Protocolo de tratamiento del edema macular quístico asociado a retinosis pigmentaria y otras distrofias hereditarias de la retina / Protocol for the treatment of cystoid macular edema secondary to retinitis pigmentosa and other inherited retinal dystrophies

Las distrofias hereditarias de la retina (DHR) son la causa principal de ceguera legal en la población laboral. El edema macular quístico (EMQ) es una de las causas tratables de pérdida visual afectando hasta un 50% de los pacientes. Se ha realizado una revisión bibliográfica combinando «inherited retinal dystrophy», «retinitis pigmentosa», «macular oedema» y un protocolo diagnóstico/terapéutico según los niveles de evidencia y recomendaciones de la «US Agency for Healthcare Research and Quality». Este protocolo se ha discutido en las reuniones mensuales del grupo XAREA DHR con la participación de más de 25 profesionales, creando un documento de consenso. La etiología del EMQ es multifactorial: disfunción de la barrera hematorretiniana, del epitelio pigmentario de la retina y de las células de Müller, inflamación y tracción vítrea. La OCT es la prueba de elección para el diagnóstico y seguimiento del EMQ asociado a las DHR. Los fármacos con mayor grado de evidencia científica son los inhibidores de la anhidrasa carbónica (IAC). Los corticoides, anti-VEGF intravítreos y vitrectomía con pelado de la membrana limitante interna no disponen de suficiente evidencia. Se propone un esquema de tratamiento en el EMQ en las DHR en adultos, otro para pacientes pediátricos y otra en las DHR y cirugía de catarata. Los IAC orales y tópicos son efectivos en el tratamiento del EMQ secundario a las DHR. El tratamiento con corticoides, anti-VEGF y vitrectomía son opciones de segunda línea. Se requieren ensayos clínicos aleatorizados para poder establecer la escala terapéutica en estos pacientes.(AU)

Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the working population. Cystic macular edema (CME) is one of the treatable causes of visual loss, affecting up to 50% of the patients. A bibliographic review has been carried out combining “inherited retinal dystrophy”, “retinitis pigmentosa”, “macular edema” and a diagnostic-therapeutic protocol according to the levels of evidence and recommendations of the “US Agency for Healthcare Research and Quality”. This protocol has been discussed in the monthly meetings of the XAREA DHR group with the participation of more than 25 experts, creating a consensus document. The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pigment epithelium, and Müller cells, inflammation, and vitreous traction.OCT is the test of choice for the diagnosis and follow-up of CME associated with IRD. The drugs with the highest degree of scientific evidence are carbonic anhydrase inhibitors (IAC). Intravitreal corticosteroids, anti-VEGF, and vitrectomy with peeling of the internal limiting membrane do not have sufficient evidence. A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patients and an another for IRD and cataract surgery. Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment with corticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinical trials are required to establish the therapeutic scale in these patients.(AU)

Protocol for the treatment of cystoid macular edema secondary to retinitis pigmentosa and other inherited retinal dystrophies.

Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the working population. Cystic macular edema (CME) is one of the treatable causes of visual loss, affecting up to 50% of the patients. A bibliographic review has been carried out combining "inherited retinal dystrophy", "retinitis pigmentosa", "macular oedema" and a diagnostic-therapeutic protocol according to the levels of evidence and recommendations of the "US Agency for Healthcare Research and Quality". This protocol has been discussed in the monthly meetings of the XAREA DHR group with the participation of more than 25 ophthalmologists, creating a consensus document. The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pigment epithelium, and Müller cells, inflammation, and vitreous traction. OCT is the test of choice for the diagnosis and follow-up of CME associated with IRD. The drugs with the highest degree of scientific evidence are carbonic anhydrase inhibitors (IAC). Intravitreal corticosteroids, anti-VEGF, and vitrectomy with peeling of the internal limiting membrane do not have sufficient evidence. A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patients and another for IRD and cataract surgery. Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment with corticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinical trials are required to establish the therapeutic scale in these patients.

