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Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare spectrum of retinal vasculitis, aneurysms, and neuroretinitis affecting young individuals in their third decade. Most of our current knowledge is based on case reports, case series, and a handful of collaborative studies. There is much diversity in treatment approaches and outcomes in the reported literature. We have aggregated published case reports and case series into quantitative and narrative synthesis to draw evidence-based conclusions toward clinical features, atypical and rare findings, systemic associations, disease course, and treatment outcomes. The analysis suggested the disease mostly affects young individuals with a female predilection. Anterior chamber and vitreous inflammation are common than previously believed. The most prevalent pattern of retinal vasculitis in IRVAN eyes is mixed vasculitis, followed by arteritis and phlebitis. Most eyes at the time of presentation have capillary nonperfusion and require treatment. Most eyes retain good visual acuity; however, treatment is required to maintain visual function. Intravitreal antivascular endothelial growth factors administered as an adjunct to retinal laser photocoagulation are more likely to improve visual outcomes. Besides, we have discussed the different hypotheses on the etiopathogenesis of the disease and stronger evidence suggests an inflammatory origin of the disease.

Purpose: This study investigated the correlation between the relative afferent pupillary defect (RAPD) and retinal nerve fiber layer thickness (RNFLT) in optic neuropathy. Methods: RAPD assessment was performed using a log unit neutral density filter bar. Spectral domain optical coherence tomography RTVue-100 (Optovue) was used to examine the subjects. The optic nerve head pattern (ONH) was subdivided and identified for the purpose of the study into circumpapillary RNFLT (cpRNFLT) and peripheral circumpapillary RNFLT (pcpRNFLT). The cpRNFLT, pcpRNFLT and ganglion cell complex (GCC) parameters were analyzed. Results: Eighteen females and twenty three males with asymmetric optic neuropathy and a RAPD participated. Thirty-three subjects had glaucoma and eight had optic neuropathy other than glaucoma. Significant correlations (p

Introduction: Central areolar choroidal dystrophy (CACD) is a rare, inherited disease that can lead to profound visual disturbance. It is characterized by atrophic changes, particularly in the macula. Specific genotypic mutations are responsible for the autosomal dominant form of this disease. However, there is a distinct retinal dystrophy that combines central areolar choroidal dystrophy with autosomal dominantly inherited drusen. It has been proposed that patients with a specific mutation in the peripherin/RDS gene may manifest a combined presentation. Case report: Here we describe a case of a patient who reported with significantly decreased best-corrected visual acuity of fifteen years of duration in the right eye more than the left. Dilated fundus examination found macular changes that were consistent with central areolar choroidal dystrophy. In addition, there was evidence of surrounding congenital drusen throughout the arcades of both eyes. Electrodiagnostics, optical coherence tomography, and fundus fluorescein angiography were used to confirm the diagnosis of CACD. Conclusion: Central areolar choroidal dystrophy normally presents without drusen. However, in patients manifesting a specific mutation, central areolar choridal dystrophy may present in conjunction with drusen. It appears that the Arg142Trp mutation is one of the factors predisposing to drusen formation.