Nystagmus and Foveal Hypoplasia in a Carrier of Oculocutaneous Albinism.

We report a 23-year-old female patient with ophthalmic features of albinism, including refractive errors, nystagmus, depigmented fundus, and foveal hypoplasia. She presented for a rhegmatogenous retinal detachment, which was surgically reattached with no complications. Further genetic testing revealed the presence of a heterozygous pathogenic oculocutaneous albinism OCA2 gene mutation, conferring carrier status. To the best of our knowledge, this is the first reported case of typical ocular phenotype of albinism, specifically nystagmus, in a patient who is carrier for oculo-cutaneous albinism. Further research is required to expand the genotype-phenotype relationship in carriers of oculocutaneous albinism. [Ophthalmic Surg Lasers Imaging Retina 2024;55:349-353.].

Longitudinal Characteristics of Choroidal Neovascular Membrane in Pediatric Patients.

PURPOSE: To report the clinical and imaging characteristics, including optical coherence tomography angiography (OCTA), and treatment outcomes of choroidal neovascular membranes (CNVMs) in children. DESIGN: Retrospective clinical cohort study. METHODS: Thirty eyes from 25 children (56% girls) with CNVM from 2 centers were examined from 2005 to 2022. Clinical features, imaging findings, treatment regimens, and outcomes are described. RESULTS: The most common causes of CNVM were idiopathic (48%) and inflammatory (20%). At diagnosis, most CNVMs were unilateral (80%), active (83.3%), and juxtafoveal (46.7%). Twenty-five eyes (83.3%) of 21 patients (84%) were treated. The most common first-line treatment was intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) (92%), with a retreatment rate of 52.2% at an average of 237 days. The average number of total injections per eye was 2.3. Injections were safely administered in the clinic (52.2%). A gain of 3 lines or 15 ETDRS (Early Treatment Diabetic Retinopathy Study) letters was observed at final visit. The average duration of follow-up was 56.46 ± 42.51 months. No ocular or systemic complication related to treatment was reported. Sixteen eyes (64%) had OCTA images at both presentation and final visit, which showed a decrease in CNVM vessel density and vessel-length density, and in the height of retinal pigment epithelium detachment (RPED). CONCLUSIONS: There are a variety of underlying etiologies for pediatric CNVMs, which are most often unilateral. Treatment with intravitreal anti-VEGF can be beneficial and does not often require frequent or chronic dosing. OCTA demonstrated a decrease in the CNVM vessel density and vessel-length density as well as in the height of RPED.

Retinopathy With Variant of Unknown Significance and Atypical Chorioretinal Coloboma in the Setting of Prematurity.

A 37-week-old girl underwent ophthalmic examination. Born at 32 weeks, the infant weighed 680 grams and received high-flow nasal cannula for respiratory distress of the newborn. Dilated fundus examination of the right eye revealed an atypical chorioretinal coloboma; the left eye revealed hyperpigmentary changes in the macula. Fluorescein angiography of both eyes showed retinal vascularization to zone II. Genetic testing revealed a heterozygous variant of uncertain significance in the catenin Alpha 1 (CTNNA1) gene. CTNNA1 gene abnormalities have been implicated as causes of familial exudative vitreoretinopathy (FEVR). It is important to recognize possible simultaneous retinopathy of prematurity and FEVR. [Ophthalmic Surg Lasers Imaging Retina 2024;55:285-288.].

En Face Depth Resolved OCTA Monitoring of Macular Outer Retinal Deposits in a Case of Multifocal Choroiditis Mimicking Syphilitic Uveitis.

PURPOSE: Multifocal choroiditis (MFC) is a rare inflammatory condition characterized by retinal and choroidal lesions that may present similarly to ocular pathology of various etiologies. Here we present a case of MFC mimicking syphilitic uveitis with unique en face optical coherence tomography angiography (OCTA) imaging characteristics. METHODS: Case report. RESULTS: A 61-year-old woman presented with blurry vision, floaters and multiple whitish subretinal deposits on en face swept-source OCTA in the left eye. Fluorescent treponemal antibody test absorption was positive which led to the initial diagnosis of syphilitic uveitis and subsequent treatment with intravenous penicillin. During follow-up, OCTA of the left eye revealed the development of new choroidal neovascular membrane and new punched-out lesions in the posterior pole. The patient was eventually diagnosed with MFC and treated with aflibercept injections. CONCLUSIONS: Immune-mediated uveitis can simulate infectious and neoplastic uveitis. En face OCTA is unique imaging modality that allowed for the complete characterization and monitoring of the sub-macular deposits. This expands the clinical spectrum of multifocal choroiditis.

