Degenerative Retinoschisis with Concurrent Outer Layer Holes: A Scoping Review and Case Series

PURPOSE: To characterize the natural history, clinical spectrum, and management of degenerative retinoschisis (DRS) with outer layer holes (OLHs). METHODS: Retrospective case-based review combining longitudinal multimodal imaging with a literature review. RESULTS: We followed 10 eyes from 9 patients with DRS and OLH with dynamic changes longitudinally (average 3.8 years, range 0.5-7.7 years) using serial ultra-widefield fundus imaging. Three (30%) eyes had an OLH at presentation, whereas the other 7 (70%) developed de novo OLHs between 5 months and 3.7 years after initial presentation. Of the 7 eyes that developed a new OLH, the DRS configuration remained stable in 2 eyes, enlarged in 2 eyes, flattened with clinical disappearance of the OLH in 1 eye, initially improved, then developed a combined schisis-rhegmatogenous retinal detachment (RRD) requiring vitrectomy in 1 eye, and improved with the development of a localized combined schisis-RRD requiring laser demarcation in the final eye. Of the 3 eyes with baseline OLHs, the DRS configuration remained stable but developed a new inner-layer hole at 3 years in 1 eye, completely flattened 1.5 years after presentation in another, and collapsed 7.7 years after presentation in the third eye. CONCLUSIONS: DRS with OLHs demonstrates a highly variable natural history ranging from spontaneous regression to progression requiring surgical intervention. Patients should be followed with serial multimodal imaging, reserving treatment solely for patients with combined schisis-rhegmatogenous retinal detachment. Detailed documentation with ultra-widefield fundus photography and OCT through the schisis cavity helps monitor retinoschisis anatomy to guide management decisions.