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

Abstract

Purpose: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Figure 1. Images from subjects 6 (OD), 12 (OS), 14 (OS), 19 (OS) and 24 (OS). Foveomacular retinoschisis is demonstrated, on macular OCT (A-E), involving HFL and extending peripherally. There is evidence of incomplete posterior vitreous separation (arrows).

Figure 2

Images from subject 1 (OS). Optos widefield SLO imaging (A&B) reveals microcystoid changes in the temporal peripheral retina. Widefield composite OCT (C) demonstrates continuity between the central foveomacular schisis and peripheral retinoschisis, with incomplete posterior vitreous separation (D). En face projection of the mid-retina (E) shows the ‘spoke-wheel’ distribution of the schisis cavity. Microperimetry (F) is normal, with evidence of scotoma in the nasal peripheral visual field (corresponding to the temporal retinal changes) on 60-4 static perimetry (G).

Figure 3. Images from subject 5 (OU). Widefield OCT (A&C) reveals a transition from HFL to the INL, with persistent attachment of the posterior hyaloid (arrows). En face projection of the mid retina demonstrates the ‘spoke-wheel’ distribution of the schisis cavity, extending temporally (B&D).

Figure 4

Images from subjects 2 (OS) and 4 (OU). Optos widefield SLO imaging (A,F,K) demonstrates peripheral microcystoid changes (white arrow heads). Widefield OCT composites (B,G,L) reveal continuity with peripheral retinoschisis (B&J) and schisis-detachment (F, arrow); asterisks denote mirror artefacts on OCT. En face projections of the mid-retina demonstrating the ‘spoke-wheel’ distribution of the schisis, extending peripherally, where it takes on a ‘speckled’ appearance (C,H,M). Microperimetry (D,I,N) is normal, while 60-4 static perimetry (E,J,O) shows loss of sensitivity in the nasal visual field.

Figure 5. Images from subject 8 (OD). Serial macular OCT (A&B) demonstrating partial resolution of foveomacular retinoschisis following spontaneous posterior vitreous detachment.