Causes of congenital corneal opacities and their management in a tertiary care center / Causas de opacidades corneanas congênitas e seu controle em um centro de cuidados terciários

ABSTRACT Purpose: To evaluate causes and management of congenital corneal opacities (CCO) diagnosed in a tertiary care eye center and to compare the data with a previous study at the same institution. Methods: Computerized medical records in all patients with congenital corneal opacities diagnosed in the Cornea Service at Wills Eye Hospital (Philadelphia, PA) between January 1, 2007, and December 31, 2015, were retrospectively reviewed. Children aged 12 years and younger at the first visit were included in the study. Patients' demographics, ocular diagnosis, laterality, associated ocular abnormalities, other ocular surgery performed prior or subsequent to the first visit, and their treatment were extracted from the medical records. Results: A total of 77 eyes in 56 patients were examined. The mean age at presentation was 32.8 ± 44.2 months, with the mean follow-up period of 26.7 ± 30.1 months. The most frequent diagnosis was Peters anomaly (53.2%), followed by limbal dermoid (13.0%), aniridia with glaucoma and microphthalmos (6.5%), sclerocornea and congenital glaucoma (5.2%), idiopathic (3.9%), Axenfeld-Rieger anomaly and Hurler syndrome (2.6%), and microcornea (1.3%). Primary keratoplasty was performed in 26 eyes, with the outcome rate in the clear cornea of 76.0% during the follow-up. Conclusion: Peters anomaly is the most common cause of congenital corneal opacities encountered at our institution. Penetrating keratoplasty is the most frequent choice of corneal surgery to treat congenital corneal opacities. Additional interventions during penetrating keratoplasty were moderately positively correlated with graft failure. This study also shows the rates of some etiologies of that changed over the recent decades in our tertiary care Cornea Service. Although Peters anomaly remains the most common presenting reason for congenital corneal opacities, its rate appears to be increasing over the recent decade. Congenital corneal opacities due to birth trauma, which is one of the preventable causes, were observed in a previous study in our clinic; however, no new cases were noted in this study.

RESUMO Objetivo: Avaliar as causas e o controle das opa cidades corneanas congênitas diagnosticadas em um centro oftal mológico de atendimento terciário e comparar os dados com um estudo anterior realizado na mesma instituição. Métodos: Prontuários médicos informatizados de todos os pacientes com opacidade corneana congênita diagnosticada no Serviço de Córnea no Wills Eye Hospital (Filadélfia, PA) entre 1º de ja neiro de 2007 e 31 de dezembro de 2015 foram revisados retrospectivamente. Crianças com 12 anos ou menos na primeira consulta foram incluídas no estudo. A demografia dos pacientes, o diagnóstico ocular, a lateralidade, as anormalidades oculares associadas, outras cirurgias oculares realizadas antes ou após a primeira consulta e o tratamento foram extraídos dos prontuários médicos. Resultados: Um total de 77 olhos de 56 pacientes foi examinado. A idade média de apresentação foi de 32,8 ± 44,2 meses, com um tempo médio de acompanhamento de 26,7 ± 30,1 meses. O diagnóstico mais frequente foi anomalia de Peters (53,2%), seguido por dermóide límbico (13,0%), aniridia com glaucoma e microftalmia (6,5%), esclerocórnea e glaucoma congênito (5,2%), idiopático (3,9%), síndrome de Axenfeld-Rieger e síndrome de Hurler (2,6%) e microcórnea (1,3%). Ceratoplastia primária foi realizada em 26 olhos, com desfecho de córnea clara de 76,0% durante o acompanhamento. Conclusão: A anomalia de Peters é a causa mais comum de opacidade corneana congênita encontrada em nossa instituição. A ceratoplastia penetrante é a escolha mais frequente de cirurgia corneana para o tratamento de opacidades corneanas congênitas. Intervenções adicionais durante a ceratoplastia penetrante foram moderadamente correlacionadas positivamente com a falha do enxerto. Este estudo também mostra as taxas de algumas etiologias do que mudou ao longo faz últimas décadas em nosso serviço de córnea de atendimento terciário. Embora a anomalia de Peters continue a ser a causa mais comum das opacidades congênitas da córnea, sua taxa parece estar aumentando na última década. Opacidades congênitas da córnea devido a trauma no nascimento, que é uma das causas evitáveis, foram observadas em um estudo anterior em nossa clínica; no entanto, nenhum caso novo foi observado neste estudo.

