Multimodal imaging of stellate nonhereditary idiopathic foveomacular retinoschisis with midperipheral retinoschisis and microvasculopathy.

PURPOSE: To describe the clinical and multimodal imaging features of a large stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) associated with midperipheral retinoschisis (MPRS) and microvascular abnormalities.

METHODS

A single patient with no prior ocular pathology underwent spectral-domain OCT, ultra-widefield swept-source OCT with OCT-angiography (OCT-A), fluorescein angiography, ultra-widefield fundus photography, adaptive optics flood-illumination ophthalmoscopy (AO-FIO), and full-field electroretinography (ERG).

RESULTS

Extensive schisis involved the henle fiber layer (HFL) in the macula and multiple retinal layers in the midperiphery. The posterior vitreous remained largely attached, without signs of pathological traction. OCT-A revealed abnormal microvasculature outside the vascular arcades without non-perfusion. Fluorescein angiography confirmed the absence of areas of non-perfusion and showed leakage from the abnormal vessels, which were confined to areas with significant inner retinal schisis. These vessels were located between the retinal nerve fiber layer and the inner limiting membrane, displaying neovascular morphological characteristics. ERG was electronegative, and genetic testing was unremarkable. AO-FIO identified hyperreflective foveal dots in the inner retina. No therapeutic intervention was initiated and the condition remained stable over more than one year of follow-up.

CONCLUSION

Extensive SNIFR with inner retinal schisis may be associated with neovascular-like changes without areas of non-perfusion on fluorescein angiography or OCT-A and can present with an electronegative ERG.