Evaluación de signos tomográficos no exudativos en casos de degeneración macular asociada a la edad de tipo exudativo / Evaluation of non-exudative tomographic signs in cases of exudative age-related macular degeneration

Objetivo Analizar la prevalencia de signos tomográficos no exudativos (signo de cebolla, seudoedema, tubulación de la retina externa, seudoquistes, hendiduras subretinianas y atrofia macular) en pacientes con degeneración macular asociada a la edad neovascular. Material y métodos Un total de 174 ojos de pacientes con degeneración macular asociada a la edad neovascular que no habían recibido tratamiento previo fueron incluidos en el estudio. Se valoró la agudeza visual, la actividad de la neovascularización y la aparición o no de los distintos signos objeto de estudio en los tiempos 0 (visita inicial), 4 meses, un año, año y medio y a los 2 y 3 años de seguimiento. Se evaluaron también: la edad, el sexo, el ojo afecto y el tipo de neovascularización (1, 2, 3, polipoidea o mixta). Los análisis se han realizado mediante el software estadístico R (versión 3.3.2) y el paquete glmmADMB (versión 0.8.3.3). Resultados La presencia de seudoquistes y tubulación de la retina externa va en aumento a lo largo del seguimiento. El signo de cebolla comienza con una frecuencia ascendente hasta los 12 meses, posteriormente desciende a los 18 meses y vuelve a incrementarse a los 24 meses. En cuanto al seudoedema, mantiene un incremento hasta los 18 meses para finalmente descender. Las hendiduras subretinianas son el signo más raro, presentándose en el 1,1% en la primera visita. Finalmente, la atrofia macular, presente en el 12,6% de los ojos inicialmente, se encuentra en el 25% a los 2 años. Conclusión Los seudoquistes, la tubulación de la retina externa y la atrofia macular fueron los signos más prevalentes, mientras que las hendiduras subretinianas fueron los más infrecuentes (AU)

Objective To analyze the prevalence of non-exudative tomographic signs (onion sign, pseudoswelling, external retinal tubulation, pseudocysts, subretinal clefts and macular atrophy) in patients with neovascular age-related macular degeneration. Material and methods A total of 174 eyes of patients with neovascular age-related macular degeneration who had not received previous treatment were included in the study. Visual acuity, neovascularization activity, and the appearance or not of the different signs under study were assessed at times 0 (initial visit), 4 months, one year, year and a half, and at 2 and 3 years of follow-up. The following were also evaluated: age, sex, affected eye and type of neovascularization (1, 2, 3, polypoid or mixed). The analysis were performed using the statistical software R (version 3.3.2) and the glmmADMB package (version 0.8.3.3). Results The presence of pseudocysts and external retinal tubulation increases throughout the follow-up. The onion sign begins with an ascending frequency up to 12 months, then decreases at 18 months and increases again at 24 months. Regarding pseudowelling, it maintains an increase until 18 months to finally decrease. Subretinal clefts is the rarest sign, presenting in 1.1% on the first visit. Finally, macular atrophy, present in 12.6% of the eyes initially, is found in 25% after 2 years. Conclusion Pseudocysts, external retinal tubulation and macular atrophy were the most prevalent signs, while subretinal clefts were the most infrequent (AU)

Evaluation of non-exudative tomographic signs in cases of exudative age-related macular degeneration.

OBJECTIVE: To analyse the prevalence of non-exudative tomographic signs (onion sign, pseudoswelling, external retinal tubulation, pseudocysts, subretinal clefts and macular atrophy) in patients with neovascular age-related macular degeneration. MATERIAL AND METHODS: A total of 174 eyes of patients with neovascular age-related macular degeneration who had not received previous treatment were included in the study. Visual acuity, neovascularization activity, and the appearance or not of the different signs under study were assessed at times 0 (initial visit), 4 months, one year, year and a half, and at 2 and 3 years of follow-up. The following were also evaluated: age, sex, affected eye and type of neovascularization (1, 2, 3, polypoid or mixed). The analysis were performed using the statistical software R (version 3.3.2) and the glmmADMB package (version 0.8.3.3). RESULTS: The presence of pseudocysts and external retinal tubulation increases throughout the follow-up. The onion sign begins with an ascending frequency up to 12 months, then decreases at 18 months and increases again at 24 months. Regarding pseudowelling, it maintains an increase until 18 months to finally decrease. Subretinal clefts is the rarest sign, presenting in 1.1% on the first visit. Finally, macular atrophy, present in 12.6% of the eyes initially, is found in 25% after 2 years. CONCLUSION: Pseudocysts, external retinal tubulation and macular atrophy were the most prevalent signs, while subretinal clefts were the most infrequent.

