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.

[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.