Diagnóstico diferencial de las reacciones inflamatorias tras uso de medicación intravítrea en degeneración macular asociada a la edad: estéril vs. infeccioso / Differential diagnosis of endophthalmitis after intravitreal drug injection for age related macular degeneration: Sterile vs. infectious

El interés por las complicaciones inflamatorias tras la inyección intravítrea de fármacos antiangiogénicos ha aumentado tras la comercialización de brolucizumab y el desarrollo de nuevas moléculas como el abicipar pegol. Dichos fármacos se asocian a una tasa de complicaciones inflamatorias mayor a los antiangiogénicos clásicos. En este contexto resulta clave el diferenciar procesos infecciosos y estériles para realizar un tratamiento efectivo y precoz. El solapamiento del cuadro clínico entre procesos infecciosos y estériles, la baja tasa de positividad en los cultivos y la heterogeneidad en la terminología son barreras para el correcto diagnóstico y reporte de las complicaciones inflamatorias tras la inyección de medicación antiangiogénica intravítrea. Los cuadros estériles comienzan de forma precoz tras la inyección, dentro de las primeras 48 h, o alrededor de 20 días después en los casos de vasculitis asociada a brolucizumab. Los procesos infecciosos comienzan como promedio en el tercer día tras la inyección, y hasta una semana después de la misma. La disminución grave de la agudeza visual, el dolor severo, la hiperemia severa, el hipopion y un mayor grado de inflamación intraocular deben orientar hacia procesos infecciosos. En los casos en que exista duda sobre la etiología de la inflamación, debe procederse a un control muy estrecho del paciente o al tratamiento antimicrobiano empírico junto con la toma de muestra para evitar complicaciones derivadas de una endoftalmitis infecciosa. Por el contrario, los fenómenos estériles deben ser tratados con observación en los casos más leves o corticoterapia adaptada a la gravedad de inflamación en los casos más graves (AU)

The recent release of brolucizumab and the development of new antiangiogenic molecules as abicipar pegol has increased the interest towards inflammatory complications after intravitreal drug injection. Those drugs are associated to a higher rate of inflammatory adverse events compared to classic drugs. In this context it is essential to differentiate between sterile and infectious cases for a fast and effective treatment. The clinical similarities between infectious and sterile cases, the high rate of culture negative patients and the heterogeneity in the terminology used are obstacles for a correct diagnosis and report of these complications. Sterile cases appear early after the injection, before 48h; or 20 days after in brolucizumab-related vasculitis cases. Infectious cases show up around the third day after injection and up to a week after it. A severe visual impairment, severe pain, severe hyperemia, hypopyon and a more severe intraocular inflammatory process are signs of a likely infectious origin. If the cause of the inflammation is uncertain we must follow up the patient closely or “tap and inject” antimicrobial agents in order to prevent the eventual complications of an infectious endophthalmitis. On the other hand, sterile endophthalmitis might be observed in mild cases or treated with steroids according to the severity of the inflammation (AU)

Differential diagnosis of endophthalmitis after intravitreal drug injection for age related macular degeneration: sterile vs. infectious.

The recent release of brolucizumab and the development of new antiangiogenic molecules as abicipar pegol has increased the interest towards inflammatory complications after intravitreal drug injection. Those drugs are associated to a higher rate of inflammatory adverse events compared to classic drugs. In this context it is essential to differentiate between sterile and infectious cases for a fast and effective treatment. The clinical similarities between infectious and sterile cases, the high rate of culture negative patients and the heterogeneity in the terminology used are obstacles for a correct diagnosis and report of these complications. Sterile cases appear early after the injection, before 48 h; or 20 days after in brolucizumab-related vasculitis cases. Infectious cases show up around the third day after injection and up to a week after it. A severe visual impairment, severe pain, severe hyperemia, hypopyon and a more severe intraocular inflammatory process are signs of a likely infectious origin. If the cause of the inflammation is uncertain we must follow up the patient closely or "tap and inject" antimicrobial agents in order to prevent the eventual complications of an infectious endophthalmitis. On the other hand, sterile endophthalmitis might be observed in mild cases or treated with steroids according to the severity of the inflammation.

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.

