Self-reported visual function and psychosocial impact of visual loss in EYS-associated retinal degeneration in a Portuguese population.

PURPOSE: To evaluate self-reported visual function and the psychosocial impact of visual loss EYS-associated retinal degeneration (EYS-RD) using two patient-reported outcome (PRO) measures: Michigan Retinal Degeneration Questionnaire (MRDQ) and Michigan Vision-related Anxiety Questionnaire (MVAQ). METHODS: Cross-sectional, single-center study conducted at a tertiary care hospital in Portugal. Patients with biallelic EYS variants were invited to participate. Clinical data including demographics, ETDRS best-corrected visual acuity (BCVA) in the better-seeing eye and genetic testing results were collected. Interviews were carried out during clinic visits or by phone between November 2021 and February 2022. A blind grader used horizontal and vertical spectral domain optical coherence tomography (SD-OCT) scans to manually measure ellipsoid zone (EZ) width in the nasal, temporal, superior and inferior macular quadrants to calculate the EZ area. RESULTS: Forty-nine patients (53.1% males; mean age 53 ± 14 years) were included. A positive correlation (p < .05) was found between age and most MRDQ domain scores (central vision, color vision, contrast sensitivity, scotopic function, photopic peripheral vision and mesopic peripheral vision). A negative correlation was found between both BCVA and EZ area across all MRDQ domains. In MVAQ, SD-OCT EZ area negatively correlated with both rod function and cone function-related anxiety. Neither age, BCVA or gender correlated with MVAQ domains. CONCLUSIONS: This study provides strong evidence supporting a correlation between PRO measures and both functional and structural clinician-reported outcomes. The use of MRDQ and MVAQ adds a new dimension to our understanding of EYS-RD and establishes both PRO measures as important disease outcome measures.

Modified Deep Anterior Lamellar Keratoplasty Technique to Rescue Failed Penetrating Keratoplasty.

Purpose: To describe a modified deep anterior lamellar keratoplasty (DALK) technique to rescue failed penetrating keratoplasty (PK) grafts and report its results. Patients and Methods: Retrospective, case-series analysis of patients who underwent modified DALK to rescue failed corneal grafts after PK was included. Every patient had corneal graft failure (GF) diagnosis due to anterior corneal (epithelium, Bowman's, and stroma layers) or refraction disorders uncorrectable with conservative management. The main objective of the surgery and primary outcome of the study was the improvement of corrected distance visual acuity (CDVA). The study's secondary outcome was the frequency of intra- and postoperative complications. Results: This series included four eyes from three patients with a mean age of 57.7 years (range: 51-63 years). Mean follow-up of patients was thirteen months (range: 12-15 months). During follow-up, continuous improvement of corneal transparency and CDVA was observed in every patient. The only intraoperative complication reported was a peripheric DM microperforations of previous graft-host junction in two eyes. No signs of double anterior chamber, endothelial failure, GF, glaucoma, or other side effects were reported. Conclusion: Our results suggest that modified DALK in patients with GF after PK, preserving a healthy endothelium, is surgically feasible. However, further studies are needed to compare this procedure to PK and other DALK technique variations.