Foveomacular Retinoschisis Research Articles

IS CATARACT SURGERY SAFE IN PATIENTS WITH STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHISIS?

To assess the retinal modifications after cataract surgery in a patient presenting with stellate nonhereditary idiopathic foveomacular retinoschisis. Retrospective analysis of the evolution of retinal status in a 64-year-old patient with unilateral stellate nonhereditary idiopathic foveomacular retinoschisis who underwent cataract surgery in the affected eye. Visual acuity improved after surgery, reaching back the level described by the patient before the cataract onset, whereas fundus retinographies and optical coherence tomographies were stable at one month after surgery, compared with the preoperative images. This first case-report of the evolution of stellate nonhereditary idiopathic foveomacular retinoschisis after cataract surgery suggests that this retinal condition is not modified by the surgical procedure, which however needs to confirmed by larger cohorts.

Stellate nonheritable idiopathic foveomacular retinoschisis in juveniles: case report

BackgroundStellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) is a rare type of retinoschisis with a spoke-like splitting that occurs in the outer plexus layer. We present a case of stellate nonhereditary idiopathic foveomacular retinoschisis in a juvenile, in which two eyes show different development trends and macular retinoschisis could be associated with mechanical force in the Henle fibre layer. The removal of mechanical force can partially restore vision.Case presentationA 14-year-old girl with bilateral SNIFR was diagnosed and followed up with spectral-domain optical coherence tomography (SD-OCT). During the two follow-up visits, vitreous adhesion was released in the left eye, and visual acuity improved. Neuroepithelial detachment was aggravated in the right eye, and visual acuity decreased. Therefore, vitrectomy was performed on the right eye. After surgery, the patient’s retina was reattached, and her vision was partially restored.ConclusionsWe reported a juvenile with bilateral SNIFR. Each of her eyes showed different development trends, so we adopted different treatment methods for each eye. Vitrectomy was performed on the patient to address progressive vision loss, which improved the patient’s vision. It was further confirmed that the Henle layer of SNIFR patients was susceptible to posterior vitreous membrane adhesion.

STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHISIS: NOVEL FINDINGS AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY ANALYSIS.

To present novel findings in stellate nonhereditary idiopathic foveomacular retinoschisis, including the largest series of optical coherence tomography angiography findings to date. A retrospective case series with multimodal imaging was obtained and reviewed. 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 µ m). In all stellate nonhereditary idiopathic foveomacular retinoschisis eyes, the retinoschisis cavities were nonvascular. 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.

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

We describe the macular morpho-functional assessment of a 65-year-old man affected by stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR), studied by visual field, SD-OCT, autofluorescence, full-field electroretinogram (ffERG), multifocal electroretinogram (mfERG) and multifocal Photopic Negative Response (mfPhNR) recordings. The typical presentation consists of the foveal appearance of radial cartwheel pattern for the splitting of the retinal layers at the level of the Henle fiber layer (HFL) and the outer plexiform layer (OPL), perfectly seen by Spectral Domain-Optical Coherence Tomography (SD-OCT). Despite a normal function of the outer retina of the peripheral and central retina evaluated by ffERG and mfERG respectively, we observed a reduced function of the retinal elements involved in the retinoschisis by recording mfPhNR that assesses mainly inner retina function (retinal ganglion cells and their axons). Therefore, it is likely that the observed impaired mfPhNR responses reflect the signaling defects derived from the delaminated middle retina and transmitted to the innermost retinal layers.

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

This review aims to introduce stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) and its differential diagnosis. We summarize findings from case reports and series published in the last few years on the clinical and imaging findings in SNIFR. SNIFR presents as either a unilateral or bilateral macular star on fundus examination without clinical or imaging evidence of exudation or frank vitreomacular traction. optical coherence tomography (OCT) imaging shows schisis cavities in the Henle fibre and outer plexiform layers that correspond to the stellate en face findings. Visual acuity is usually minimally affected, and the presence of significant vision loss should prompt high clinical suspicion for alternate diagnoses. SNIFR is a recently characterized clinical entity that serves as an important addition to the differential diagnosis of a macular star. It is a diagnosis of exclusion and should be distinguished from other causes of macular star such as neuroretinitis, vitreomacular traction, ocular manifestations of malignant hypertension, congenital juvenile X-linked macular schisis, myopic maculopathy, optic pit maculopathy, nicotinic acid maculopathy or taxane maculopathy among others.

AN ASSOCIATION BETWEEN STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHISIS, PERIPHERAL RETINOSCHISIS, AND POSTERIOR HYALOID ATTACHMENT.

Stellate nonhereditary idiopathic foveomacular retinoschisis is a disorder characterized by splitting of the retina at the macula, without a known underlying mechanical or inherited cause. This study investigates demographic, anatomical, and functional characteristics of subjects with stellate nonhereditary idiopathic foveomacular retinoschisis, to explore potential underlying mechanisms. In this single-site, retrospective, and cross-sectional, observational study, data were collected from 28 eyes from 24 subjects with stellate nonhereditary idiopathic foveomacular retinoschisis. Descriptive statistics were reported, based on the observed anatomico-functional features. The visual acuity remained stable (median 20/20) in all subjects over a median follow-up of 17 months. All cases demonstrated foveomacular retinoschisis within Henle's fiber layer, at the junction of the outer plexiform and outer nuclear layers. This schisis cavity extended beyond the limits of the macular OCT temporally in all eyes. In most affected eyes, there were documented features of peripheral retinoschisis and broad attachment of the posterior hyaloid at the macula. Functional testing in a cross-sectional subset demonstrated normal retinal sensitivity centrally but an absolute scotoma peripherally. Stellate nonhereditary idiopathic foveomacular retinoschisis seems to be associated with peripheral retinoschisis and anomalous or incomplete posterior hyaloid detachment. Despite chronic manifestation, this does not significantly affect central visual function but can manifest with profound loss of peripheral visual function.