[X-linked blue cone monochromatism. A familial case report]. / Monocromatismo de conos azules ligado a X. Una familia afecta.

CLINICAL CASE: A family affected by X-linked blue cone monochromatism is presented. There are 4 male affected individuals and 9 female carriers. DISCUSSION: The diagnosis of blue cone monochromatism is based on severely affected color vision with preserved blue function, poor visual acuity, nystagmus, nearly absent photopic ERG, and a family pedigree compatible with X-linked inheritance. The female carriers showed normal visual function and ocular motility. It is important to be familiar with non progressive cone dysgenesis in order to make a genetic diagnosis of the illnesses in this group.

Monocromatismo de conos azules ligado a X. Una familia afecta / X-linked blue cone monochromatism. A familial case report

Caso Clínico: Se presenta una familia afecta de monocromatismo de conos azules ligada a X. Hay 4 varones afectos y 9 mujeres portadoras identificadas.Discusión: El diagnóstico clínico de monocromatismo de conos azules se basó en la presencia de una visión de colores severamente afectada, mala AV, nistagmus, ERG fotópico casi ausente con conservación de la función azul, y una historia familiar compatible con herencia recesiva ligada a X. Las mujeres portadoras tenían función visual y motilidad ocular normal. Es importante conocer las disgenesias no progresivas de conos para poder hacer el diagnóstico genético de las enfermedades incluídas en este grupo

Clinical Case: A family affected by X-linked blue cone monochromatism is presented. There are 4 male affected individuals and 9 female carriers.Discussion: The diagnosis of blue cone monochromatism is based on severely affected color vision with preserved blue function, poor visual acuity, nystagmus, nearly absent photopic ERG, and a family pedigree compatible with X-linked inheritance. The female carriers showed normal visual function and ocular motility. It is important to be familiar with non progressive cone dysgenesis in order to make a genetic diagnosis of the illnesses in this group

[Corneal epithelial complications after vitrectomy: a retrospective study]. / Complicaciones corneales epiteliales de las vitrectomías: estudio retrospectivo.

PURPOSE: To establish the incidence, evolution and final visual outcome of corneal epithelial complications after vitrectomy in a consecutive series performed throughout a period of one year. METHODS: 195 consecutive medical records of patients vitrectomized in 2001 have been retrospectively reviewed. Patients were clinically evaluated the day after vitrectomy, during the first week, and then weekly for 3 months (minimum follow-up). Functional results have been established at the end of this period. RESULTS: 12 out of 195 eyes developed corneal epithelial complications after surgery (6.1%). Eight cases were diagnosed of persistent epithelial defect (4.1%), and four cases (2%) of necrotizing herpetic keratitis. Six out of 12 were diabetic. Corneal epithelial complications affected 10.5% of diabetic and 4.3% of non-diabetic patients (p=0.10). The majority of persistent epithelial defects healed without sequelae in less than two months. Herpetic keratitis required between 3 to 4 months to heal and caused corneal scarring in all cases. CONCLUSIONS: Persistent corneal epithelial defects after vitrectomy are a relatively frequent complication, mainly in diabetic patients. Recurrence of herpetic keratitis must also be kept in mind. This finding has not been previously reported and needs further studies.

[Iatrogenic retinal breaks during vitrectomy: retrospective study]. / Roturas retinianas iatrogénicas en las vitrectomías: estudio retrospectivo.

