Iris freckle: a distinct entity.

PURPOSE: To report distinctive clinical and imaging features of iris freckles to differentiate them from iris nevi. DESIGN: Retrospective observational study. SUBJECTS: 53 patients (277 freckles) with incidental iris freckles and 102 patients (104 nevi) with iris nevi that are either clinically stable or pathologically confirmed. METHODS: Patient data were collected from the Department of Ophthalmic Oncology, Cleveland Clinic, Cole Eye Institute database (2012-2023). Lesion characteristics were recorded from slit-lamp examination descriptions and review of colour photographs. Ancillary imaging features observed using anterior segment optical coherence tomography (AS-OCT) and ultrasound biomicroscopy (UBM) were assessed in patients (where available). MAIN OUTCOME MEASURES: Comparison of clinical and imaging features of iris freckles and iris nevi. RESULTS: A total of 277 iris freckles and 104 iris nevi were analysed. Iris freckles were more frequently bilateral (17%; nevi 0%) and multiple (69%; nevi 2%) and located centrally (89%; nevi 17%) compared with iris nevi (p<0.001). The median freckle largest basal diameter and thickness were 0.8 mm (nevi; 2.1 mm, p<0.001) and 0.04 mm (nevi 1.0 mm, p<0.001), respectively. All iris freckles had irregular margins without any secondary effects compared with iris nevi. Iris freckles appeared flat without effacement of iris folds compared with iris nevi on AS-OCT (p<0.001). Iris freckles were not detectable by UBM. Heat map revealed that freckles demonstrated several features with uniform or near uniform values, whereas nevi demonstrated more variability in values across features. CONCLUSIONS: Iris freckles exhibit specific clinical and imaging features reflective of their characteristic histological composition that support their classification as a distinct entity within the spectrum of iris pigmented lesions.

Choroidal Nevi in Children: Size, Growth, and Topographic Distribution.

PURPOSE: To explore size, growth, and topographic distribution of choroidal nevi in children to gain insights into choroidal nevogenesis. DESIGN: Retrospective consecutive case series using pediatric clinic - and population-study data, comparing to adult data. METHODS: Clinical data from Cole Eye Institute (CEI) database (December 2005-January 2023) was derived from a retrospective consecutive case series of 20 children (< 20 years) with choroidal nevi. For population data, 48 children from previously reported pooled data of the participants of the Sydney Pediatric Eye Disease Study, Sydney Myopia Study, Sydney Childhood Eye Study, and Sydney Adolescent Vascular and Eye Disease Study were included. Fundus photographs were reviewed and the locations of 18 choroidal nevi seen at CEI with widefield imaging were mapped on a radial scatter plot. For comparison, 100 consecutive adults with choroidal nevi were identified from CEI database. Main outcomes were size, growth, and topographic distribution of choroidal nevi. RESULTS: The median largest basal diameter was 1.6 mm (range 0.4-4.2) in children. Most choroidal nevi (75%) remained stable, and 16% demonstrated growth at follow-up. The mean growth rate was calculated as 0.12 mm/year (range 0.10-0.15). Malignant transformation was not noted during childhood. All secondary changes (drusen, orange pigment, and subretinal fluid) associated with choroidal nevi in children were less common than those in adults (p < .05). Choroidal nevi in children were located significantly more posterior than in adults. The median distance to fovea was 2.1 mm (range 0.5-8.5) in children and 5.1 mm (range 0.4-16) in adults (p < .0001). CONCLUSIONS: The onset and growth of choroidal nevi in children suggest active choroidal nevogenesis in childhood. A posterior topographic distribution may support the developmental framework for migration and maturation of choroidal melanoblasts.

Uveal melanoma in African Americans: Diagnostic challenges.

Uveal melanoma (UM) is uncommon in African Americans. Owing to its rarity, UM may not be suspected in African Americans leading to delayed diagnosis. In addition, socioeconomic factors may also play a role in delayed diagnosis. Clinical and ultrasonographic features may be atypical due to racial pigmentation, necessitating diagnostic fine needle aspiration biopsy. Herein, we report an illustrative case series of 12 African Americans with UM highlighting clinical features and diagnostic challenges.

