Protocolo de tratamiento del edema macular quístico asociado a retinosis pigmentaria y otras distrofias hereditarias de la retina

Abstract

Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the wor-king 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”, “reti-nitis pigmentosa”, “macular edema” and a diagnostic-therapeutic protocol according to thelevels of evidence and recommendations of the “US Agency for Healthcare Research andQuality”.This protocol has been discussed in the monthly meetings of the XAREA DHR group withthe participation of more than 25 experts, creating a consensus document.The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pig-ment 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 internallimiting membrane do not have sufficient evidence.A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patientsand an another for IRD and cataract surgery.Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment withcorticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinicaltrials are required to establish the therapeutic scale in these patients.