fase inicial, ainda com transparência corneana preservada (edema corneano subclínico), associado à pressão intraocular (PIO) normal por tonometria de aplanação de Goldman (TAG), que teve entretanto, o diagnóstico de glaucoma primário de ângulo aberto com base em alterações estruturais do nervo óptico. O estudo tomográfico demonstrou padrão de paquimetria espacial compatível com edema subclínico. O estudo biomecânico da córnea com o ORA (Ocular Response Analyzer, ® Reichert), associado à medida corrigida da PIO, possibilitou o entendimento da influência da córnea, que apesar de mais espessa, levava a uma TAG falsamente reduzida (hipoestimada). O estudo da PIO com sistema de tonografia digital de contorno (PASCAL) corroborou com os achados do ORA. Este exemplo ressalta a importância de novas tecnologias na avaliação de pacientes com suspeita de glaucoma, e destaca que a correção da TAG, com base em algoritmos lineares relacionados com a paquimetria central apenas, pode determinar sérios erros de interpretação clínica.

Case report of a patient, with a thick cornea, for Fuchs dystrophy in its early stages, yet with preserved corneal transparency (subclinical corneal edema), associated with normal intraocular pressure (IOP) by Goldman applanation tonometry (GAT), which was however, the diagnosis of primary open-angle glaucoma on the basis of structural changes of the optic nerve. The tomographic study showed a pattern of spatial pachymetry compatible with subclinical edema. The corneal biomechanical study with ORA (Ocular Response Analyzer, Reichert ®) associated with corrected IOP measurement, allowed the understanding of the corneal influence, which, although thicker, leading to a falsely low TAG (underestimated). The IOP study with tonography system digital (PASCAL) corroborated with the findings of ORA. This example highlights the importance of new technologies in patients' evaluation with suspected glaucoma, and highlights that the correction of TAG, based on linear algorithms related to central corneal thickness alone, can determine serious errors in clinical interpretation.

Complicações em transplantes endoteliais realizados por estagiários de córnea / Complications in endothelial keratoplasty performed by cornea fellows

OBJETIVO: Reportar complicações precoces numa série de transplantes endoteliais realizados por estagiários de córnea. MÉTODOS: Revisão retrospectiva dos transplantes endoteliais realizados por estagiários de córnea do segundo ano. Descritas as complicações precoces e respectivo tratamento. RESULTADOS: Foram realizados 34 transplantes endoteliais pelos estagiários de córnea entre julho de 2007 e agosto de 2008. Destes, 29 foram realizados pela técnica de "Descemet stripping endothelial keratoplasty" e 5 "Descemet stripping automated endothelial keratoplasty". Foram realizadas 15 cirurgias combinadas, 14 associadas a facoemulsificação com implante de lente intraocular e 1 associada a facectomia com implante de lente intraocular. A principal indicação foi por distrofia de Fuchs com 18 casos, 11 foram indicados por ceratopatia bolhosa após cirurgia de catarata, 4 por falência primária após transplante endotelial e 1 por distrofia endotelial congênita hereditária. A complicação mais encontrada foi descolamentos do botão doado em 8 olhos (23,5%). Falência primária foi encontrado em 7 olhos (20,6%). Glaucoma agudo por bloqueio pupilar foi encontrado em 1 paciente (2,9%). CONCLUSÕES: O alto número de complicações encontradas nestas cirurgias, que foram as primeiras de um grupo de 6 estagiários de córnea, mostra a longa curva de aprendizado para este procedimento cirúrgico. A orientação das primeiras cirurgias por cirurgiões mais experientes pode diminuir a curva de aprendizado e a taxa de complicações.

PURPOSE: To report early complications in endothelial keratoplasty performed by cornea fellows. METHODS: Retrospective study of endothelial keratoplasty performed by second-year cornea fellows. Described the early complications and its treatments. RESULTS: Thirty four endothelial keratoplasty were performed by cornea fellows, from July 2007 to August 2008. From this, 29 were Descemet stripping endothelial keratoplasty and 5 Descemet stripping automated endothelial keratoplasty; 14 combined with phacoemulsification and 1 with extracapsular cataract surgery. The main indication was Fuchs' dystrophy (18 cases), followed by bullous keratopathy (11 cases), primary failure after endothelial keratoplasty (4 cases) and congenital hereditary endothelial dystrophy (1 case). Main surgery complication was donor button detachment in 8 eyes (23.5%), followed by primary graft failure in 7 eyes (20.6%) and acute glaucoma after pupillary block in 1 eye (2.9%). CONCLUSIONS: The complication rate in this series, which were the first ever in a group of six cornea fellows, was high. This shows the steep learning curve for this surgical procedure. The assistance of an experienced surgeon could help to achieve a less steep learning curve with a lower complication rate.

