Macular edema associated with gyrate atrophy managed with intravitreal triamcinolone: a case report

Abstract

There is a transient therapeutic effect with 4-mg IVTA injection for GA-related ME. After drug clearance, edema recurs, with return of visual acuity to pretreatment level.

The Prevalence of Macular Cysts in Patients with Clinical Cone-Rod Dystrophy Determined by Spectral-Domain Optical Coherence Tomography

“…Macular cysts have been extensively described and proven to be potentially treatable in various hereditary retinal dystrophies, such as RP, 12-14 X-linked retinoschisis, 15,20 choroideremia, 7 gyrate atrophy, 8,9 and enhanced S-cone syndrome. 10,21 In RP, it has been shown that in those patients without cysticappearing macular changes on fundus examination, 32% had macular cysts that were evident on OCT testing in at least one eye, and 18% showed cystic lesions in both eyes.…”

“…1,2 The prevalence of CORD is estimated to be around 1:40,000 in Europe, 3 but may be underestimated because many of the patients diagnosed with cone dystrophy may actually have early stages of CORD. [2][3][4] Macular cysts have been reported in various hereditary night-blinding retinal dystrophies, including retinitis pigmentosa, 5,6 choroideremia, 7 gyrate atrophy, 8,9 and enhanced S-cone syndrome. 10 Cystic macular lesions are also known to occur in X-linked retinoschisis.…”

Gyrate atrophy: clinical and genetic findings in a female without arginine-restricted diet during her first 39 years of life and report of a new OAT gene mutation

“…Oliveira et al [26] detected cystoid macula edema in a 12-year-old boy with advanced gyrate atrophy. Vasconcelos-Santos et al [27] treated a 27-year-old female with intravitreal triamcinolone because of cystoid macula edema, but only with temporary success. In our patient, we saw no reason to inject triamcinolone or VEGF inhibitors, because (i) the pathomechanism of the edema was still unclear, (ii) the cystoid spaces were in the parafoveal area and not in the center, and (iii) the VA after cataract surgery was similar to that 17 years before.…”

“…Additionally, myopia and early posterior subcapsular cataract are common in gyrate atrophy [25]. A further pathomorphological characteristic is cystoid macula edema, which can be detected in optical coherence tomography (OCT) scans [26,27].…”

Retinal detachment and gyrate atrophy of the choroid and retina: case report

“…Areas of progressive atrophy of the choroid and retina can be ob served in the midperiphery with exposure of the sclera and reduced visual acuity, as poor as 20/200 or worse. The low visual acuity may be related to subcapsular cataract, optic atrophy, macular edema or vasculitis (3,4) . Only sporadic cases associating GA with retinal detach ment (RD) have been observed in the literature (3) .…”