UNRAVELED FRINGE-LIKE MARGINS AND BIPHASIC AUTOFLUORESCENCE OF UNILATERAL RETINAL PIGMENT EPITHELIUM DYSGENESIS.

PURPOSE: To illustrate the features of unilateral retinal pigment epithelium dysgenesis (URPED) in an African-American male patient. METHODS: Case report. RESULTS: A 47-year-old asymptomatic African-American man was referred for an atypical subretinal pigmented mass in the left eye. On examination, visual acuity was 20/20 in both eyes. The right eye was unremarkable. The left eye revealed a darkly pigmented grey-black lesion at the level of the RPE with irregular, unraveled fringe-like margins, consistent with URPED. The lesion measured 5 mm in basal dimension and was located 400 µm from the foveola. The dark portion of the lesion was grey-black and demonstrated homogeneous hypoautofluorescence, particularly at the site of grey-white peripheral fringe of fibrous metaplasia. By contrast, there was an additional, subtle lacey arrangement of normal-appearing RPE traversing over the entire lesion demonstrating isoautofluorescence. On fluorescein angiography, the lesion was generally hypofluorescent, particularly in the dark portion of the lesion, but the peripheral fringe of fibrous metaplasia displayed angiographic hyperfluorescent staining, and the subtle lacey normal RPE showed isofluorescence. Optical coherence tomography demonstrated RPE hyperplasia and shallow RPE detachment interspersed with normal-appearing RPE and thinning of outer retina and preservation of the foveola and choroid. CONCLUSION: In this case, URPED demonstrated biphasic autofluorescence implying RPE dysfunction in the hypoautofluorescent area and partial RPE function in the lacey isoautofluorescent region.

Circumscribed Choroidal Hemangioma: Visual Outcome in the Pre-Photodynamic Therapy Era versus Photodynamic Therapy Era in 458 Cases.

PURPOSE: To analyze visual outcomes after treatment of choroidal hemangioma in the pre-photodynamic therapy (PDT) era versus PDT era. DESIGN: Retrospective, comparative case series. PARTICIPANTS: A total of 458 patients with circumscribed choroidal hemangioma. METHODS: Comparison of hemangioma managed in the pre-PDT (1967-2001) era versus PDT (2002-2018) era. MAIN OUTCOME MEASURE: Visual acuity outcome. RESULTS: A total of 458 tumors were treated over this 51-year period. A comparison (pre-PDT [n = 220 cases] vs. PDT [n = 238 cases]) revealed PDT era patients were of older mean age (48.9 vs. 53.8 years, P = 0.002) and were more likely to have systemic hypertension (17.7% vs. 33.8%, P < 0.001), tumor location in the macula (57.4% vs. 67.5%, P = 0.01), subretinal fluid on OCT (33.3% vs. 70.7%, P = 0.01), and greater extent of overlying lipofuscin (P = 0.001). Findings of tumor basal diameter and thickness and fluorescein and indocyanine green angiography were no different in the 2 eras. Treatment (pre-PDT vs. PDT) included argon laser photocoagulation (42.1% vs. 0.4%), PDT (0% vs. 43.8%), transpupillary thermotherapy (0% vs. 0.4%), plaque radiotherapy (7.0% vs. 5.2%), external beam radiotherapy (1.4% vs. 1.3%), enucleation (0.9% vs. 0.4%), and observation (48.6% vs. 47.6%). After treatment, patients in the PDT era demonstrated better mean logarithm of the minimum angle of resolution visual acuity (1.28 vs. 0.51, P < 0.001) (Snellen equivalent 20/400 vs. 20/63, P < 0.001). Final visual acuity was ≥20/40 for those with entering vision of ≥20/40 (59.6% vs. 74.7%, P = 0.001) and for those with entering vision of 20/50-20/200 (25.4% vs. 47.3%, P < 0.001). CONCLUSIONS: Management of choroidal hemangioma in the PDT era has allowed for significantly better visual outcome compared with the pre-PDT era, with mean final visual acuity of 20/400 (pre-PDT era) versus 20/63 (PDT era).

Circumscribed choroidal hemangioma: Clinical features and outcomes by age category in 458 cases.

PURPOSE: To investigate features and outcomes of circumscribed choroidal hemangioma by patient age. METHODS: Retrospective review of circumscribed choroidal hemangioma from 3/29/1967-6/4/2018 based on age at presentation (≤20 vs. >20-50 vs. >50 years). RESULTS: There were 458 circumscribed choroidal hemangiomas diagnosed at mean age (13 vs. 41 vs. 64 years, p < 0.001). The youngest age group had worse presenting visual acuity (20/400 vs. 20/150 vs. 20/100, p < 0.001), larger tumor basal diameter (13.5 vs. 6.6 vs. 6.2, p < 0.001), greater tumor thickness (5.8 vs. 3.1 vs. 2.9, p < 0.001), closer distance to foveola (0.5 vs. 1.4 vs. 1.2, p = 0.03), and greater extent of subretinal fluid (4 quadrants, 26% vs. 8% vs. 2%, p < 0.001). The youngest patients were less likely to be treated with primary observation (39% vs. 39% vs. 56%) or photodynamic therapy (10% vs. 27% vs. 22%) and more likely to be treated with plaque radiotherapy (26% vs. 6% vs. 3%) or external beam radiotherapy (13% vs. 1% vs. 0%) (p < 0.001). The youngest patients required greater total number of treatments (mean 4 vs. 2 vs. 1, p < 0.001). At mean follow-up (44 vs. 68 vs. 60 months, p = 0.37), the youngest patients had worse visual acuity (20/400 vs. 20/200 vs. 20/100, p = 0.03), but no difference in visual acuity loss of 3 or more Snellen lines (27% vs. 13% vs. 16%, p = 0.55). CONCLUSION: Younger patients (≤20 years) with circumscribed choroidal hemangioma present with worse visual acuity and larger, more posterior tumors. Future studies are needed to improve early detection and treatment for this subgroup of patients.