Utilidad del sistema Scheimpflug por Pentacam para la cuantificación de la opacidad de la cápsula posterior en pacientes pseudofáquicos con aceite de silicona / Usefulness of Pentacam Scheimflug system for quantification of the posterior capsule opacity in pseudophakic patients with silicon oil-filled eyes

Objetivo: determinar si el sistema Scheimpflug por Pentacam tiene utilidad en la cuantificación objetiva de la opacidad de la cápsula posterior en los pacientes pseudofáquicos con aceite de silicona. Métodos: se realizó un estudio descriptivo, prospectivo y observacional en el Instituto Cubano de Oftalmología Ramón Pando Ferrer en el período comprendido entre abril del año 2012 y abril de 2014. Se evaluó, mediante sistema Scheimpflug del Pentacam para la cuantificación de la opacidad de cápsula posterior, a un grupo de 31 pacientes seudofáquicos sometidos a una vitrectomía previa con aceite de silicona y se compararon los resultados con los obtenidos en 26 pacientes operados solo de cirugía del cristalino con lente intraocular, para lo cual se utilizaron las variables edad, sexo, opacidad en grados por lámpara de hendidura y la intensidad media de píxeles en un área circular de 3 mm centrales seleccionada en los tomogramas, analizadas por el software Image J 1.42 q. Resultados: en los pacientes pseudofáquicos con aceite de silicona el sistema Scheimpflug no fue capaz de discernir entre los diferentes grados de opacidad de la cápsula posterior(p= 0,210) y no existió concordancia entre ambas mediciones (p= 0,120). Las mediciones realizadas en los pacientes pseudofáquicos con aceite de silicona con la lámpara de hendidura por dos observadores fueron similares (p= 0,042), al igual que existió buena correlación en las realizadas con el sistema Scheimpflug (r= 0,981). Cuando se compararon las mediciones realizadas con el sistema Scheimpflug en los dos grupos de pacientes se observó que las medias de intensidad en los grados 1 y 2 eran superiores a las halladas en los pacientes no vitrectomizados previamente, no así en los grados 3; solo en este último caso la diferencia fue significativa (p= 0,001). Conclusiones: el sistema Scheimpflug no es útil para cuantificar la opacidad de la cápsula posterior en los pacientes vitrectomizados previamente que aún tienen aceite de silicona(AU)

Objective: to determine whether the Pentacam Scheimpflug imaging system is useful for objective quantification of the posterior capsule opacity in pseudophakic patients with silicon oil-filled eyes. Methods: observational, prospective and descriptive study conducted in Ramon Pando Ferrer Cuban Institute of Ophthalmology in the period of April 2012 through April 2014. With the Pentacam Scheimpflug imaging system for the quantification of the posterior capsule opacity, 31 pseudophakic patients, who had undergone previous vitrectomy with silicon oil, were evaluated and their results were then compared with those of patients operated on through crystalline lens surgery and intraocular lens implantation (26 patients). To this end, the analyzed variables were age, sex, opacification degrees measured with slit lamp and average intensity in pixels in a 3mm round area selected from tomographs and analyzed by Image J 1.42 q software. Results: in pseudophakic patients with silicon oil-filled eyes, the Scheimpflug system could not differentiate the different levels of the posterior capsule opacity (p= 0.210) and there was no agreement between both measurements (p= 0.120). The measurements taken by two observers in pseudophakic patients using silicon oil and slit lamp were similar (p= 0.042) and good correlation in those taken with the Scheimpflug system (r= 0.981). When comparing the Scheimpflug system measurements taken in the two groups of patients, it was observed that the intensity means in grades 1 and 2 were higher than those found in non-vitrectomized patients, but in grade 3, the difference was significant (p= 0.001). Conclusions: Scheimpflug system is not useful to measure the posterior capsule opacity in previously vitrectomized patients who still have silicon oil-filled eyes(AU)

AcrySof toric intraocular lens for post-keratoplasty astigmatism.

We report a 63-year-old male who had undergone left eye optical penetrating keratoplasty for central leucomatous corneal opacity 10 years earlier. The eye had clear donor graft with residual astigmatism of –6.50 diopter cylinder (DC) at 30°. The patient underwent clear corneal phacoemulsification with implantation of +6.0 D spherical equivalent AcrySof SN60T9 intraocular lens (IOL). Postoperatively, at 10 months, the patient had distance corrected visual acuity of 20/30 with –2.00 DC at 20°. AcrySof toric IOL offers an effective treatment option for post-keratoplasty high corneal astigmatism in patients with cataract.