WIDEFIELD SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN WAGNER SYNDROME.

PURPOSE: To describe novel clinical and angiographic findings in Wagner syndrome. METHODS: A retrospective case series of three related patients with Wagner syndrome. Patients underwent standard optical coherence tomography (OCT), B-scan ultrasonography, and fluorescein angiography in addition to wide field swept-source OCT angiography (WF SS-OCTA) (PLEX Elite 9000, Carl Zeiss Meditec Inc). Patients underwent genetic testing for a panel of hereditary vitreoretinopathies. RESULTS: Three related patients with Wagner syndrome were identified. All were found to have prominent vitreous strands, abnormal vitreoretinal adhesions, peripheral retinal holes, and varying degrees of myopia. A mid-peripheral tractional ridge was identified in all six eyes. All patients were positive for a known pathologic intron variant in the VCAN gene (4004-5T-A). Wide field swept-source OCT angiography (12 mm × 12 mm) was performed in two patients and demonstrated perivascular capillary loss in the superficial capillary plexus along the arcades bilaterally. One patient demonstrated associated retinal atrophy within the area of capillary loss. The capillary loss extended beyond the margin of retinal atrophy. CONCLUSION: The unusual finding of a mid-peripheral tractional ridge of the retina associated with myopia led to a genetic diagnosis of Wagner syndrome. Widefield swept-source OCT angiography demonstrated a novel feature of perivascular loss of the superficial retinal capillary plexus. This result suggests that vitreous traction may cause localized microvasculature dysfunction and subsequent retinal atrophy in Wagner syndrome. This is the first known evaluation of Wagner syndrome using OCT angiography.

A Cost Effectiveness Analysis of Avacincaptad Pegol for the Treatment of Geographic Atrophy with Comparison to Pegcetacoplan.

PURPOSE: The purpose of this study was to evaluate the cost effectiveness of the treatment of geographic atrophy (GA) with intravitreal avacincaptad pegol (ACP) and to compare it with pegcetacoplan (PEG). DESIGN: Cost analysis based on data from published studies. SUBJECTS: None; based on data from published sham control compared with 2 treatment groups in each of the index studies. METHODS: Costs were based on 2022 Medicare reimbursement data for both facility (hospital-based) and nonfacility settings in Miami. Specific usage and outcomes were derived from the GATHER2 study as well as DERBY and OAKS trials. For ACP, all patients were treated every month (EM) in year 1 then randomized to every other month (EOM) or EM in year 2. Two-year models were created for patients in the facility setting for extrafoveal (ACP and PEG) and all patients (PEG). MAIN OUTCOME MEASURES: Cost, cost utility, and cost per area of GA (in United States dollars). RESULTS: The cost to treat GA with ACP in EM and EOM treatment groups over the 2 years as reported was $67 400 and $40 600, respectively. With ACP treatment over 2 years, the daily cost of delaying GA 3.4 months (EM) and 4.5 months (EOM) was $649 (EM) and $356 (EOM). The (facility-based) costs per unit area of retinal pigment epithelium saved for patients with extrafoveal GA over the 2-year period were $119 000/mm2 (EM ACP) versus $54 000/mm2 (EM PEG) (P < 0.001), $57 100/mm2 (EOM ACP) versus $31 400/mm2 (EOM PEG) (P < 0.001), and $45 300/mm2 (hypothetical EOM from outset ACP). CONCLUSION: Treatment of GA with intravitreal ACP EOM was more cost effective than EM. When assessing extrafoveal lesions, ACP was less cost effective than PEG for both EM and EOM treatment. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

In-Office Lens Repositioning for Anterior Crystalline Lens Dislocation.

BACKGROUND AND OBJECTIVE: We describe an in-office lens repositioning technique for anterior crystalline lens dislocation. PATIENTS AND METHODS: We present a case series of four patients with spontaneous or traumatic anterior crystalline lens dislocation. RESULTS: The technique included supine patient positioning, gentle pressure with a cotton swab on the peripheral cornea to guide the lens into the posterior chamber, and the use of a miotic agent afterward to prevent subsequent subluxation. In the four cases described, the in-office technique successfully restored the lens to the posterior chamber, improved vision, and decreased intraocular pressure in most instances by resolving the angle closure secondary to pupillary block. CONCLUSIONS: The in-office lens repositioning technique is appropriate as an acute non-surgical intervention or temporizing measure for anterior crystalline lens dislocation. [Ophthalmic Surg Lasers Imaging Retina 2024;55:293-298.].