Long-term Evaluation of Endothelial Cell Changes in Fuchs Corneal Dystrophy: The Influence of Phacoemulsification and Penetrating Keratoplasty

PURPOSE: To evaluate the natural course of the long-term endothelial cell changes in Fuchs corneal dystrophy (FCD) patients and investigate the effects of phacoemulsification on the annual rate of change in endothelial indices in FCD patients. METHODS: Thirty-four patients diagnosed with FCD at Seoul National University Hospital from 1994 to 2010 were retrospectively reviewed. Sixteen patients who had been followed up for more than 1 year were selected and classified into 3 groups: group A, patients with no ocular surgery; group B, patients who had undergone phacoemulsification only; and group C, patients who had undergone penetrating keratoplasty with cataract surgery. Endothelial cell density, polymegethism, pleomorphism, and pachymetry were measured and the exponential rates of endothelial cell and pachymetry change were analyzed. RESULTS: A non-linear mixed model of non-operated FCD patients showed that only pachymetric data tended to increase with statistical significance (p = 0.001) with a mean follow-up period of 4.15 years. Using an exponential regression analysis fitting curve, the mean rates of annual endothelial cell loss were 0.82%/yr, 20.39%/yr, and 29.27%/yr in groups A, B, and C respectively, and statistical significance was seen only in group C (p < 0.05). CONCLUSIONS: Retrospective long-term follow-up data showed that changes in endothelial density did not significantly decrease over at least 4 years in middle-aged FCD patients. The changes in pachymetric corneal thickness appeared to increase over the same period. Considering that no exponential changes were aggravated after performing cataract surgery alone, cataract surgery would be a preferable option in FCD patients compared to an approach of "wait-and-do" penetrating keratoplasty combined with cataract surgery.

Relevância da biomecânica da córnea no glaucoma / Relevancy of corneal biomechanical in glaucoma

Relato de caso de uma paciente, com córnea espessa, por distrofia de Fuchs em fase inicial, ainda com transparência corneana preservada (edema corneano subclínico), associado à pressão intraocular (PIO) normal por tonometria de aplanação de Goldman (TAG), que teve entretanto, o diagnóstico de glaucoma primário de ângulo aberto com base em alterações estruturais do nervo óptico. O estudo tomográfico demonstrou padrão de paquimetria espacial compatível com edema sub-clínico. O estudo biomecânico da córnea com o ORA (Ocular Response Analyzer, ® Reichert), associado à medida corrigida da PIO, possibilitou o entendimento da influência da córnea, que apesar de mais espessa, levava a uma TAG falsamente reduzida (hipoestimada). O estudo da PIO com sistema de tonografia digital de contorno (PASCAL) corroborou com os achados do ORA. Este exemplo ressalta a importância de novas tecnologias na avaliação de pacientes com suspeita de glaucoma, e destaca que a correção da TAG, com base em algoritmos lineares relacionados com a paquimetria central apenas, pode determinar sérios erros de interpretação clínica.

Case report of a patient, with a thick cornea, for Fuchs dystrophy in its early stages, yet with preserved corneal transparency (subclinical corneal edema), associated with normal intraocular pressure (IOP) by Goldman applanation tonometry (GAT), which was however, the diagnosis of primary open-angle glaucoma on the basis of structural changes of the optic nerve. The tomographic study showed a pattern of spatial pachymetry compatible with subclinical edema. The corneal biomechanical study with ORA (Ocular Response Analyzer, Reichert ®) associated with corrected IOP measurement, allowed the understanding of the corneal influence, which, although thicker, leading to a falsely low TAG (underestimated). The IOP study with tonography system digital (PASCAL) corroborated with the findings of ORA. This example highlights the importance of new technologies in patients' evaluation with suspected glaucoma, and highlights that the correction of TAG, based on linear algorithms related to central corneal thickness alone, can determine serious errors in clinical interpretation.

Rabies in a blind patient: Confusion after corneal transplantation.

Two blind persons received corneal transplants from a single donor who showed no signs of rabies before he died. One of the recipients, a young girl, died 16 days later of rabies and the other recipient survived. We discuss the possible mode of transmission of rabies to the first recipient and the management of the second recipient.

Descemet membrane endothelial keratoplasty with a stromal rim in the treatment of posterior polymorphous corneal dystrophy.

A 20-year-old patient, diagnosed with posterior polymorphous corneal dystrophy, developed corneal edema for which he underwent Descemet membrane endothelial keratoplasty with a stromal rim (DMEK-S) in the right eye. No intra- or postoperative complications were noted. At the last follow-up 2 years and 9 months after the procedure, the best corrected visual acuity was 1.0 and endothelial cell density declined from 3533 cells/mm2 to 1012 cells/mm2. Despite the endothelial cell loss, DMEK-S appears to be a good alternative to other surgical techniques for the treatment of corneal endotheliopathies, and it may be of benefit to young patients.