Fabry disease - importance of screening in cornea verticillata: case report / Doença de Fabry - importância do rastreamento em córnea verticilata: relato de caso

Fabry disease is an X-linked disease of glycosphingolipid (GL) metabolism. The accumulation of GL in tissues can affect multiple organ systems. Initial symptoms includes episodes of severe pain in the extremities, cornea verticillata and skin lesions. In late stages, kidney, heart and brain can be involved. This report attempts for importance of cornea verticillata in the diagnosis of Fabry disease in a young patient with pain in the lower limb extremities.

A doença de Fabry é um erro inato no metabolismo de glicosfingolipides (GL) ligado ao cromossomo X. O acúmulo de GL nos tecidos pode afetar múltiplos órgãos e sistemas. Os sintomas iniciais incluem episódios de dor nas extremidades, córnea verticilata e lesões na pele. Em estágios avançados, os rins, coração e cérebro podem ser envolvidos. Este relato de caso enfatiza para importância da córnea verticilata para o diagnóstico de doença de Fabry em paciente jovem com dor nos membros inferiores.

Amblyopia and Strabismus by Monocular Corneal Opacity Following Suspected Epidemic Keratoconjunctivitis in Infancy

PURPOSE: To identify the long term clinical course of amblyopia and strabismus that developed secondary to a monocular corneal opacity following suspected epidemic keratoconjunctivitis (EKC) in infancy. METHODS: This was a retrospective study analyzing the medical records of seven patients, treated in our clinic, who were followed for more than five years. RESULTS: Four patients in our clinic underwent a corneal ulcer treatment following suspected EKC. Each developed a monocular corneal opacity. Three patients with a chief complaint of corneal opacity were transferred to our clinic from other clinics. These patients had documented histories of treatment for EKC in infancy. All patients were treated with early occlusion therapy, but amblyopia persisted in four patients. Furthermore, all patients had strabismus and showed a significant reduction of stereoscopic vision. CONCLUSIONS: Although infants with EKC are not always cooperative, slit lamp examination should be performed as early as possible, and appropriate medical treatment should be performed, thus reducing the development of corneal opacity. Careful follow up should be regularly performed, and the occurrence of amblyopia or strabismus should be verified at an early stage using visual acuity or ocular alignment examination. Ophthalmologic treatments, including active occlusion therapy, should also be pursued.

Causes of blindness in people aged 50 years and over: community-based versus hospital-based study

The causes of blindness in Yemen were determined in people aged 50+ years in both a communityand hospital-based study and documented using WHO/PBL criteria. In the community sample of 707 individuals in a rural area of Taiz governorate the prevalence of bilateral blindness was 7.9% and the main causes were cataract [71.4%] and age-related macular degeneration [ARMD] [14.3%]. Corneal opacities and uncorrected aphakia were rare [1 case each] and there were no cases of diabetic retinopathy. Unilateral blindness was found in 8.6% of the community sample. In a case-notes review of 1320 new patients attending an eye clinic in Sana'a, bilateral blindness was documented in 26.5% and unilateral blindness in 9.0% [main causes: cataract, glaucoma, ARMD, diabetic retinopathy, corneal opacities and trauma

Ultrasonography-guided scleral buckle retinopexy and cryotherapy: case report / Retinopexia com "buckle" escleral e crioterapia guiada por ultrassom: relato de caso

If the ocular media are clear, indirect binocular ophthalmoscopy allows retinal detachment and retinal tear identification and treatment under direct visualization. However, if opacities are present preventing direct fundus examination, ultrasonography becomes the most important tool for evaluating the posterior segment. In addition, ultrasonography can be useful in retinal tear treatment by guiding cryotherapy. In this article we describe a rhegmatogenous retinal detachment treatment technique applied to a patient with corneal opacity. Cryopexy and circumferential and radial buckle positioning were guided by ultrasonography, resulting in retinal attachment during the 6-month follow-up period.

Quando os meios oculares são transparentes, oftalmoscopia binocular indireta permite a identificação de descolamento de retina e roturas, bem como seu tratamento sob visibilização direta. Porém, em olhos que apresentam opacidades de meios impedindo o exame oftalmoscópico, a ultrassonografia constitui o exame mais importante do segmento posterior do olho. Além disso, o tratamento de roturas retinianas também pode ser auxiliado pelo uso desse equipamento, orientando a crioterapia. Neste trabalho será apresentada técnica de tratamento de descolamento de retina regmatogênico, no qual a criopexia e o posicionamento dos "buckles" episclerais circunferencial e radial foram guiados pelo ultrassom em paciente com leucoma corneano. O tratamento resultou em aplicação retiniana durante o seguimento em seis meses.

