Retinoschisis in Coats Disease: Clinical Picture, Therapeutic Considerations, and Management Outcomes

Purpose: Retinoschisis in inflammatory and exudative retinopathy is a known but rare entity. We describe the presentation and clinical and surgical outcomes of a series of patients with retinoschisis in the setting of Coats disease. Methods: This retrospective case series of patients with Coats disease recorded visual acuity and the number and type of treatments (eg, angiography-guided photocoagulation, intravitreal bevacizumab, sub-Tenon triamcinolone, or vitreoretinal surgery). Results: Eighteen of 133 patients with Coats disease were diagnosed with retinoschisis by color imaging. All (100%) of the areas of schisis or macrocyst were associated with dense subretinal exudation, whereas only 7 (38.8%) were associated with exudative retinal detachment. Initial best-corrected visual acuity in the schisis cohort was 1.14 ± 1.19 (Snellen equivalent, 20/276) vs 1.42 ± 1.40 (Snellen, 20/526) in those without ( P = .56). Final best-corrected visual acuity was 1.76 ± 1.37 (Snellen, 20/1150) and 1.45 ± 1.44 (Snellen, 20/563), respectively ( P = .43). The mean number of treatments in individuals with schisis was 4.8 ± 2.9 for angiography-guided photocoagulation, 4.5 ± 2.9 for intravitreal bevacizumab, and 1.7±1.2 for sub-Tenon triamcinolone. Intraocular surgery was required in 39% (7 of 18) of patients with schisis vs 22% (25 of 115) for patients without schisis ( P = .14). One eye required enucleation, and histopathology of the schisis pocket was obtained. Conclusions: The pathophysiology of retinoschisis in Coats disease is not completely understood. We propose that telangiectatic leakage, hypoxia, and toxicity to the retinal pigment epithelial cells play a role in cavity development, and therefore may represent a clinical feature of advanced or uncontrolled disease.