Radiation Optic Neuropathy: Management Options.

Background: Radiation optic neuropathy may be one side effect of ionizing radiation exposure to the eye found in a minority of patients. It is generally devastating for visual function and has been the subject of a small but growing literature with respect to its pathophysiology, treatment, and expected outcomes. Summary: Clinical features include optic disc edema, peripapillary hemorrhages, cotton wool spots, and hard exudates. Visual acuity is generally significantly reduced. Treatment has been attempted with outcomes that have not been assessed by randomized trials. Observation may be indicated in addition to treatment. Key Messages: Radiation optic neuropathy is known to generally be devastating to vision though an uncommon side effect of radiation. Treatment has been attempted with mixed results.

Surveillance for Metastasis in High-Risk Uveal Melanoma Patients: Standard versus Enhanced Protocols.

PURPOSE: to evaluate the effectiveness of enhanced surveillance protocols (EP) utilizing high frequency (HF) or enhanced modality (EM) compared to the standard protocol (SP) in detecting metastasis and determining their impact on overall survival (OS) in high-risk uveal melanoma (UM) patients. METHODS: A total of 87 consecutive patients with Class 2 (high risk) primary UM were enrolled, with negative baseline systemic staging. The patients underwent systemic surveillance with either SP (hepatic ultrasonography [US] every 6 months) or EP (either HF [US every 3 months] or EM [incorporation hepatic computed tomography/magnetic resonance imaging]) following informed discussion. The main outcome measures were largest diameter of largest hepatic metastasis (LDLM), number of hepatic metastatic lesions, time to detection of metastasis (TDM), and OS. RESULTS: This study revealed significant differences in LDLM between surveillance protocols, with the use of EP detecting smaller metastatic lesions (HF, EM, and SP were 1.5 cm, 1.6 cm, and 6.1 cm, respectively). Patients on the EM protocol had a lower 24-month cumulative incidence of >3 cm metastasis (3.5% EM vs. 39% SP; p = 0.021), while those on the HF protocol had a higher 24-month cumulative incidence of ≤3 cm metastasis compared to SP (31% HF vs. 10% SP; p = 0.017). Hazard of death following metastasis was significantly reduced in the EP (HR: 0.25; 95% CI: 0.07, 0.84), HF (HR: 0.23; 95% CI: 0.06, 0.84), and EM (HR: 0.11; 95% CI: 0.02, 0.5) groups compared to SP. However, TDM and OS did not significantly differ between protocols. CONCLUSIONS: Enhanced surveillance protocols improved early detection of hepatic metastasis in UM patients but did not translate into a survival advantage in our study cohort. However, early detection of metastasis in patients receiving liver-directed therapies may lead to improved overall survival.

INTRAVITREAL FLUOCINOLONE ACETONIDE IMPLANT FOR RADIATION RETINOPATHY: Report of Preliminary Findings.

PURPOSE: To assess the efficacy of a 0.18 mg intravitreal fluocinolone acetonide (FA) implant (Yutiq, EyePoint Pharmaceuticals, Watertown, MA) as a treatment option for patients with radiation retinopathy-related cystoid macular edema. METHODS: A retrospective review of seven patients treated for uveal melanoma who developed radiation retinopathy-related cystoid macular edema. They were initially treated with intravitreal anti-vascular endothelial growth factor and/or steroid injections and then transitioned to intravitreal FA implant. Primary outcomes include best-corrected visual acuity, central subfield thickness, and number of additional injections. RESULTS: After FA implant insertion, best-corrected visual acuity and central subfield thickness remained stable in all patients. The variance in best-corrected visual acuity decreased from 75.5 ETDRS letters (range 0-199 letters) to 29.8 (range 1.2-134) after FA implant insertion. Mean central subfield thickness was 384 µ m (range 165-641) and 354 µ m (range 282-493) before and after FA implant insertion, resulting in a 30- µ m mean reduction. The number of intravitreal injections (average 4.9, range 2-10) decreased after intravitreal FA implant insertion with only two patients requiring one additional FA implant (average 0.29, range 0-1) over a mean of 12.1 months (range 0.9-18.5) follow-up. CONCLUSION: Intravitreal FA implant is an effective treatment for cystoid macular edema radiation retinopathy. The slow release of steroid allows for sustained control of macular edema, which correlated with stable visual acuity and decreased injection burden for patients.