Causes of corneal blindness: a multi-center retrospective review.

OBJECTIVE: To assess prevalence and causes of corneal blindness in four hospitals in Thailand and to compare the causes between hospitals. MATERIAL AND METHOD: A retrospective chart review of six hundred and sixty four patients records with best-corrected visual acuity not better than 6/60 in at least one eye due to corneal disease in Siriraj, Mettapracharak, Sappasitthiprasong and Banmee Hospitals in a one-year period were reviewed. The collected data included age, sex, laterality, causes of corneal blindness, and prognosis. RESULTS: Seven hundred and sixty nine eyes with corneal blindness and a median age of 55.0 years were reviewed Leading causes of cornea blindness were corneal infection (35.6%), surgical bullous keratopathy (27.8%), and trauma (14.0%). The prevalence at Siriraj Hospital was 1.7% (498/28,728 patients). Most of the treatable cases (84.6%) underwent penetrating keratoplasty procedures and 73% of patients had a chance of recovery. CONCLUSION: Corneal infection and surgical bullous keratopathy were the leading causes of corneal blindness.

Anestesia congênita de córnea associada à anestesia de ramo do trigêmio: relato de caso / Congenital corneal anesthesia related to trigeminal anesthesia: case report

A anestesia corneana por ser uma condição rara, freqüentemente é confundida ou não diagnosticada durante o exame de rotina do segmento anterior. Relato de caso de um paciente de 18 anos encaminhado ao ambulatório de córnea e doenças externas com quadro clinico de síndrome de olho seco e com diagnóstico provável de síndrome de Sjõgren. Era amblíope de olho direito devido à opacidade corneana no eixo visual secundária a trauma com unha na infância. Foi pesquisada sensibilidade corneana que era ausente em ambos os olhos; olho seco grave e com BUT (tempo de quebra do filme lacrimal) menor que 4 segundos. Foi feito diagnóstico de anestesia corneana congênita associada a hipoestesia do nervo trigêmio pela avaliação neurológica da sensibilidade facial e movimentos bruscos do queixo que evidenciavam alterações sensoriais do nervo. O oftalmologista geral e principalmente o especialista em segmento anterior devem ter como rotina a pesquisa da sensibilidade corneana no exame do segmento anterior.

Sistema de medidas automáticas de áreas de úlceras de córnea / Automatic measure systems of corneal ulcer area

Úlceras1 são feridas na córnea com alta potencialidade de cegueira, pois mesmo curadas tendem a tirar a transparência. Nos países em desenvolvimento e de natureza agrícola elas respondem por cerca de 10 por cento das causas de cegueira. Uma das formas do clínico acompanhar o tratamento delas é pela estimativa subjetiva de sua dimensão. Evoluções favoráveis tedem a promover uma diminuição da ferida. No entanto, as medidas existentes para essa estimativa são rudimentares. Assim, este trabalho apresenta o desenvolvimento de um sistema de captação de imagem em Lâmpada de Fenda (instrumento utilizado para analisar as córneas) e um "software" dedicado para o delineamento e cálculo da área úlcerada para ser implementado num hospital público (400 pacientes por semana). Inserido no contexto será desenvolvida uma biblioteca de arquivamento e análise de imagens de enfermidades corneanas.

Corneal Ulcer is a very common disease in agricultura! countries and it is responsible for 10% of the blindness causes. One of the main aspects to be observed in these cases is the increasing or decreasing of the affected area. We have been developing an automatic optical system in order to evaluate the affected area (the ulcer) to be implemented in a public hospital (400 patients per week are analyzed). The optical system is implemented in a Slit Lamp and connected to a CCD detector. The image is displayed in a PC monitor by a commercial frame grabber anda dedicated software for detennining the area ofthe ulcer has been developed.

Implantation of posterior chamber intraocular lenses by suture fixation without capsular and zonular support

We implanted sulcus-fixated posterior chamber intraocular lenses (PCL) in three cataractous, six aphakic, and four eyes during penetrating keratoplasty due to corneal opacity in the absence of capsular and zonular support. This technique has been successfully performed in all cases and produced good visual outcome in 12 eyes (93%). In four eyes which had penetrating keratoplasty and the fixation of PCL, one developed vitreous hemorrhage and one developed corneal graft rejection.