Guía de recomendaciones para el manejo de brolucizumab / Guidance on brolucizumab management recommendations

Objetivo: Brolucizumab, un anti-VEGF de nueva generación, ha demostrado su eficacia y seguridad en la degeneración macular asociada a la edad neovascular exudativa (DMAEn) en los ensayos pivotales HAWK y HARRIER. Tras su comercialización, se han reportado eventos adversos relacionados con la inflamación intraocular no detectados previamente. Una revisión post hoc independiente de los ensayos pivotales cifra la tasa de inflamación intraocular (IIO) en el 4,6%. El objetivo de este trabajo es proponer una serie de recomendaciones para implementar el manejo de brolucizumab en la práctica clínica. Método: Las recomendaciones realizadas por los autores se han basado en su experiencia clínica y en la revisión crítica de: 1)los ensayos pivotales; 2)el análisis post hoc del Comité de Revisión de Seguridad, y 3)la literatura publicada. Resultados: En los ensayos pivotales, brolucizumab mostró ganancias funcionales sostenidas, resultados anatómicos superiores con intervalos entre inyecciones potencialmente más prolongados y un perfil de seguridad global bien tolerado. Los eventos adversos reportados tras la comercialización incluyen vasculitis retiniana y la oclusión vascular retiniana. De acuerdo con la información disponible, los expertos recomiendan: 1)descartar los perfiles de pacientes no recomendados (historial previo de IIO); 2)explorar al paciente antes de cada inyección para descartar la presencia de IIO activa; 3)monitorizar al paciente para detectar precozmente los signos de alerta, y 4)tratar de inmediato en el caso de que se desarrolle algún evento adverso. ConclusionesLos : eventos adversos reportados son poco frecuentes, pero pueden estar asociados con una pérdida severa e irreversible de agudeza visual. Las recomendaciones realizadas pretenden facilitar el manejo de brolucizumab en la práctica habitual de los retinólogos, garantizar la seguridad del paciente y, en caso de que se produzca alguno de los eventos adversos, minimizar su impacto sobre la visión. (AU)

Purpose: Brolucizumab, a new generation anti-VEGF, has demonstrated efficacy and safety in AMD in the pivotal HAWK and HARRIER trials. Post-marketing, previously undetected adverse events related to intraocular inflammation have been reported. An independent post hoc review of the pivotal trials puts the rate of intraocular inflammation (IOI) at 4.6%. The aim of this paper is to propose a set of recommendations for implementing the management of brolucizumab in clinical practice. Method: The recommendations made by the authors are based on their clinical experience and critical review of (i)the pivotal trials; (ii)the post-hoc analysis of the Safety Review Committee, and (iii)the published literature. Results: In the pivotal trials, brolucizumab showed sustained functional gains, superior anatomical outcomes with potentially longer intervals between injections and a well-tolerated overall safety profile. Adverse events reported post-marketing include retinal vasculitis and retinal vascular occlusion. Based on the available information, experts recommend (i)ruling out non-recommended patient profiles (prior history of ORI); (ii)screening the patient prior to each injection to rule out active IOI; (iii)monitoring the patient for early warning signs, and (iv)treating immediately should any adverse events develop. Conclusions: The adverse events reported are rare, but may be associated with severe and irreversible loss of visual acuity. The recommendations made are intended to facilitate the management of brolucizumab in the routine practice of retinologists, to ensure patient safety and, should any adverse events occur, to minimise their impact on vision. (AU)

Guidance on brolucizumab management recommendations.

PURPOSE: Brolucizumab, a new generation anti-VEGF, has demonstrated efficacy and safety in AMD in the pivotal HAWK and HARRIER trials. Post-marketing, previously undetected adverse events related to intraocular inflammation have been reported. An independent post hoc review of the pivotal trials puts the rate of IOI at 4.6%. The aim of this paper is to propose a set of recommendations for implementing the management of brolucizumab in clinical practice. METHODS: The recommendations made by the authors are based on their clinical experience, critical review of (i) the pivotal trials, the post-hoc analysis of the Safety Review Committee, (ii), and (iii) the published literature. RESULTS: In the pivotal trials, brolucizumab showed sustained functional gains, superior anatomical outcomes with potentially longer intervals between injections and a well-tolerated overall safety profile. Adverse events reported post-marketing include retinal vasculitis and retinal vascular occlusion. Based on the available information, experts recommend (i) ruling out non-recommended patient profiles (prior history of ORI), (ii) screening the patient prior to each injection to rule out active ORI, (iii) monitoring the patient for early warning signs, and (iv) treating immediately should any adverse events develop. CONCLUSIONS: The adverse events reported are rare, but may be associated with severe and irreversible loss of visual acuity. The recommendations made are intended to facilitate the management of brolucizumab in the routine practice of retinologists, to ensure patient safety and, should any adverse events occur, to minimise their impact on vision.

Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1 g of corticosteroids and mycophenolate mofetil.

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3 logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1 g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2 g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1 g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100 mg per day), subtenon triamcinolone (40 mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs.

Vogt-Koyanagi-Harada: tratamiento de la recurrencia tras la administración de 3 bolos intravenosos de 1g de corticoides y micofenolato de mofetilo / Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1g of corticosteroids and mycophenolate mofetil

Presentamos el caso de una mujer hondureña de 27 años sin antecedentes médicos de interés que presentó múltiples áreas de desprendimientos de retina (DR) exudativos y una mejor agudeza visual corregida de 1,3logMAR en ambos ojos. Fue diagnosticada de síndrome de Vogt-Koyanagi-Harada incompleto y tratada con prednisolona intravenosa (1g/24h) durante 3 días, tal y como recomienda la evidencia publicada, junto con micofenolato de mofetilo (2g/24h). Durante el descenso paulatino de corticoesteroides, los DR recidivaron, por lo que se reintrodujeron las megadosis de 1g/24h de corticoesteroides intravenosos durante 6 días hasta la resolución completa de los DR y se añadieron ciclosporina (100mg/24h), triamcinolona subtenoniana (40mg/mL) e inyecciones intravítreas mensuales de ranibizumab en ambos ojos, con un adecuado control de la inflamación coroidea que se tradujo en la remisión de los síntomas y signos (AU)

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100mg per day), subtenon triamcinolone (40mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs (AU)

Vogt-Koyanagi-Harada: treatment of recurrence after administration of 3 intravenous bolus of 1g of corticosteroids and mycophenolate mofetil. / Vogt-Koyanagi-Harada: tratamiento de la recurrencia tras la administración de 3 bolos intravenosos de 1g de corticoides y micofenolato de mofetilo.

The case is presented on a young Honduran female with no medical history of note, who presented with multiple areas of exudative retinal detachment (RD), and a best-corrected visual acuity of 1.3logMAR in both eyes. She was diagnosed with incomplete Vogt-Koyanagi-Harada syndrome, and treated early with a combination of intravenous therapy with 1g of prednisolone per day for 3 days, as recommended by published evidence, as well as mycophenolate mofetil (2g per day). During the corticosteroids tapering, there was a recurrence of exudative retinal detachments, and megadoses of 1g of intravenous corticosteroids per day were reintroduced for 6 days until the complete resolution of the fluid of the exudative RD, and cyclosporine (100mg per day), subtenon triamcinolone (40mg/mL), and intravitreal ranibizumab once a month in the both eyes were added to the treatment, with a great control of choroidal inflammation that resulted in the remission of symptoms and signs.

Retinal pigment epithelial tears.

A retinal pigment epithelial (RPE) tear is a well-known complication of retinal pigment epithelial detachments (PED) and may cause a significant visual impairment. The most common cause is a vascularized PED in patients with exudative age-related macular degeneration (AMD). The development of diagnostic imaging techniques brings us closer to the etiology and pathophysiological mechanisms of this entity, offering us new strategies for treatment and follow-up. The advent of intravitreal antiangiogenic treatment (anti-VEGF) has led to an increase in the number of reported cases of RPE tears, which are an important vision-limiting factor during treatment. However, RPE tears may occur spontaneously or as a consequence of thermal laser treatment, photodynamic therapy or anti-VEGF therapy. It is accepted that the mechanism of RPE tears is multifactorial. The optimization of the functional outcome of this complication has been described with continuous treatment with antiangiogenic drugs. The goal of the present review is to evaluate the incidence, risk factors and treatment of RPE tears.