Impact of Coronavirus Disease 2019 Restrictions on Retinal Detachment: A Multicenter Experience.

In this retrospective, multicenter study of 261 eyes (259 patients), patients who underwent rhegmatogenous retinal detachment repair during the coronavirus disease 2019 (COVID-19) post-lockdown period experienced an additional 22-day delay, leading to significantly more epiretinal membrane and proliferative vitreoretinopathy and lower single-surgery anatomic success rates. During lockdown, perfluoropropane gas was used more commonly, and pneumatic retinopexy was used more commonly in COVID-19-positive patients.

Outcomes of Rhegmatogenous Retinal Detachment Repair When Comparing Surgeon Continuity in a Team-Based Practice.

BACKGROUND AND OBJECTIVE: To facilitate timely surgery and efficient use of operating room time, our practice uses a team-based approach so patients may undergo primary rhegmatogenous retinal detachment (RRD) surgery with a different surgeon instead of the diagnosing surgeon. PATIENTS AND METHODS: This was a retrospective cohort study of 331 eyes that underwent RRD surgery. Patients were divided into two groups: RRD surgery performed by the diagnosing surgon, and RRD surgery performed by a different surgeon. RESULTS: Of 331 eyes, 200 eyes (60.4%) were repaired by the diagnosing surgeon and 131 eyes (39.6%) were repaired by a different surgeon. Primary anatomic success (PAS) rates at 3 months postoperatively were equivalent between the two groups (87.0% and 87.8% in the diagnosing surgeon and different surgeon groups, respectively [P = .83]). There was no significant difference in preoperative (P = .08) or final (P = .28) visual acuity between the groups. Time between diagnosis and RRD repair was shorter in the different surgeon group (median of 1.5 days [IQR: 1.0-3.6] in the surgeon group versus 2.2 days [IQR: 0.8-5.7] in the diagnosing surgeon group) (P = .03). Logistic regression analysis gave no evidence to suggest that PAS rates depended on day of week, time of day surgery was performed, group, or the interaction between those factors (P = .93). CONCLUSIONS: Visual and anatomic success in RRD repair are equivalent when surgery is performed by either the diagnosing surgeon or a surgical colleague because time to surgery is reduced. Neither time of day nor day of the week had any influence on the outcomes. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:560-566.].

Management of Retinal Detachment Associated with Morning Glory Disc Syndrome.

We report a case of morning glory disc anomaly in a young patient with tractional retinal detachment successfully repaired with complex pars plana vitrectomy, membrane peel, laser, and oil tamponade. A 19-year-old female with a history of right morning glory disc anomaly associated with PAX6 gene mutation presented with floaters, photopsia, central scotoma, and visual acuity (VA) of 1/200. A complex macula-involving tractional retinal detachment centered around the optic nerve with a morning glory disc anomaly. Retinal detachment was treated with 25-gauge pars plana vitrectomy with difficult separation of the posterior hyaloid. Fibrous preretinal membranes were peeled, a temporal relaxing retinotomy was required, subretinal fluid was drained through a superonasal retinotomy during air-fluid exchange, endolaser was applied, and tamponade was achieved with 1,000-centistoke silicone oil. The retina remained attached at 1-year follow-up, with VA count fingers throughout. Morning glory disc is a rare congenital anomaly associated with PAX6 gene mutation that most often occurs unilaterally. It is rarely associated with tractional retinal detachment. Optimization of visual outcome is imperative despite a poor visual prognosis.

Non-paraneoplastic autoimmune retinopathy: multimodal testing characteristics of 13 cases.

