Abstract

Purpose: The purpose of this study is to evaluate macular anatomy in patients with X-linked retinoschisis (XLRS) using spectral-domain OCT (SD-OCT).

Methods: Consecutive observational case series. Clinical features were obtained through retrospective chart review. Only eyes without prior surgical interventions and those scanned with SD-OCT were included. The OCT images were analyzed.

Results: Fourteen eyes of 7 males with XLRS scanned with SD-OCT, age 5 to 45 years, were identified. On clinical examination, stellate spoke-like cystic maculopathy was present in 9 eyes, and an atrophic foveal lesion in 5 eyes. SD-OCT revealed cystoid spaces accounting for retinal splitting in the inner nuclear layer in 12 eyes, and outer plexiform layer in two eyes of one patient. A few small cysts, not accounting for the foveal splitting, were seen in the outer nuclear layer in 4 eyes and in the ganglion cell layer and/or nerve fiber layer in 6 eyes.

Conclusions: SD-OCT localized the foveomacular retinoschisis in XLRS to the retinal layers deeper than the nerve fiber layer. In the present study, the foveomacular schisis was seen most frequently in the inner nuclear layer.