Incidencia y resultados clínicos de las distintas formas neovasculares de degeneración macular asociada a la edad en Valencia (España) / Incidence and clinical outcomes of the different neovascular forms of age-related macular degeneration in Valencia (Spain)

OBJETIVO: Analizar la incidencia y los resultados visuales de cada uno de los subtipos de lesión neovascular en pacientes con degeneración macular asociada la edad (DMAE). MATERIAL Y MÉTODOS: Revisión retrospectiva de pacientes con DMAE neovascular tratados en el Hospital Universitario y Politécnico la Fe de Valencia por un mismo retinólogo (RGP) desde diciembre de 2012 hasta julio del 2015. Se registraron las formas anatómicas del complejo neovascular, así como el número de tratamientos intravítreos administrados y el cambio de visión obtenido con este. RESULTADOS: Fueron incluidos 174 ojos de 156 pacientes con una edad media de 79,9 años y un seguimiento de al menos 4 meses. El 40,8% presentaban neovascularización coroidea (NVC) tipo 1; el 12%, tipo 2; el 31%, tipo 3; el 14,4% presentaban formas mixtas y el 1,7%, vasculopatía polipoidea. La agudeza visual inicial media era de 0,32 y de 0,38 a los 24 meses, habiendo recibido una media de 9,3 inyecciones. Las formas neovasculares tipo 2, 3 y mixtas mostraron un resultado visual significativamente inferior a las tipo 1, no existiendo significación estadística en la vasculopatía polipoidea. CONCLUSIONES: La NVC tipo1 fue la más observada, y además se relacionó con un mejor pronóstico visual, en comparación con el resto de lesiones neovasculares, en pacientes tratados con ranibizumab

OBJECTIVE: To analyse the incidence and outcomes of the different neovascular subtypes in age-related macular degeneration (AMD). MATERIAL AND METHODS: A retrospective review was carried out on patients with neovascular AMD treated in the University and Polytechnic Hospital la Fe in Valencia by the same retinal physician (RGP) between December 2012 and July 2015. The anatomic classification of the neovascular lesions was recorded, as well as the number of intravitreal treatments administered and the change in visual acuity (VA) obtained throughout the follow-up. RESULTS: A total number of 174 eyes of 156 patients (mean age: 79.9 years) with a minimum follow-up of 4 months were included. The anatomic classification of choroidal neovascularisation (CNV) showed the presence of type 1 lesions in 40,8%, type 2 lesions in 12%, type 3 lesions in 31%, and mixed lesions in 14.4%, with 1.7% showing polypoidal choroidal vasculopathy features. Overall, the mean baseline VA was 0,32, improving to 0,38 at 24 months, after having received a mean of 9.3 injections. Type 2, 3, and mixed forms showed a visual result significantly lower than type1, but there was no significant difference in the polypoidal vasculopathy. CONCLUSIONS: Type 1 CNV was the most common finding, and was associated with a better visual prognosis, compared to the other neovascular lesions

Angiografía mediante tomografía de coherencia óptica: ¿presente o futuro en la práctica clínica diaria? / Optical coherence tomography angiography: Present or future in daily clinical practice?

No disponible

Incidence and clinical outcomes of the different neovascular forms of age-related macular degeneration in Valencia (Spain). / Incidencia y resultados clínicos de las distintas formas neovasculares de degeneración macular asociada a la edad en Valencia (España).

OBJECTIVE: To analyse the incidence and outcomes of the different neovascular subtypes in age-related macular degeneration (AMD). MATERIAL AND METHODS: A retrospective review was carried out on patients with neovascular AMD treated in the University and Polytechnic Hospital la Fe in Valencia by the same retinal physician (RGP) between December 2012 and July 2015. The anatomic classification of the neovascular lesions was recorded, as well as the number of intravitreal treatments administered and the change in visual acuity (VA) obtained throughout the follow-up. RESULTS: A total number of 174 eyes of 156 patients (mean age: 79.9years) with a minimum follow-up of 4 months were included. The anatomic classification of choroidal neovascularisation (CNV) showed the presence of type1 lesions in 40,8%, type2 lesions in 12%, type3 lesions in 31%, and mixed lesions in 14.4%, with 1.7% showing polypoidal choroidal vasculopathy features. Overall, the mean baseline VA was 0,32, improving to 0,38 at 24months, after having received a mean of 9.3 injections. Type2, 3, and mixed forms showed a visual result significantly lower than type1, but there was no significant difference in the polypoidal vasculopathy. CONCLUSIONS: Type 1 CNV was the most common finding, and was associated with a better visual prognosis, compared to the other neovascular lesions.

