A case of bilateral stellate nonhereditary idiopathic foveomacular retinoschisis with 14-month follow-up: clinical features, OCT findings and treatment outcome.

Abstract

Background: Stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) is a relatively recent and rare classification introduced. Currently, there is no reliable treatment for the disease.

Case presentation: We discussed an additional case multimodal imaging including Optical coherence tomography (OCT), fluorescein angiography and Optical coherence tomography angiography (OCTA) as well as treatment result. The case was a healthy, non-myopic woman, where foveal cystic changes persisted despite 9 months of topical dorzolamide and an additional 5 months of oral acetazolamide. Genetic testing for Congenital X-linked retinoschisis (CXLR) was negative. ERG results were near normal. Optical coherence tomography showed no vitreomacular traction, while fluorescein angiography ruled out vascular disease.

Conclusions: Our findings suggest that bilateral SNIFR can occur in non-myopic females, although this patient did not respond to systemic and topical carbonic anhydrase inhibitors.