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.

Historia natural del macroaneurisma arterial de nervio óptico / Natural history of an optic disc macroaneurysm

CASO CLÍNICO: Varón de 76 años con agudeza visual (AV) de 0,05, que presentaba retinopatía hipertensiva, con hemorragias y manchas algodonosas en haz papilomacular y edema macular. La angiografía fluoresceínica mostró una dilatación sacular hiperfluorescente arterial correspondiente con un macroaneurisma papilar y la tomografía de coherencia óptica (OCT), edema macular. A los 2 meses, la AV mejoró a 0,7, con cierre espontáneo del macroaneurisma y resolución del edema macular. DISCUSIÓN: Los macroaneurismas papilares son infrecuentes y presentan dificultades en su diagnóstico. No existe ningún tratamiento seguro y eficaz y, en concordancia con los pocos casos publicados, la involución espontánea permite la recuperación visual

CASE REPORT: A 75-year old man with reduced vision in his left eye (0.05) presented with hypertensive retinopathy, retinal haemorrhages and cotton wool spots in the papillomacular bundle, as well as macular oedema. Fluorescein angiography showed a saccular hyperfluorescent lesion corresponding to arterial macroaneurysm on the optic disc, with the optical coherence tomography demonstrating macular oedema. At 2 months follow-up, vision had improved to 0.7 with spontaneous closure of the macroaneurysm and resolution of the macular oedema. DISCUSSION: Arterial macroaneurysms on the optic disc are unusual and are difficult to diagnose clinically. There is no safe and effective treatment, and our case, as the few single cases reported, showed that they can spontaneously close and enable visual function to recover

[Natural history of an optic disc macroaneurysm]. / Historia natural del macroaneurisma arterial de nervio óptico.

CASE REPORT: A 75-year old man with reduced vision in his left eye (0.05) presented with hypertensive retinopathy, retinal haemorrhages and cotton wool spots in the papillomacular bundle, as well as macular oedema. Fluorescein angiography showed a saccular hyperfluorescent lesion corresponding to arterial macroaneurysm on the optic disc, with the optical coherence tomography demonstrating macular oedema. At 2 months follow-up, vision had improved to 0.7 with spontaneous closure of the macroaneurysm and resolution of the macular oedema. DISCUSSION: Arterial macroaneurysms on the optic disc are unusual and are difficult to diagnose clinically. There is no safe and effective treatment, and our case, as the few single cases reported, showed that they can spontaneously close and enable visual function to recover.

[Delayed diagnosis of ophthalmic artery obstruction due to atrial myxoma]. / Diagnóstico tardío de obstrucción de arteria oftálmica por mixoma atrial.

CASE REPORT: A 56 year old woman with atrial myxoma presented with a visual acuity of no light perception after acute ophthalmic artery obstruction (OAO) associated with stroke. She developed late retinal pigmentary changes due choroidal infarction, typical of the OAO. DISCUSSION: Simultaneous obstruction of the retinal and choroidal circulation was observed in the OAO. Atrial myxoma should be suspected in patients who suffer from OAO associated with stroke. Systemic studies should be performed to find the origin of OAO.

[Subretinal neovascularization and hemorrhages in angioid streaks]. / Neovascularización y hemorragias subretinianas asociadas a estrías angioides.

PURPOSE: To study the development of subretinal neovascularization and/or subretinal hemorrhages in patients with angioid streaks. METHODS: Fifty two eyes from twenty six patients with angioid streaks were included in our study. The diagnosis of angioid streaks was established by ophthalmoscopic and florescein angiographic features. All the patients underwent a complete ophthalmological study that included an examination of the ocular fundus and a retinography every six months. RESULTS: Subretinial neovascularization was found in 38% in this group of patients. Subretinal hemorrhages, either with or without neovascularization, were found in 59%. CONCLUSIONS: There is no known treatment capable of preventing these conditions from appearing, however the patients should be informed about the risk of subretinal hemorrhages after relatively minor ocular trauma. These results may be helpful in the evaluation, prognosis and surgical planning for patients with angioid streaks.

Neovascularización y hemorragias subretinianas asociadas a estrías angioides / Subretinal neovascularization and hemorrhages in angioid streaks

Objetivo: Estudiar el desarrollo de neovascularización subretiniana y/o hemorragias subretinianas en pacientes afectos de estrías angioides. Métodos: 52 ojos, de 26 pacientes, fueron incluidos en nuestro estudio. El diagnóstico de las estrías angioides se estableció mediante oftalmoscopia y angiografía fluoresceínica. Todos los pacientes fueron sometidos, cada seis meses:, a un estudio oftalmológico completo, incluyendo un examen de fondo de ojo y retinografía. Resultados: En ese grupo de pacientes encontramos neovascularización subretiniana en un 38 por ciento. El hallazgo de hemorragias subretinianas, con o sin neovascularización, fue del 59 por ciento. Conclusiones: No se conoce ningún tratamiento capaz de prevenir la aparición de estas complicaciones, pero los pacientes deben ser informados del riesgo de hemorragias subretinianas ante mínimos traumatismos oculares. Estos resultados pueden ser útiles para la evaluación, pronóstico y planificación quirúrgica de los pacientes afectos de estrías angioides. (AU)