Stellate nonhereditary idiopathic foveomacular retinoschisis treated as cystoid macular edema

Stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) is a rare eye disease, hitherto unreported in Indians. We report a case of SNIFR in an Indian female wrongly treated as cystoid macular edema (CME). Differentiation between chronic CME and foveoschisis is paramount while dealing with rare diseases like SNIFR.

RAPID PROGRESSION OF FOVEOMACULAR RETINOSCHISIS IN YOUNG MYOPICS.

To describe a foveomacular retinoschisis that has not been described. Patients with foveomacular retinoschisis were included. Exclusion criteria included refractive error over -6.00 diopters, presence of posterior staphyloma, positive RS1-gene mutation, family history of retinoschisis, optic disk abnormalities, or glaucoma. Vitrectomy was performed on eyes with functional or structural deterioration. Seventeen eyes from 10 patients (15-30 years old, 8 females and 2 males) with foveoschisis were recruited, with bilateral involvement in 7 patients and unilateral in 3 patients. Vitrectomy was performed in 13 eyes (13/17, 76.5%). Seven eyes (6 patients) were operated soon after the first presentation because of poor vision and severe foveoschisis. Six eyes (6 patients) were operated 2 weeks to 13 months later because of deterioration of vision and foveoschisis. Preoperative vision was 20/134 ± 20/165, and postoperative vision was 20/25 ± 20/57, with visual improvement of 6.9 (4-14) lines. The mean postoperative follow-up period was 36.5 (15-69) months. Four eyes (4 patients) were asymptomatic, despite progression of foveoschisis. Three eyes (3 patients) maintained normal macula structures. We report a foveomacular retinoschisis characterized by young age of onset, female predominant, no highly myopia, mostly bilateral involvement, and rapid progression of foveoschisis and visual acuity. Vitrectomy is effective in restoring anatomical structure and stabilize vision.

Stellate nonhereditary idiopathic foveomacular retinoschisis concomitant to exudative maculopathies

PurposeTo report the clinical course of patients presenting with stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) concomitant with exudative maculopathies.MethodsRetrospective case series. Multimodal imaging findings, including spectral-domain optical coherence tomography (SD-OCT) were reviewed. Genetic testing for the RS1 gene was performed in one patient.ResultsWe identified two female patients who fit the definition of SNIFR and presented with concomitant neovascular age-related macular degeneration (n-AMD). In both the patients, SD-OCT showed exudative macular features and splitting (bilateral in patient 1, unilateral in patient 2) of the outer plexiform layer (OPL) in the macula with no other evidence of hereditary or an acquired predisposing condition. Genetic testing excluded mutation of RS1 gene in patient 1. The fundi of both the patients showed characteristic signs of active choroidal neovascularization (CNV) and following anti-VEGF treatment, visual acuity improved and CNV-related exudative changes resolved. However, the split along the OPL remained unaltered.ConclusionsSNIFR may be associated with n-AMD. It is important to recognise the presence of retinoschisis when there is other exudative pathology as the former may be misinterpreted as intraretinal fluid, prompting unnecessary treatment.

Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis

To describe a new classification of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR). Retrospective case series and literature review. A total of 17 patients from 5 institutions. Detailed case history, multimodal imaging, and genetic testing were reviewed for patients with macular schisis without a known predisposing condition. Patients with a stellate appearance centered on the fovea with correlating confirmed expansion of the outer plexiform layer (OPL) by optical coherence tomography (OCT) were included. Exclusion criteria included a family history of macular retinoschisis, a known genetic abnormality associated with retinoschisis, myopic traction maculopathy, epiretinal membrane, vitreoretinal traction, optic or scleral pit, or advanced glaucomatous optic nerve changes. Clinical features, anatomic characteristics, and visual acuity. A total of 22 eyes from 16 female patients and 1 male patient with foveomacular schisis were reviewed from 5 institutions. Initial visual acuity was ≥ 20/50 in all eyes (mean, 20/27), but visual acuity in a single eye decreased from 20/20 to 20/200 after the development of subfoveal fluid. The refractive status was myopic in 16 eyes, plano in 3 eyes, and hyperopic in 2 eyes. Three eyes had a preexisting vitreous separation, and 19 eyes had an attached posterior hyaloid. Follow-up ranged from 6 months to >5 years. In this largest known series of patients with SNIFR, all patients demonstrated splitting of the OPL in the macula with relatively preserved visual acuity (≥ 20/40) except in a single patient in whom subretinal fluid developed under the fovea.

Macular spectral-domain optical coherence tomography in patients with X linked retinoschisis

Aim:To evaluate macular anatomy in patients with X linked retinoschisis (XLRS) using spectral-domain optical coherence tomography (SD-OCT).Methods:Consecutive observational case series. Clinical features were obtained through retrospective chart review. Only eyes...