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.