PURPOSE: To establish the incidence and characteristics of iatrogenic retinal breaks occurring during vitrectomy and to propose a protocol for their management. METHODS: Retrospective study of 195 consecutive vitrectomies performed in 2001. Minimum follow up was 3 months for all cases. RESULTS: 13 patients with iatrogenic retinal breaks (6.6%) have been detected. In 8 cases the breaks affected the peripheral retina, in 4 cases they were located in the posterior pole and there was one retinal dialysis. In 4 cases there were more than one retinal break. 9 patients (70%) were vitrectomized for diabetic retinal complications. In 12 cases, the breaks were detected during surgery and treated with endolaser and/or cryoretinopexy (3 cases located inferiorly). Nevertheless 7 cases (54%) developed a retinal detachment, 2 of which could be reattached. CONCLUSIONS: Among iatrogenic retinal breaks produced during vitrectomy, the peripheral ones are slightly more frequent than the breaks in the posterior pole. Iatrogenic breaks occur more frequently in diabetic patients, having a bad prognosis and often developing a retinal detachment despite endolaser or cryoretinopexy.

[Functional improvement after vision rehabilitation in low monocular vision after myopic macular degeneration and retinal detachment]. / Mejora funcional tras la rehabilitación visual en una paciente con visión monocular deficitaria debido a degeneración macular miópica y desprendimiento de retina.

PURPOSE: We present a case of a 62 year-old woman, with a single eye functional vision (VA of 0.16) who improved her vision after following a vision rehabilitation program, which included optical and non-optical devices for daily performance. CONCLUSIONS: A correct optometric evaluation and a training program are key factors to improve quality of life in low vision patients, whose ocular pathology allows no other treatment.

Mejora funcional tras la rehabilitación visual en una paciente con visión monocular deficitaria debido a degeneración macular miópica y desprendimiento de retina / Functional improvement after vision rehabilitation in low monocular vision after myopic macular degeneration and retinal detachment

Objetivo: En este artículo se presenta el caso de una mujer de 62 años con visión funcional en un solo ojo (0,16) que mejoró sus actividades cotidianas con el uso de ayudas ópticas y no ópticas mediante un programa de Rehabilitación Visual adaptado a sus necesidades. Conclusión: Los únicos requisitos necesarios para conseguir la reincorporación de un paciente de baja visión a sus actividades diarias son una correcta evaluación optométrica acompañada de un programa de entrenamiento visual (AU)

[First results of the IOBA low vision unit]. / Primeros resultados de la Unidad de Baja Visión del IOBA.

PURPOSE: To verify the efficacy a treatment for the rehabilitation of vision in our Unit for Clinical Low Vision in the testing of improvements in patient's efficiency for daily life activities. MATERIAL AND METHOD: 80 patients were studied. Ophthalmologic and optometric evaluation followed by a customised vision rehabilitation program were performed according to patient's immediate needs. In a second stage a telephoned questionnaire was filled in order to record efficacy of prescribed visual aids in their own environment. RESULTS: Rehabilitation training resulted in 89% of patients showing improvement in their quality of life in both fields; functional and psychological. 85% of patients showed improvement in near activities and 35% in far activities. DISCUSSION: A correct and specialised low vision refraction and a posterior vision rehabilitation training in the use of optical and non optical aids show increase in quality of life in patients with severe visual deficiencies.

Primeros resultados de la unidad de baja visión del IOBA / First results of the IOBA low vision unit

Objetivo: Verificar la eficacia del tratamiento mediante Rehabilitación Visual de nuestra Unidad de Baja Visión comprobando la mejora en el rendimiento en las actividades de la vida cotidiana de pacientes con deficiencias visuales graves. Material y métodos: Se estudió un total de 80 pacientes. Tras una evaluación oftalmológica y optométrica completas, fueron sometidos a un programa de rehabilitación visual individualizado adaptado a su resto visual y necesidades inmediatas. Posteriormente se realizó una encuesta telefónica para comprobar la eficacia de las ayudas visuales prescritas en su entorno. Resultados: Un 89 por ciento de los pacientes mejoraron su calidad de vida tanto en el plano funcional como en el psicológico, un 85 por ciento mejoró en las tareas que requerían visión de cerca y un 35 por ciento en las de lejos, posterior al entrenamiento de la rehabilitación visual. Discusión: Una correcta refracción y el posterior entrenamiento en el uso de ayudas ópticas y no ópticas aumenta la calidad de vida de los pacientes con deficiencias visuales severas (AU)