Stellate Unilateral Nonhereditary Idiopathic Foveomacular Retinoschisis: A Multimodal Imaging Analysis and Case Report

Abstract

Purpose: To describe a case of stellate nonhereditary idiopathic foveomacular retinoschisis in a middle-aged woman and to depict the classic retinal fluorangiography (FA) findings, structural characteristics using macular spectral-domain optical coherence tomography angiographic data of vascular and perfusion density using optical coherence tomography angiography (OCT-A), and standardized multifocal electroretinography (mfERG) findings.Methods: This is a case report of a 53-year-old ophthalmologist who was incidentally diagnosed with unilateral idiopathic foveomacular retinoschisis. Stellate nonhereditary idiopathic foveomacular retinoschisis is defined as a foveal elevation without alternative explanation for retinoschisis. FA, spectral-domain optical coherence tomography, optical coherence tomography angiography, and multifocal electroretinography were used as tools to obtain an integral multimodal diagnosis of this entity.Results: Clinical examination and multimodal imaging were able to detect unilateral idiopathic retinoschisis, revealing a stellate pattern of retinal concentric cysts with minimal changes in vascular and perfusion density metrics and confirming the absence of bridging vessels. There were consistent FA findings, with almost unaltered foveal changes. Multifocal electroretinography depicted a subtle reduction in dark-adapted a-wave and b-wave amplitudes.Conclusion: Improvements and innovations in technology for ophthalmic diagnosis have revolutionized our capacity for diagnostic decision-making. Spectral-domain optical coherence tomography and optical coherence tomography angiography are useful tools for diagnosis and follow-up assessment. This fortuitous case gives a window on the importance of a routine specialized ophthalmic examination and how multimodal imaging can depict important and specific findings not evident from a clinical point of view. The subtle but important changes observed in optical coherence tomography angiography and multifocal electroretinography will help better define this clinical entity.

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

“…Vascular densities of SNIFR eyes were similar to the nonaffected fellow eye of Case 1, pointing to a preserved vasculature despite the distorted anatomy. A case by Montano et al 9 also showed OCT-A in a SNIFR patient with similar results to our three cases. The acircularity of the FAZ was normal, and the vessel density also seemed normal.…”

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

“…On the contrary, the OCTA of the patient with SNIFR did not visualize blood flow in the avascular Henle’s fiber layer [ 7 ]. Given the challenge of detecting these conditions through clinical examination alone, Montano M et al emphasized the crucial role of OCT and OCTA in illustrating the novel findings of SNIFR [ 8 ]. In fact, in our case, the OCT set the suspicion of the diagnosis, indicating the role of multimodal imaging in routine clinical practice.…”

Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis: Cataract Surgery

“…A stellate macular retinoschisis pattern in an RS1-negative patient in an eye without any other anatomical or functional defects is rather rare and can be classified as SNIF. Typically, this disease category represents stellate retinoschisis patterns in the foveomacular regions in an RS1-negative patient without a known family history of retinal diseases [2][3][4][5]. SNIF is a rather new diagnostic entity, with few case reports.…”