Idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN) syndrome, pigmented paravenous chorioretinal atrophy (PPCRA) are rare clinical manifestations of unknown etiology. In recent years, with the widespread use multimodal imaging methods in diseases, information about pathogenesis course these diseases has increased. PPCRA is often detected incidentally asymptomatic, whereas IRVAN,...

This report describes an atypical case of pigmented paravenous chorioretinal atrophy, associated with focal progressive peripheral retinal microangiopathy, in a 51-year-old black female. The eyes were asymmetrically involved. Although several cases have been reported with typical features of this uncommon entity, none of them have been known to be associated with retinal microangiopathy. The oc...

kindler's syndrome is a rare entity of unknown cause characterized by acral blisters early in life followed by progressive diffuse poikiloderma and cutaneous atrophy. the inheritance pattern of this syndrome is not clear. we report four iranian siblings (three boys and one girl) with this syndrome, who were the result of a consanguineous marriage. in addition to the usual manifestations of the ...

PURPOSE To report extensive chorioretinal atrophy during the long-term course of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initial phase. CASES Four patients with VKH disease were examined more than 10 years after onset of the disease. OBSERVATIONS They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-cl...

PURPOSE To describe a case of bilateral pigmented paravenous chorioretinal atrophy. METHODS Observational case report. RESULTS A 50-year-old female patient complained of right eye pain and decreased near visual acuity. She had a best-corrected visual acuity of 20/20 in both eyes. In the posterior segment, there were atrophic changes along retinal vessels, and bony spicule pigmentation was o...

The aim of this study is to investigate fundus autofluorescence (FAF) as well as fluorescein angiography (FA), indocyanine green angiography (IA), and optical coherence tomography (OCT) in a patient with pigmented paravenous chorioretinal atrophy (PPCRA). A funduscopic examination revealed chorioretinal atrophy along the paravenous area in both eyes. A marked bone spicule pigment clumping toget...

Gyrate atrophy of the choroid and retina is a rare chorioretinal dystrophy inherited in an autosomal recessive pattern. We describe the first documented case of gyrate atrophy from Australia in a 56-year-old woman with a history of previous diagnosis of retinitis pigmentosa and worsening night vision in her right eye over several years. She was myopic and bilaterally pseudophakic, and fundus ex...

A pair of 19-year-old female identical twins was referred to our hospital with progressive visual loss. They exhibited bilateral chorioretinal atrophy involving the midperiphery on fundoscopy and fluorescein angiography. Bilateral visual field constriction was noted on dynamic Goldmann perimetry, and a markedly impaired response was observed on both photopic and scotopic electroretinograms. Cys...