Purpose

To report the case of an adult male with X-linked retinoschisis (XLRS) who presented with cystoid macular edema (CME) that responded consistently to treatment with intravitreal steroids.

Observations

A 39 year old male with unilateral presentation of CME after repair of a retinal detachment secondary to XLRS responded initially to an injection of intravitreal triamcinolone acetonide (IVTA). Central subfield thickness on OCT was reduced. Three months later, the CME recurred and he was unresponsive to topical treatment so repeat IVTA was given, and the CME once again was reduced dramatically. After the next recurrence, intravitreal dexamethasone implant treatment was initiated and successful at treating recurrences in 3 month intervals for 5 additional injections. Finally, an intravitreal fluocinolone acetonide implant was surgically placed with control of CME.

Conclusions and importance

Corticosteroids have never been reported to be effective in CME related to XLRS. Here, we document a case of a man who successfully had decrease of intraretinal fluid and schisis with treatment of intravitreal corticosteroids as demonstrated by spectral domain optical coherence tomography.

PURPOSE:: Quantitative end points for uveitis are needed. Here we quantify the rate of ellipsoid zone (EZ) recovery on optical coherence tomography (OCT) and correlate it with visual acuity (VA) improvement in patients with acute posterior multifocal placoid pigmented epitheliopathy (APMPPE). We use automated and manually graded EZ area analysis to assess EZ recovery in APMPPE. METHODS:: We performed a retrospective review of 9 APMPPE cases (18 eyes) that had characteristic clinical examination and fluorescein angiography findings, outer retinal disruption on spectral-domain OCT, and treatment with systemic steroids after an unambiguous laboratory workup. The EZ was delineated using custom software to perform automated analysis and manual grading by 2 independent physicians. Quantitation of EZ changes was performed in ImageJ (National Institutes of Health). EZ maps were compared with equivalent findings from EZ en face OCT segmentation. RESULTS:: The 9 cases in our study were followed for an average of 198 days. Symptomatic improvement occurred in all eyes. VA recovery occurred in 83% of eyes and depended on presenting foveal involvement. Positive slopes of EZ area over time demonstrated recovery. EZ recovery profiles determined by manual and automated software demonstrated high Pearson correlation coefficients (0.78-0.94). Slab en face EZ analysis demonstrated moderate agreement. CONCLUSIONS:: EZ recovery correlates with symptomatic and VA recovery. Automated EZ analysis shows strong agreement with manually graded EZ analysis in APMPPE. EZ recovery in patients with APMPPE provides a biomarker for recovery and may be applied to other diseases affecting the outer retina.