Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis: Novel Findings and Optical Coherence Tomography Angiography Analysis

Abstract

Purpose: To present novel findings in stellate nonhereditary idiopathic foveomacular retinoschisis, including the largest series of optical coherence tomography angiography findings to date.Methods: A retrospective case series with multimodal imaging was obtained and reviewed.Results: All three patients were women, aged 59-63. Two cases were unilateral, and one was bilateral. Vision ranged from 20/20 to 20/60 in the affected eyes. Peripheral retinoschisis was observed in all three patients. All patients were followed for a minimum of 1 year. In one case, progressive macular retinoschisis leading to foveal involvement was observed over two years, with an associated vision decline from 20/25 to 20/60. Attempted interventions included topical dorzolamide in all cases and intravitreal bevacizumab in one patient; however, no treatment effect was observed. The foveal avascular zone size was within normal limits (mean 280 mm). In all stellate nonhereditary idiopathic foveomacular retinoschisis eyes, the retinoschisis cavities were nonvascular.Conclusion: Novel findings regarding stellate nonhereditary idiopathic foveomacular retinoschisis include the progressive nature of foveal involvement and the lack of response to topical dorzolamide and intravitreal bevacizumab. Foveal avascular zone was normal in all eyes, consistent with the relatively preserved vision in these cases. Retinoschisis cavities were nonvascular in all eyes, a finding which may give insight into the mechanism of this disease.

Stellate nonhereditary idiopathic foveomacular retinoschisis and an approach to the differential diagnosis of macular star

“…In one case report, a patient showed total resolution of visual symptoms and SNIFR-related changes on OCT after 1 year of topical dorzolamide [47]. However, a later case series of three patients with SNIFR showed no change after treatment with dorzolamide [8]. At the present time, there is insufficient evidence to support the routine use of dorzolamide in patients with SNIFR.…”

“…In the rare cases of vision worse than 20/50, other factors such as subretinal fluid or exudative maculopathy are usually seen [1,7]. SNIFR can present unilaterally or bilaterally, but the preponderance of cases appears to be unilateral [1,2 ▪▪ ,8]. On fundus examination, the foveal reflex may be blunted with apparent elevation and foveal cysts in a stellate configuration (Fig.…”

Bilateral Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis (SNIFR) Incidentally Identified in a Non-Myopic Female

“…Nonetheless, significant deterioration of visual acuity and the development of subretinal fluid indicate the need for therapeutic intervention [ 10 ]. While vitrectomy with tamponade and internal limiting membrane peeling is the therapeutic intervention of choice, more conservative options such as the administration of topical dorzolamide and intravitreal injection of bevacizumab with both controversial results have been reported in the literature [ 1 , 3 , 11 , 12 ]. Ajlan RS et al described a unique case of SNIFR treated successfully with topical dorzolamide hydrochloride 2% [ 13 ].…”

Middle-Inner Macular Layers Dysfunction in a Case of Stellate Foveomacular Retinoschisis Detected by Abnormal Multifocal Photopic Negative Response Recordings

“…The diagnosis is based on the typical appearance of the fovea presenting with the characteristic radial cartwheel pattern and with the splitting of the retinal layers at the level of the Henle fiber layer (HFL) and the outer plexiform layer (OPL) seen by Spectral Domain-Optical Coherence Tomography (SD-OCT). At the present, the SNIFR condition has been studied by multimodal imaging combining together SD-OCT [ 1 , 2 ], OCT angiography (OCTA), [ 3 , 4 , 5 ] fluorescin angiography (FA), [ 1 ] and multifocal electroretinogram (mfERG) [ 4 ]. However, there is a lack of information about the function of cellular elements located in middle-inner retinal layers, where the retinoschisis resides.…”

“…Although accurate multimodal imaging of retinas has been already performed by previous authors in SNIFR [ 1 , 2 , 3 , 4 , 5 ], there is a lack of information about the functional assessment of the middle-inner retina, involved in the structural damage. We thought the application of the novel technique of mfPhNR recordings that records mainly the function of retinal ganglion cells and their axons [ 12 ] from localized areas valuable.…”