Value of anti-VEGF treatment in choroidal neovascularization associated with autosomal recessive bestrophinopathy.

A 26-year-old white woman presented with a 1-year history of reduced vision in both eyes, bilateral yellowish deposits in the central macula, and pale yellow retinal flecks extending to midretinal periphery. Choroidal neovascularization (CNV) was confirmed in her left eye. On optical coherence tomography, both eyes showed diffuse intraretinal cystic spaces, thickening and separation of the photoreceptor layer from the retinal pigment epithelium (RPE), subretinal fluid, and focal thickening at the level of the RPE at the fovea. A diagnosis of autosomal recessive bestrophinopathy was confirmed by electrodiagnostic and molecular genetics testing. The CNV responded well to intravitreal ranibizumab therapy, and visual acuity in the left eye improved and stabilized; however, retinoschisis due to fluctuations in intraretinal fluid persisted. This case highlights the fact that current optical coherence tomography-driven protocols used widely to treat neovascular age-related macular degeneration may not be appropriate for CNV associated with other retinal diseases.

Therapeutic challenges to retinitis pigmentosa: from neuroprotection to gene therapy.

Syndromic retinitis pigmentosa (RP) is the result of several mutations expressed in rod photoreceptors, over 40 of which have so far been identified. Enormous efforts are being made to relate the advances in unraveling the patho-physiological mechanisms to therapeutic approaches in animal models, and eventually in clinical trials on humans. This review summarizes briefly the current clinical management of RP and focuses on the new exciting treatment possibilities. To date, there is no approved therapy able to stop the evolution of RP or restore vision. The current management includes an attempt at slowing down the degenerative process by vitamin supplementation, trying to treat ocular complications and to provide psychological support to blind patients. Novel therapeutic may be tailored dependant on the stage of the disease and can be divided in three groups. In the early stages, when there are surviving photoreceptors, the first approach would be to try to halt the degeneration by correction of the underlying biochemical abnormality in the visual cycle using gene therapy or pharmacological treatment. A second approach aims to cope with photoreceptor cell death using neurotrophic growth factors or anti-apoptotic factors, reducing the production of retino-toxic molecules, and limiting oxidative damage. In advanced stages, when there are few or no functional photoreceptors, strategies that may benefit include retinal transplantation, electronic retinal implants or a newly described optogenetic technique using a light-activated channel to genetically resensitize remnant cone-photoreceptor cells.

Early multifocal electroretinogram findings during intravitreal ranibizumab treatment for neovascular age-related macular degeneration.

PURPOSE: To evaluate changes in the multifocal electroretinogram (mfERG) in patients with neovascular age-related macular degeneration (nAMD) undergoing ranibizumab treatment. METHODS: This was an observational, longitudinal, prospective study. Treatment-naive patients with nAMD who met the inclusion and exclusion criteria underwent a course of monthly injections of ranibizumab over 3 months. At baseline and month 3, each subject was evaluated with best corrected visual acuity (BCVA), contrast sensitivity (CS), fluorescein and indocyanine green angiography, optical coherence tomography (OCT), and mfERG. Additional mfERGs were performed at weeks 1 and 4 and BCVA and OCT at weeks 4 and 8. RESULTS: Eighteen patients were enrolled. Between baseline and week 12, median BCVA improved from 59 to 69 ETDRS letters (P = 0.001), median CS improved from 29 to 30 letters (P = 0.05), mean OCT central foveal subfield thickness (CFT) decreased from 294 to 199 µm (P = 0.005), mean P1 amplitude density of the mfERG central zone increased from 35.85 to 51.55 nV/deg(2) (P = 0.009). The mfERG response correlated positively with BCVA (F = 22; P < 0.0001) and negatively with CFT (F = 12.73; P = 0.00078). CONCLUSIONS: Intravitreal ranibizumab therapy appears to induce an increase in mfERGs centrally in patients with nAMD at least in the short term. Longer term studies to investigate the prognostic value of mfERG responses to predict changes in visual acuity in nAMD and other diseases are warranted. (ClinicalTrials.gov number, NCT01023971.).

Bilateral severe fibrinous anterior uveitis--an unusual complication of pamidronate therapy exacerbated by topical latanoprost.

AIM: The aim of this study was to report a case of severe bilateral fibrinous anterior uveitis following pamidronate therapy in a patient on latanoprost. METHODS: This study is presented as an interventional case report. RESULTS: Clinical examination showed bilateral severe fibrinous uveitis following an intravenous infusion of disodium pamidronate. Ocular signs and symptoms responded to stopping latanoprost and treatment with oral prednisolone (60 mg) and hourly topical prednisolone acetate 1%. The reintroduction of latanoprost resulted in a recurrence, which was stopped with subsequent improvement. CONCLUSIONS: Mild anterior uveitis is an unfamiliar adverse effect of pamidronate therapy. However, severe fibrinous uveitis has not been previously described. This may be due to the compounding effect of latanoprost. This case highlights the importance of history taking and awareness of the otherwise uncommon side effect of this commonly prescribed medication.