Title

PROGRESSIVE DEVELOPMENT OF STELLATE NONHEREDITARY IDIOPATHIC FOVEOMACULAR RETINOSCHYSIS AFTER VITREOMACULAR TRACTION SPONTANEOUS RELEASE: MULTIMODAL IMAGES CASE REPORT

Introduction

Macular retinoschisis is generally associated with X-Linked Retinoschisis (XLR), however, a new form of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) has recently been described in women, unilaterally and without RS1 gene defect or other secondary causes 1.

Methods

Case report with multimodal images of progressive development of SNIFR after vitreomacular traction (VMT) resolution.

Results

In May 2015, a healthful 56-year-old white woman complained of blurred vision in OD. Ophthalmic exam revealed mild hyperopia, macular hole in OD and VMT in OS. Two months later, OCT showed partial posterior vitreous detachment (PVD) with little cystic formation in inferior fovea in OS (Figure 1). Until May 2019, OS remained asymptomatic (20/25), but OCT showed progression of the macular retinoschisis with increased splits in outer plexiform layer (OPL) and without peripheral retinoschisis. Fluorescein angiography and fundus autofluorescence were normal (Figure 2). Red-free photography and en face OCT evidenced stellate pattern of foveomacular retinoschisis (Figure 3).

Discussion

Structurally, SNIFR differs from XLR as SNIFR primarily affects the OPL 2. It has been described in association with exudative maculopathies 3, macular telangiectasias 4 and vassalva maneuvers 5. The OPL low resistance can also allow previous VMT to leave degenerative retinal splits and these may remain even after PVD 2; 6; 7. In our case, we believe that resolution of VMT may have created microholes in internal limiting membrane and also exerted mechanical stress inducing splits in the OPL. Similar to optic disc PIT maculopathy and glaucoma associated retinoschisis8, we believe that leakage of vitreous fluid through microholes in the ILM allowed enlargement of foveal cysts between 2015 and 2019. This is the first case reported in literature of SNIFR associated with VMT and is also the first showing progressive formation of retinal splits through a 4-year follow-up.

Keywords

Retinoschisis, Maculopathy, Vitreomacular traction

Area

CLINICAL CASE

Authors

Talita Goulart Machado, João Carlos Diniz Arraes, Danielle Ramos Castro, Emanuela Pereira Holanda Silva, Tatiana Azevedo Arraes