Purpose: To report early safety and efficacy of Descemet stripping only (DSO) supplemented with ripasudil. Methods: A pre-post clinical trial with a historical control group for time to heal and cell count parameters. The study received ethics approval and was conducted with oversight of a data safety monitoring board. All enrolled patients had a superior endothelial cell count of >1000 cells/mm2 and were symptomatic from the presence of central guttata degrading vision and/or producing glare. DSO was carried out with a peeling technique and not combined with any other intervention. Ripasudil 0.4% was applied topically from day 1 postoperatively at a dose of 6 times/d until corneal clearance. Cases with relapse of edema were permitted to restart on ripasudil at a reduced dose of 2 drops/d for a further 2 weeks. Stopping rules with progression to a corneal graft were established. Baseline ocular and systemic investigations were carried out and repeated at varying intervals to monitor for local and systemic adverse events. Results: Twenty-three eyes of 23 patients met the inclusion criteria and underwent DSO. Twenty-two of 23 eyes achieved corneal clearance at a mean time of 4.1 weeks. In all patients achieving clearance, improvement in vision was recorded. Improvement in mean uncorrected visual acuity was 0.20 Logarithm of the minimum angle of resolution (LogMar), and improvement in mean best spectacle corrected visual acuity was 0.156 LogMar. One patient failed to clear and underwent Descemet membrane endothelial keratoplasty at week 12. Twenty-one of 22 patients achieving corneal clearance expressed satisfaction with the procedure. The commonest systemic side effect of topical ripasudil was gastrointestinal upset (24%), and the commonest local side effect was ocular irritation (43%). No patient experienced a serious adverse event in the course of the trial. Thirty-nine percent of patients experienced a relapse of edema on ceasing ripasudil, with clearance again on recommencing. Conclusions: This trial of DSO supplemented with ripasudil included local and systemic safety analysis. We judge that this treatment option is emerging as a reliable intervention for select patients with Fuchs' Endothelial Corneal Dystrophy (FECD) with an acceptable safety profile. The observation of relapse edema is strong evidence of a drug effect. The longevity of these results remains unknown.

Purpose:The purpose of this study was to report the medium-term outcome of our index case of Descemet stripping only (DSO) in the clinical setting of Fuchs endothelial corneal dystrophy with pancorneal guttae.Methods:This was a retrospective case report.Results:A 44-year-old woman with bilateral Fuchs endothelial corneal dystrophy was referred for consideration of DSO. At initial slit-lamp examination, widespread guttae were observed with no clear zone visible. Confocal microscopic examination also failed to isolate a population of undisturbed endothelial cells. DSO with supplemental ripasudil was performed with corneal clearance achieved at 2.5 months. A stable result was obtained for 18 months with a subsequent slow decline in vision and return of diurnal fluctuation. At 3.5 years after DSO, DMEK was performed with resolution of symptoms.Conclusions:Medium-term failure in this clinical setting is further evidence that DSO is likely best offered to patients with central guttae but a clear corneal periphery, indicative of a healthy cell reservoir.

Purpose The purpose of this study was to investigate cone function deterioration in patients with retinitis pigmentosa (RP) using full field electroretinogram (ffERG), pattern electroretinogram (pERG), and optical coherence tomography (OCT) and their correlation with visual acuity (VA). Methods Clinical records (2008–2018) of patients with RP undergoing repeat electrophysiology were reviewed. Results of ffERG (30 Hz flicker and fused flicker amplitude [FFAmp]), pERG [p50 and n95], and macular OCT (ellipsoid zone [EZ] and outer segment thickness) were collected. Results One hundred twenty-six eyes from 63 patients (33 women, mean age 35 years) were included. The mean decline in VA was 0.11 ± 0.14 logarithm of minimum angle of resolution (logMAR). The FFAmp decreased by 3.01 ± 5.9 µV with global cone function deteriorating by 18.7% annually. The percentage change in FFAmp (RE [ r = 0.553], LE [ r = 0.531]), and 30 Hz flicker amplitude (RE [ r = 0.615], LE [ r = 0.529]) strongly correlated with VA ( P < 0.00001). The pERG p50 (15 and 30 degrees) change analyzed in 34 patients showed reduction by 23% and 23.4%, respectively. The percentage change in p50 30 degrees ( r = 0.397) correlated with VA and EZ layer ( P < 0.05). The EZ layer change was calculated in 45 patients and the shortening and thinning rate was 4.3% and 4.4% annually, respectively. The EZ length percentage change correlated with VA (RE [ r = 0.34] and LE [ r = 0.466; P < 0.05). Conclusions We quantified the decline in cone function in patients with RP utilizing ffERG and FFAmp measures of residual cone function. These parameters correlated with VA and OCT when measurable. These objective measures may assist in monitoring disease progression. Translational Relevance Residual cone function provides an objective estimate of residual visual function, which aids in counselling patients regarding prognosis.

was a previous consultant of Kowa India Private Limited, manufacturers of Ripasudil; the other authors have no conflicts of interest to disclose. Use of ripasudil for corneal hydrops is an off-label use of the medication.