Occlusive Retinal Vasculitis in a Pediatric Patient With Kikuchi-Fujimoto Disease and Sickle Cell Trait.

A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.].

Recovery of Vision in Open Globe Injury Patients with Initial No Light Perception Vision.

PURPOSE: To identify clinical characteristics of injured eyes associated with visual recovery in patients with open globe injuries (OGIs) and presenting with no light perception (NLP) vision. DESIGN: Retrospective chart review. SUBJECTS: All patients presenting to Massachusetts Eye and Ear with OGI and NLP vision from January 1999 to March 2022. METHODS: Manual data extraction to collect patient demographic characteristics, preoperative, intraoperative, and postoperative characteristics of OGI injury, laceration versus rupture, history of intraocular surgery, time from injury to repair, timing of vitrectomy, lensectomy, choroidal drainage, and silicone oil placement, visual acuity (VA) at last follow-up, and subsequent B-scan ultrasound findings of retinal detachment, choroidal hemorrhage, vitreous hemorrhage, and disorganized intraocular contents. Patients with >1 week of follow-up and a documented VA at most recent follow-up were included. Exclusion criteria included age <10 years. Multivariable regression was performed. MAIN OUTCOME MEASURES: Visual acuity recovery defined as light perception or better in patients with OGI and initial NLP vision. RESULTS: One hundred forty-seven eyes with NLP vision after OGI were included. Twenty-five (17%) eyes regained vision at last follow-up. The majority of patients recovered light perception vision (n = 15, 60%) followed by 20/500 or better (n = 5, 20%), hand motions (n = 3, 12%), and counting fingers (n = 2, 8%). Most injuries were zone III (n = 102, 69%) and presented with rupture (n = 127, 86%). The mean time from OGI to surgical repair was 0.85 ± 1.7 days. B-scan was obtained in 104 (71%) cases. Pars plana vitrectomy was performed in 9 eyes (6%) with NLP at time of vitrectomy. Disorganized intraocular contents on B-scan (odd ratio, 0.170; 95% confidence interval, 0.042-0.681; P = 0.012) was the only clinical variable significantly associated with visual recovery, corresponding to a lack of visual improvement. CONCLUSIONS: Recovery of vision in OGI with NLP vision at presentation cannot be predicted based on presenting clinical features. B-scan findings of disorganized intraocular contents after initial OGI repair was the only factor negatively associated with vision recovery in this patient population. Therefore, all eyes presenting with an OGI and NLP vision should undergo primary repair in hopes of subsequent visual recovery. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Academic versus Community Retinal Surgery for Primary Retinal Detachment: Characteristics, Duration, and Value Analysis of Teaching Modifier.

PURPOSE: To compare operative time and case characteristics of primary rhegmatogenous retinal detachment (RRD) repairs between academic and community vitreoretinal surgeons. DESIGN: A retrospective, observational clinical study. SUBJECTS: Patients who underwent primary RRD repair surgeries at Massachusetts Eye and Ear between 2019 and 2021. METHODS: A random sample of 20 vitreoretinal surgeons distributed evenly among the academic or community setting was selected. Fifteen consecutive cases of primary RRD repair surgeries were included from each surgeon. A cost analysis was performed for the teaching modifier for the physician fee and for hospital costs. MAIN OUTCOME MEASURES: Length of surgery. RESULTS: Of 300 primary RRD repairs, fellows were present in 75%, which comprised all academic surgeon cases and 50% of community surgeon cases, P < 0.001. Mean operation length was shorter for community surgeon cases without fellows (55.0 ± 24.1) than either academic (73.0 ± 30.8) or community surgeon cases with fellows (75.7 ± 32.5) (P < 0.001). There was a higher percentage of macula-off RRDs in academic versus community surgeon cases (52.7% vs. 38.0%, P = 0.002) and higher rates of combined scleral buckle (SB)/pars plana vitrectomy (PPV) repairs (14% vs. 3%, P < 0.001). When excluding combined SB/PPV cases, there was no difference in operative time between academic and community surgeon cases. Among RRDs repaired by PPV only, there was a 31.4% (16.6 minutes) greater procedure duration in cases with fellows compared with cases without fellows (P < 0.001). Covariates associated with greater surgery time: addition of an SB (ß = 32.6), membrane peel (ß = 18.5), presence of a fellow (ß = 14.5), proliferative vitreoretinopathy (ß = 12.8), and greater number of retinal breaks (ß = 2.4). The teaching modifier adds 16% extra reimbursement ($184.16) to the physician fee, which is 50.9% of what is necessary to cover the percentage increase in surgeon time (31.4%). Using a time-driven activity-based costing for hospital costs, the extra 16.6 minutes leads to an additional $1038.00, which is 5.6 times more than the reimbursement for the modifier. CONCLUSIONS: Retinal detachment repair cases performed by academic surgeons are more likely to be macula-off and include the addition of an SB, which drive longer operative times. Medicare's reimbursement of the assistant modifier in a teaching facility significantly undercompensates the time-driven activity-based costing of trainee participation. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Validation of the "TWO-ROP" Algorithm at a Multi-Neonatal Intensive Care Unit Tertiary Referral Center.