Telangiectasias maculares idiopáticas de tipo 2: prevalencia en nuestra área y estudio morfométrico y fenotípico / Idiopathic macular telangiectasia type 2: Prevalence and a morphometric and phenotypic study

Objetivo: Las telangiectasias maculares idiopáticas de tipo 2 (MacTel 2) son una enfermedad retiniana poco frecuente y aún no completamente caracterizada. El objetivo del presente trabajo es describir las características clínicas en imagen multimodal de la retina, presentar los resultados funcionales y estimar la prevalencia de la enfermedad en nuestro medio. Métodos: Estudio retrospectivo en el que se analizaron 12 ojos de 6 pacientes con MacTel 2. Se realizaron fotografías en color de fondo, autofluorescencia, angiografía fluoresceínica, tomografía de coherencia óptica (OCT) y angiografía por OCT y se analizaron posteriormente. Se registró prospectivamente la agudeza visual. La prevalencia se estableció en base en los pacientes referidos a una unidad especializada en enfermedades maculares. El período mínimo de seguimiento fue de 18 meses. Resultados: La prevalencia de MacTel2 en nuestro medio fue de 0,12%. Se presentaron las características clínicas en imagen multimodal de la retina. La agudeza visual permaneció estable durante el seguimiento (p = 0,492). Tres pacientes desarrollaron neovascularización coroidea (NVC), requiriendo tratamiento intravítreo con agentes antiangiogénicos. Conclusiones: La prevalencia de la enfermedad en nuestro medio es del 0,12%. El estudio mediante imágenes multimodales permite un diagnóstico y seguimiento de esta patología más preciso. La agudeza visual se mantuvo durante los 18 meses de seguimiento. Los pacientes que desarrollan NVC y son tratados con agentes antiangiogénicos parecen responder adecuadamente a ellos. Se necesitan más estudios para establecer estas conclusiones (AU)

Objective: Type 2 idiopathic macular telangiectasia (MacTel2) is a rare retinal disease that has still not been well-defined. The aim of the present manuscript is to describe the clinical features by multimodal retinal imaging, to present the functional characteristics, and to estimate the prevalence of the disease. Methods: A retrospective study was conducted on the 12 eyes of 6 patients with MacTel2. Fundus colour photographs, fundus autofluorescence, fluorescein angiography, optical coherence tomography (OCT), and OCT-angiography were performed and subsequently analysed. Visual acuity (VA) was prospectively recorded. The prevalence was established based on the patients referred to a specialised macular diseases unit. Minimum follow-up period was 18 months. Results: Prevalence of MacTel 2 in our study was 0.12%. Clinical features were presented using multimodal retinal imaging. VA remained stable during follow-up. Three patients developed choroidal neovascularisation (CNV), requiring intravitreal treatment with antiangiogenic agents. Conclusions: The prevalence of the disease found was 0.12%. The study using multimodal imaging allows a more accurate diagnosis and follow-up of this pathology. The VA is maintained during the 18-month follow-up (P = .492). Patients who develop CNV and are treated with antiangiogenic agents appear to respond adequately to them. More studies are needed to establish these conclusions (AU)

Atrofia geográfica: etiopatogenia y terapias actuales / Geographic atrophy: Etiopathogenesis and current therapies