BACKGROUND: Non-paraneoplastic autoimmune retinopathy (npAIR) is a rare autoimmune disease that primarily affects retinal photoreceptor function and results in profound and often times permanent vision loss. Delay in diagnosis and treatment initiation may contribute to the poor visual prognosis. METHODS: A retrospective chart review of all patients diagnosed with autoimmune retinopathy at the University of Wisconsin-Madison Eye Clinics between January 2012 and January 2017 was performed. Twenty eyes of 15 patients had evidence of any form of autoimmune retinopathy through a combination of symptoms, ocular findings, visual fields, optical coherence tomography, fundus autofluorescence, full-field and multifocal electroretinography, and serum anti-retinal antibodies. Clinical records were also analyzed for demographic data, systemic comorbidities, visual acuity, treatment employed, and disease progression. RESULTS: We identified 18 eyes from 13 patients who fit the criteria for non-paraneoplastic autoimmune retinopathy. Sixty-nine percent of patients were female with a mean age of symptom onset of 56.9 ± 20.3 years. Sixty-seven percent of eyes had an associated autoimmune condition, most commonly hypothyroidism. Serum testing revealed a preponderance of antibodies against carbonic anhydrase II, while imaging revealed characteristic changes. Fundus autofluorescence most commonly showed hyperautofluorescence around the macula. The delayed diagnosis led to a larger reduction in the horizontal extent of ellipsoid zone in 1-mm perifoveal area on optical coherence tomography with resulting visual decline. There was no difference in the change of visual acuity when stratifying for patients with autoimmune conditions (p = 0.52) or treatment status (p = 0.50). None of the patients who received treatment developed contralateral eye involvement or experienced disease progression based on visual acuity or symptoms. CONCLUSION: Non-paraneoplastic autoimmune retinopathy has a wide and often challenging to diagnose spectrum of clinical symptoms and imaging findings. Immunosuppressive therapy can be considered empiric in the face of a suggestive presentation and can be initiated after an evaluation of clinical findings and multimodal testing, though treatment does not appear to affect regeneration of the ellipsoid zone on OCT or impact visual acuity. Treatment should be primarily used to prevent disease progression and contralateral eye involvement. TRIAL REGISTRATION: N/A.

Bilateral corneal perforation due to MRSA keratitis in a crosslinking patient.

Introduction: The cornea may become infected and perforated after epithelium-on collagen crosslinking. Case presentation: A healthy 33-year-old male who underwent corneal collagen crosslinking in both eyes developed a purulent keratitis and bilateral corneal perforations, requiring bilateral penetrating keratoplasties. He was exposed to methicillin resistant staphylococcus aureus (MRSA) by a family member with a tracheostomy and was treated with MRSA-directed antibiotics. After prolonged recovery and treatment of his infection, he had acceptable but limited uncorrected visual acuity, with excellent corrected visual acuity. Conclusion: While epithelium-on crosslinking is commonly thought to be associated with a lower risk of postoperative infection, this case illustrates that even epithelium-on treatment may present the patient with a risk of infection. Patients in higher risk groups who are exposed to infectious disease may be more predisposed.

Comparison of Baerveldt 101-350 Glaucoma Implant (BGI) versus Trabeculectomy with ExPress™ Shunt for the Treatment of Primary Open Angle Glaucoma.