PURPOSE: We aim to validate the previously published TWO-ROP algorithm on an external data set. DESIGN: Retrospective consecutive study. SUBJECTS: Infants screened for retinopathy of prematurity (ROP) between January 2013 and August 2023 at a tertiary referral multi-site. METHODS: Infants with higher birth weight (BW) and greater gestational age (GA) were included and stratified into 3 groups as follows: group 1 (BW <1500 g, GA ≥30 weeks), group 2 (BW ≥1500 g, GA <30 weeks), and group 3 (BW ≥1500 g, GA ≥30 weeks). MAIN OUTCOME MEASURES: The rate of ROP, treatment-warranted ROP (TW-ROP), and number of inpatient examinations were evaluated in the 3 groups. RESULTS: In total, 1095 (33.8%) patients met the inclusion criteria. The number of patients in groups 1, 2, and 3 was 837 (76.4%), 72 (6.6%), and 186 (17.0%), respectively. Retinopathy of prematurity was detected in 120 (11.0%) patients; the rate was 9.8% in group 1, 20.8% in group 2, and 12.4% in group 3 (P = 0.013). The overall mean number of inpatient examinations for patients undergoing traditional, TWO-ROP 36-week, and TWO-ROP 40-week screening systems was 1.95, 1.43, and 0.99, respectively (P < 0.001). Stage 3 was found in 9 eyes of 5 patients (0.5%, all zone II). Three eyes of 2 patients (0.2%) had plus disease. Two patients had bilateral laser treatment at 44 and 39.4 weeks postconceptional age (PCA); 3 out of 4 of these eyes met type 1 treatment criteria. Overall, the ROP screening burden saved was 9.0% and 16.7% for the TWO-ROP 36-week and 40-week systems, respectively. The sensitivity for TW-ROP was 100% for TWO-ROP 36-week system and 99.4% for TWO-ROP 40-week system. CONCLUSION: The TWO-ROP algorithm can reduce the number of inpatient examinations while maintaining safety. To ensure timely management, we recommend that the single first ROP examination occur at 38 to 39 weeks PCA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Fluorescein Angiography Parameters in Premature Neonates.

Purpose: To describe fluorescein angiography (FA) parameters observed in premature neonates with retinopathy of prematurity (ROP). Design: Retrospective case series. Subjects: Patients with ROP who underwent FA imaging using Retcam at Holtz Children's Hospital from November 2014 to October 2022. Methods: Fluorescein angiography images of the included patients were analyzed with a focus on the timing of angiography phases, including choroidal flush, retinal, and recirculation phases. Gestational age, birth weight (BW), age at imaging, treatment choice, and any FA complications were documented. Main Outcome Measures: Dose of fluorescein administered, onset and duration of each angiography phase, and FA findings in ROP-treated patients. Results: A total of 72 images of 72 eyes were reviewed. Image quality was deemed suitable for inclusion in 64 eyes (88.9%) of 43 patients. The mean gestational age and BW at birth were 24.4 ± 1.9 weeks and 607.8 ± 141.3 g, respectively. The mean postmenstrual age at FA imaging was 50.5 ± 40.8 weeks. All eyes (100%) received treatment with intravitreal injection of anti-VEGF at a mean age of 35.5 ± 2.4 weeks. The onset and duration of angiography phases were relatively variable within the cohort. Choroidal flush occurred at a mean time of 12.2 seconds (range: 6-22 seconds). A subsequent retinal phase was documented at a mean time of 11.96 seconds (range: 3-22 seconds). Recirculation phase was complete at an average time of 2.15 minutes (range: 1-5.45 minutes) postfluorescein injection. None of patients developed allergic reactions to fluorescein injection, such as rash, respiratory distress, tachycardia, fever, or local injection site reactions. Conclusions: Angiographic phases on FA in preterm infants with ROP are variable and may occur earlier than the established references for adults. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Widefield Swept-Source OCTA Findings in HELLP Syndrome: Choroidal Infarcts.

