Background and Objective: The purpose of this study was to assess the association between patient age and two high-risk molecular features in uveal melanoma (UM): class 2 gene expression profile (GEP) and positive preferentially expressed antigen in melanoma (PRAME) expression status. Patients and Methods: This retrospective study included 481 UM patients who underwent molecular profiling. Multivariate logistic regression evaluated associations between GEP class 2 or positive PRAME expression status and demographic and clinical features. Nonlinear effects of age on GEP class were assessed using a 3-knot restricted cubic spline. A multivariable model using age cohorts was employed to examine GEP class 2 risk across clinically meaningful age groups. Results: Multivariate analysis confirmed that older age is associated with GEP class 2 profile. The nonlinear component of the spline model reached borderline significance ( P = .07) and the spline model demonstrated a superior fit compared to standard multivariable linear regression, identifying a transition in GEP class 2 risk from a plateau to an escalation starting around the sixth decade of life. Multivariable age cohort analysis revealed that patients aged 70 to 79 years had a 2.5-fold increase in the odds of a class 2 tumor, while patients ≥ 80 years of age experienced a 3.6-fold increase compared to patients < 50 years of age. Tumor largest basal diameter and ciliary body involvement were also associated with GEP class and PRAME expression. Both linear and nonlinear models revealed no significant association between age and PRAME expression. Conclusions: Older age is strongly associated with GEP class 2 status, with risk escalating in later life, suggesting that advanced age, particularly greater than 60 to 70 years, should prompt a high index of suspicion in the management of uveal nevi or melanoma.
Background and Objective: The purpose of this study was to evaluate a simple ring-based visual field screening test to determine whether, when used at home, the findings were similar to clinical evaluation in terms of visual field defects associated with retinal and optic nerve diseases. Patients and Methods: This was a prospective observational cross-sectional diagnostic accuracy study. Consecutive patients presenting between May 2023 and February 2025 with acute vision loss, known history of retinal or optic nerve disorders, or symptoms of acute posterior vitreous detachment or floaters were enrolled. A total of 594 patients (667 eyes) were included. With the fellow eye occluded, patients viewed a 360° ring target and reported any missing or distorted segments. Ring-test findings were compared with clinical reference standards including dilated fundus examination, fundus photography, and Humphrey Visual Field (HVF) testing. Concordance between ring test defects and HVF defects was assessed based on location and orientation. Results: Of a total of 667 eyes, 240 eyes underwent HVF testing. Among 88 eyes with HVF defects, the ring test identified 80 (sensitivity, 0.91; 95% CI, 0.83–0.96). Among 152 eyes without HVF defects, 149 were correctly classified (specificity, 0.98; 95% CI, 0.94–1.00). Ring test findings demonstrated strong spatial concordance with clinical pathology. Conclusions: Concordance between ring test defects and HVF defects was high, with strong sensitivity and specificity for detecting mid-to-peripheral visual field defects in rhegmatogenous retinal detachment, retinal vascular occlusions, and certain optic neuropathies. Preliminary results of this initial proof-of-concept study suggest that the ring test has potential utility as a home-based screening tool to facilitate earlier awareness of vision-threatening conditions. Further studies are needed to evaluate its role in routine clinical practice.
This multicenter case series describes severe rotisserie tilt of scleral-fixed CT Lucia 602 intraocular lenses (IOL) observed across multiple surgeons and centers between 2021 to 2023. The study reviewed 1,408 intrascleral haptic fixation (ISHF) surgeries performed by 20 surgeons from 18 centers, including both retina and anterior segment specialists, at both academic and private practice settings, and identified 45 tilted IOL cases with the CT Lucia requiring surgical intervention. There was a wide variation in instrumentation, tunnel construction approaches, and lens insertion techniques. The average estimated tilt was 74.8º ± 40.1º. Abnormalities in the haptic-optic junction were found in 34 of 45 (76%) cases. Management included replacing the IOL, placing an anterior chamber IOL, refixating the IOL, or leaving the patient aphakic. Of those receiving another CT Lucia lens, seven of 16 (44%) cases experienced repeat tilt, representing a meaningful risk of recurrence. Given the heterogeneity of surgeon technique, our findings may represent a concerning reproducible failure phenotype.
Background and Objective: The purpose of this study was to evaluate the prevalence, spatial distribution, and multimodal imaging features of white without pressure (WWOP) and dark without pressure (DWOP) associated with congenital hypertrophy of the retinal pigment epithelium (CHRPE). Patients and Methods: This retrospective study included 101 patients (104 eyes; 106 lesions) with clinically diagnosed CHRPE evaluated between 2010 and 2025. Ultra-widefield fundus photography and optical coherence tomography (OCT) were reviewed for WWOP and DWOP prevalence, adjacency to CHRPE, and outer retinal changes. Results: WWOP was identified in 24 eyes (23%) and DWOP in 19 eyes (18%); four eyes (4%) exhibited both. WWOP was lesion-adjacent in 42% of cases, and DWOP was adjacent in 79% of cases. DWOP was associated with progressive ellipsoid-zone attenuation beyond the CHRPE margin on OCT. WWOP typically spanned a broader retinal area. Conclusion: WWOP and DWOP are relatively common in CHRPE and demonstrate distinct spatial and OCT features. Multimodal imaging may aid differentiation of CHRPE from other pigmented lesions.