La atrofia geográfica se caracteriza por un déficit visual severo cuya etiología y fisiopatología aún están por dilucidar. Como hipótesis de trabajo, el daño oxidativo desencadenaría una inflamación crónica en el complejo membrana de Bruch-EPR-coriocapilar, cobrando protagonismo la activación del complemento. Algunos sujetos con mutaciones en el sistema del complemento y otros factores tienen menor capacidad en la modulación de la respuesta inflamatoria, lo que se traduce en daño celular y acumulación de desechos. Esta acumulación de desechos intracelulares y extracelulares se manifiesta como drusas y alteraciones pigmentarias que preceden a la atrofia de fotorreceptores, EPR y coriocapilar, existiendo un proceso isquémico de base con disminución del flujo coroideo. Todos estos procesos se objetivan como hallazgos tomográficos y signos de la autofluorescencia que son útiles en la evaluación de los pacientes con DMAE atrófica, pudiendo establecer un pronóstico individualizado. Terapias antiinflamatorias, antioxidantes y reductora de la acumulación de toxinas para la preservación de células del EPR y fotorreceptores están siendo investigadas para disminuir el avance de esta enfermedad

Geographic atrophy is characterized by severe visual deficit whose etiology and pathophysiology are yet to be elucidated. As a working hypothesis, oxidative damage could trigger a chronic inflammation in Bruch's membrane-RPE-choriocapillaris complex, mostly due to complement pathway overactivation. Some individuals with mutations in the complement system and other factors have diminished capacity in the modulation of the inflammatory response, which results in cell damage and waste accumulation. This accumulation of intracellular and extracellular waste products manifests as drusen and pigmentary changes that precede the atrophy of photoreceptors, RPE, choriocapillaris with an ischemic process with decreased choroid flow. All these processes can be detected as tomographic findings and autofluorescence signals that are useful in the evaluation of patients with atrophic AMD, which helps to establish an individualized prognosis. Anti-inflammatory, antioxidant and therapies that decrease the accumulation of toxins for the preservation of the RPE cells and photoreceptors are being investigated in order to slow down the progression of this disease

Geographic atrophy: Etiopathogenesis and current therapies. / Atrofia geográfica: etiopatogenia y terapias actuales.

Geographic atrophy is characterized by severe visual deficit whose etiology and pathophysiology are yet to be elucidated. As a working hypothesis, oxidative damage could trigger a chronic inflammation in Bruch's membrane-RPE-choriocapillaris complex, mostly due to complement pathway overactivation. Some individuals with mutations in the complement system and other factors have diminished capacity in the modulation of the inflammatory response, which results in cell damage and waste accumulation. This accumulation of intracellular and extracellular waste products manifests as drusen and pigmentary changes that precede the atrophy of photoreceptors, RPE, choriocapillaris with an ischemic process with decreased choroid flow. All these processes can be detected as tomographic findings and autofluorescence signals that are useful in the evaluation of patients with atrophic AMD, which helps to establish an individualized prognosis. Anti-inflammatory, antioxidant and therapies that decrease the accumulation of toxins for the preservation of the RPE cells and photoreceptors are being investigated in order to slow down the progression of this disease.

Idiopathic macular telangiectasia type 2: Prevalence and a morphometric and phenotypic study. / Telangiectasias maculares idiopáticas de tipo 2: prevalencia en nuestra área y estudio morfométrico y fenotípico.

OBJECTIVE: Type 2 idiopathic macular telangiectasia (MacTel2) is a rare retinal disease that has still not been well-defined. The aim of the present manuscript is to describe the clinical features by multimodal retinal imaging, to present the functional characteristics, and to estimate the prevalence of the disease. METHODS: A retrospective study was conducted on the 12 eyes of 6 patients with MacTel2. Fundus colour photographs, fundus autofluorescence, fluorescein angiography, optical coherence tomography (OCT), and OCT-angiography were performed and subsequently analysed. Visual acuity (VA) was prospectively recorded. The prevalence was established based on the patients referred to a specialised macular diseases unit. Minimum follow-up period was 18 months. RESULTS: Prevalence of MacTel 2 in our study was 0.12%. Clinical features were presented using multimodal retinal imaging. VA remained stable during follow-up. Three patients developed choroidal neovascularisation (CNV), requiring intravitreal treatment with antiangiogenic agents. CONCLUSIONS: The prevalence of the disease found was 0.12%. The study using multimodal imaging allows a more accurate diagnosis and follow-up of this pathology. The VA is maintained during the 18-month follow-up (P=.492). Patients who develop CNV and are treated with antiangiogenic agents appear to respond adequately to them. More studies are needed to establish these conclusions.