PURPOSE: Pre-eclampsia, eclampsia, and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome have been previously implicated with ophthalmic complications including serous retinal detachments and disorders of the choroidal vasculature. Herein, we report a case of macular serous detachment associated with HELLP syndrome in which wide field swept-source optical coherence tomography angiography (WF SS-OCTA) was used. METHODS: Retrospective case report of a patient who developed HELLP syndrome. The patient underwent multimodal retinal imaging and wide field swept-source OCT angiography (WF SS-OCTA) (PLEX® Elite 9000, Carl Zeiss Meditec Inc.). RESULTS: A 36-year-old female patient diagnosed with HELLP syndrome presented with bilateral blurry vision. At presentation, dilated fundus exam revealed localized subretinal fluid in the macula. WF SS-OCTA showed areas of peripapillary and subfoveal flow signal attenuation in the choroid OD, consistent with choroidal infarction. CONCLUSIONS: These findings support the hypothesis that HELLP syndrome is associated with vascular changes that lead to choroidal dysfunction and subsequent serous retinal detachments. Furthermore, this case highlights a role for the non-invasive WF SS-OCTA technology in diagnosing and further characterizing the pathophysiology without the use of dye-based angiography.

Efficacy and Safety of Anti-VEGF Injections and Surgery for AMD-Related Submacular Hemorrhage: A Systematic Review & Meta-Analysis.

TOPIC: This systematic review and meta-analysis investigates the efficacy and safety of anti-vascular endothelial growth factor (anti-VEGF) injections compared to surgical intervention in improving visual acuity (VA) and reducing complications for patients with submacular hemorrhage (SMH) due to neovascular age-related macular degeneration (AMD). CLINICAL RELEVANCE: Determining the optimal intervention for SMH in AMD is crucial for patient care. METHODS: We included studies on anti-VEGF injections or surgical interventions for SMH in AMD from 7 databases, searched up to May 2024. Data extraction and quality assessment were done by two independent reviewers. Certainty of evidence was assessed GRADE approach. Meta-analysis employed random-effects models. Primary outcomes were pooled mean logMAR VA difference (initial examination minus last follow-up VA) and adverse events rates. RESULTS: A total of 43 observational studies were included: 21 (960 eyes) on anti-VEGF and 22 (455 eyes) on surgery. Comparisons were made across separate studies due to lack of head-to-head studies. Meta-analysis included 11 anti-VEGF studies (444 eyes) and 12 surgical studies (195 eyes) for VA outcomes. The mean difference (MD) in VA was -0.16 (95%CI: -0.26,-0.07) for anti-VEGF and -0.36 (95%CI: -0.68,-0.04) for surgery, with no significant difference between groups (X2=1.70, df =1, p=0.19). Heterogeneity was high in surgical studies (I2=96.2%, tau2=0.23, p<0.01) and negligible in anti-VEGF studies (I2=7%, tau2=0.003, p=0.38). GRADE certainty was moderate for anti-VEGF and low for surgery. Anti-VEGF had lower rates of cataract (0% vs 4.6%), proliferative vitreoretinopathy (PVR, 0.1% vs 2.0%), and retinal detachment (RD, 0.1% vs 10.6%), but similar rates of recurrent hemorrhage (5.4% vs 5.3%). Complications were summarized descriptively due to zero cell problem. CONCLUSION: Both anti-VEGF and surgery treat SMH in AMD with similar VA outcomes but different safety profiles. Anti-VEGF is preferred for less severe hemorrhage, while surgery is suited for extensive hemorrhage. Despite uncertain comparative VA outcomes, treatment should be guided by clinical judgment and patient factors.