Importance: Trabeculectomy is very effective in lowering intraocular pressure for the treatment of glaucoma, but it carries with it possible complications and failure. The ExPress shunt (Alcon Laboratories, Fort Worth, TX, USA) is an adjunctive device that can be used at the time of trabeculectomy to create an external fistuliztion. An alternative established and highly efficacious technique is the implantation of a glaucoma drainage device for sustained intraocular pressure (IOP) lowering. Specifically, evidence has established the Baerveldt 101-350 glaucoma implant (BGI) to have the best sustained IOP lowering in long-term follow-up amongst the many options for glaucoma drainage devices. Objective: To compare outcomes in eyes that underwent Baerveldt 101-350 glaucoma implant (BGI) and trabeculectomy with ExPress shunt (Trab) in primary open angle glaucoma without any prior incisional glaucoma surgery. Design, Setting, and Participants: This was a retrospective study of outcomes in patients identified by CPT codes as having undergone glaucoma implantation or trabeculectomy (with ExPress shunt) for the treatment of Primary Open Angle Glaucoma between 2012 and 2015 at a single institution by 2 fellowship trained glaucoma surgeons. A total of 57 eyes that underwent Baerveldt 101-350 glaucoma implant and 38 eyes that underwent trabeculectomy cases with ExPress™ shunt were included in the study. All patients were diagnosed with primary open angle glaucoma. Cases were included into the study if the patient underwent BGI or trabeculectomy with ExPress shunt without any prior incisional glaucoma surgery. Main Outcomes and Measures: Main outcomes included IOP, medications, visual acuity (VA), and secondary glaucoma surgery, if any. Results: Survival rate at 12 months was 85% in the BGI group and 80% in trabeculectomy with ExPress Shunt. A statistically significant difference was not found in the survival distributions between surgery groups using the log-rank test. A total of 12 trabeculectomy and 9 BGI cases failed by our definition of success. These cases were included in the analysis of IOP, number of glaucoma medications, and VA. The mean IOP was reduced from 20.6 ± 5.6 mmHg to 12.4 ± 3.2 mmHg and from 20.7 ± 5.5 mmHg to 11.3 ± 4.8 mmHg at one year post-operation in the BGI group and the trabeculectomy with ExPress shunt group, respectively. On average, the BGI group showed an IOP reduction of 7.7 ± 6.1 mmHg, while trabeculectomy with ExPress shunt experienced a decrease of 7.9 ± 5.2 mmHg at one year post-operation. Medications were reduced from 3.5 ± 0.8 to 2.6 ± 1.3 at one year in the BGI group and from 3.7 ± 0.5 to 0.6 ± 1.8 in the trabeculectomy with ExPress shunt group. At one year post-operation, the BGI group had an average of 0.9 ± 1.1 medication reduction, while trabeculectomy with ExPress shunt cases had a reduction of 3.2 ± 1.3 medications. VA was compared in logMar. At baseline, the average for BGI logMar was 0.5 ± 0.7 and the average for trabeculectomy was 0.2 ± 0.3. At one year post-operation, the BGI group's VA was 0.4 ± 0.4 while the trabeculectomy with ExPress shunt group's VA was 0.1 ± 0.1. Conclusions and Relevance: The Baerveldt 101-350 glaucoma implant and trabeculectomy (with ExPress™ shunt) may have similar rates of success in the surgical treatment of primary open angle glaucoma in eyes that are naïve to prior incisional glaucoma surgery, with a higher dependence on topical medications post-operation in patients undergoing Baerveldt glaucoma implantation.

Soluble vascular endothelial growth factor receptor 3 is essential for corneal alymphaticity.

Corneal transparency is a prerequisite for optimal vision and in turn relies on an absence of blood and lymphatic vessels, which is remarkable given the cornea's proximity to vascularized tissues. Membrane-bound vascular endothelial growth factor receptor 3 (VEGFR-3), with its cognate ligand vascular endothelial growth factor C (VEGF-C), is a major mediator of lymphangiogenesis. Here, we demonstrate that the cornea expresses a novel truncated isoform of this molecule, soluble VEGFR-3 (sVEGFR-3), which is critical for corneal alymphaticity, by sequestering VEGF-C. sVEGFR-3 binds and sequesters VEGF-C, thereby blocking signaling through VEGFR-3 and suppressing lymphangiogenesis induced by VEGF-C. sVEGFR-3 knockdown leads to lymphangiogenesis and hemangiogenesis in the mouse cornea, while overexpression of sVEGFR-3 inhibits lymphangiogenesis and hemangiogenesis in a murine suture injury model. Pax6(+/-) mice spontaneously develop corneal and lymphatic vessels and are deficient in sVEGFR-3. sVEGFR-3 suppresses hemangiogenesis by blocking VEGF-C-induced phosphorylation of VEGFR-2. Overexpression of sVEGFR-3 leads to a 5-fold increase in corneal transplant survival in mouse models. sVEGFR-3 holds promise as a molecule to control and regress lymphatic-vessel-based dysfunction. Therefore, sVEGFR-3 has the potential to protect the injured cornea from opacification secondary to infection, inflammation, or transplant rejection.