Long-Term Outcomes of Penetrating Keratoplasty in Treating Macular Corneal Dystrophy, TGFBI Dystrophy, and Fuchs' Dystrophy

PURPOSE: To compare long-term outcomes of penetrating keratoplasty in treating macular corneal dystrophy, transforming growth factor beta induced (TGFBI) dystrophy, and Fuchs' dystrophy. METHODS: The present retrospective study describes the postoperative results of 123 eyes in 70 patients; 53 eyes with macular corneal dystrophy, 20 eyes with TGFBI dystrophy, and 50 eyes with Fuchs' dystrophy. All patients received penetrating keratoplasty. The outcome measures included recurrence rate, graft rejection, graft survival rate and corneal endothelial density and other complications. RESULTS: As compared with the other groups, TGFBI dystrophy had the highest recurrence rate (p-value < 0.0001). Fuchs' dystrophy had the lowest graft survival rate (p-value = 0.03). Corneal endothelial cell density was lowest in Fuchs' dystrophy 10 years postoperatively (p-value = 0.0006), but there was no significant difference in corneal endothelial cell density between the 3 groups 1 and 5 years postoperatively. There was no significant difference between the 3 groups in other complications such as graft rejection, secondary glaucoma and persistent epithelial defect (p-value = 0.809, p-value = 0.584, p-value = 0.972, respectively). CONCLUSIONS: Penetrating keratoplasty showed different long-term outcomes in treating 3 types of corneal dystrophies. The difficulty may depend on the pathogenesis of corneal dystrophies.

Dupla membrana de Descemet após ceratoplastia penetrante - abordagem cirúrgica durante a facoemulsificação: relato de caso / Double Descemet's membrane after penetrating keratoplasty - surgical approach during phacoemulsification: case report

Apresentamos um caso retenção inadvertida de membrana de Descemet durante ceratoplastia penetrante por distrofia endotelial de Fuchs. Comentamos as dificuldades encontradas para sua remoção durante a cirurgia de catarata subsequente, bem como propomos uma nova abordagem cirúrgica.

A case of inadvertent retention of Descemet's membrane after penetrating keratoplasty for Fuchs dystrophy is reported. The challenges to remove it during cataract surgery are commented, as well as a proposal of a new surgical approach.

Amyloid corneal deposition in corneal buttons of congenital hereditary endothelial dystrophy [CHED] - a clinical and histopathological case series

To determine the frequency, pathology and clinical relevance of amyloid deposited in corneas of CHED. Clinical and histopathological case series. Amyloid subepithelial deposition was found in 5 [6.6%] corneal buttons of 75 patients with histopathologically confirmed CHED diagnosis. Clinical findings included history of parental consanguinity, poor vision [ranging from counting fingers from one foot to 3/200], corneal edema, and central whitish subepithelial corneal nodules in all the five cases and positive family history in 4 of 5 cases. The patients underwent PKP at a mean age of 15 years [range 3-22 years]. Histological findings included attenuated endothelium [6/6] thickened Descemet's membrane [6/6], stromal edema [2/6], and subepithelial amyloid deposits [6/6]. All patients improved from vision point of view. To date, no recurrence of the amyloid has been seen in the grafts. Considering the consanguinity, family history, early onset, and bilaterality, this study supports our hypothesis that the amyloid is primary in nature in our patients and indicates a new subtype of autosomal recessive CHED that require further chemical and genetic analysis. This subtype has the same prognosis for PKP as all CHED patients, if not better

Distrofia endotelial de Fuchs / Fuchs' endothelial dystrophy

La distrofia endotelial de Fuchs es una enfermedad de patogenia desconocida, que cursa en forma asintomática hasta el desarrollo del edema corneal, el cual se desarrolla a partir de una falla primaria del endotelio (menor número de células funcionantes). Suele ser bilateral, lentamente progresiva y generalmente asimétrica. El cuadro se da más frecuentemente en mujeres. Los primeros síntomas son el déficit visual y el deslumbramiento matutinos, que aparecen desde la quinta o sexta década de la vida. El signo más precoz para la detección en la etapa asintomática es el hallazgo de guttas al examen biomicroscópico, con aspecto clásico "en metal golpeado" del endotelio corneal. El tratamiento varía en función del estadio y severidad del cuadro, desde el tratamiento tópico con gotas hiperosmóticas a la intervención quirúrgica, siendo la queratoplastia penetrante la técnica de elección para la resolución del problema visual y el dolor en estos pacientes. Actualmente también se utiliza la queratoplastia laminar posterior, con buenos resultados.