Effectiveness of Ripasudil, a Rho-Associated Coiled/Coil-Containing Protein Kinase Inhibitor, in Improving Retinoschisis and Cystic-Like Foveal Cavities in Eyes with X-Linked Retinoschisis

Abstract

This is the first reported case of a successful resolution of cystic-like foveal cavities in eyes with X-linked juvenile retinoschisis (XLRS) treated with topical ripasudil hydrochloride hydrate, a Rho-associated coiled/coil-containing protein kinase (ROCK) inhibitor. A chart review was performed on 1 patient to collect all relevant clinical information and the optical coherence tomographic (OCT) images. A healthy 18-year-old young man presented with bilateral visual disturbances. The patient was diagnosed with XLRS from the spoke-wheel pattern around the macula, negative electroretinograms, and retinoschisis with cystic-like foveal cavities in the OCT images. Significant reductions of the retinoschisis and cystic-like cavities were observed after treatment with topical ripasudil. This is the first case of XLRS that had a resolution of cystic-like foveal cavities after topical ripasudil, a ROCK inhibitor. Since many XLRS patients have a worsening of their visual acuities due to the progressive nature of retinoschisis and cystic-like foveal cavities, topical ripasudil offers a potential treatment option.

Keywords: Cystic-like foveal cavities; Retinoschisis; Rho-associated coiled/coil-containing protein kinase inhibitor; Ripasudil hydrochloride hydrate; X-linked retinoschisis.

Fig. 1

Results of ophthalmic examinations at the initial visit. Although the color fundus images show no abnormalities in the right eye (a) and left eye (b), spoke-wheel patterns (circle) can be seen around the macular area in the red-free fundus image for the right eye (c) and left eye (d). OCT images show retinoschisis in the inner nuclear layer combined with cystic-like foveal cavities in the right eye (e) and left eye (f). ERGs have a negative-type ERG pattern (g). Arrow: OCT-scanned line shown in e and f. Circle: spoke-like appearance around the macula. Asterisk: cystic-like foveal cavities.

Fig. 2

OCT images during the follow-up period. OCT images for the right eye (a, c, e) and for the left eye (b, d, e) are shown. Four months after beginning the topical ripasudil treatment, a reduction of the cystic-like foveal cavities was observed (a, b). Six months after discontinuing ripasudil, there was a recurrence of the cystic-like foveal cavities (c, d). Twenty months after the re-starting of topical ripasudil treatment, there was a resolution of the retinoschisis and cystic-like foveal cavities (e, f).