Contrast Sensitivity Better Reflects Wide-Field Swept-Source Optical Coherence Tomography Angiography Vascular Metrics Among Healthy Eyes Compared to Visual Acuity.

BACKGROUND AND OBJECTIVE: To evaluate the association between widefield swept-source optical coherence tomography angiography (WF SS-OCTA) and visual function in healthy eyes. PATIENTS AND METHODS: Fifty-seven eyes of 45 patients were evaluated with visual acuity (VA), contrast sensitivity (CS), and WF SS-OCTA (3 × 3, 6 × 6, and 12 × 12 mm images) on the same day. Mixed-effects multivariable regression analyses were performed. RESULTS: Contrast sensitivity metrics, including CS between 6 to 18 cycles per degree (cpd) and area under the logarithm CS function, were significantly associated with vessel density (VD) and vessel skeletonized density (VSD), whereas VA was not. The largest effect size was between CS at 18 cpd and VD (ß = 0.41, P = 0.007) and VSD (ß = 0.42, P = 0.006) on 12 × 12 mm images. CONCLUSIONS: Reduced VSD and VD on WF SSOCTA was significantly associated with decreased CS, whereas VA was not. These results suggest CS could serve as a screening tool for early stage retinal and neurologic disorders. [Ophthalmic Surg Lasers Imaging Retina 2024;55:XX-XX.].

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.].

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.

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.

Associations of quantitative contrast sensitivity with vascular metrics on widefield swept-source OCT angiography across stages of diabetic retinopathy.

PURPOSE: To investigate structure-function associations between contrast sensitivity (CS) and widefield swept-source optical coherence tomography angiography (WF SS-OCTA) vascular metrics across stages of non-proliferative (NPDR) and proliferative diabetic retinopathy (PDR), without diabetic macular oedema. METHODS: Prospective cross-sectional study in 140 eyes of 99 patients: 33 mild NPDR, 24 moderate/severe NPDR, 15 PDR, 33 diabetic without DR (DMnoDR) and 46 control eyes. Mixed-effects multivariable regression models to evaluate associations between quantitative contrast sensitivity function (Adaptive Sensory Technology) and vessel density (VD) and vessel skeletonised density (VSD) in the superficial capillary plexus (SCP) and deep capillary plexus (DCP) on same-day imaging with WF SS-OCTA (Plex Elite 9000, Carl Zeiss Meditec). RESULTS: Standardised ß coefficients for area under the logarithm of contrast sensitivity function curve (AULCSF) versus visual acuity (VA) at 3×3 mm scans: SCP VSD (ß=0.32, p<0.001 vs -0.18, p=0.044), DCP VSD (ß=0.30, p<0.001 vs -0.21, p=0.02), SCP VD (ß=0.25, p=0.004 vs -0.13, p=0.129), DCP VD (ß=0.26, p=0.003 vs -0.19, p=0.034). AULCSF was significantly reduced in mild NPDR (ß=-0.28, p<0.001) and DMnoDR (ß=-0.19, p=0.005) versus controls, while VA was not significantly different. AULCSF performed better than VA in differentiating between controls and DMnoDR (0.69 vs 0.50), controls and mild NPDR (0.76 vs 0.61) and controls and moderate/severe NPDR (0.89 vs 0.73). CONCLUSIONS: DR-induced microvascular changes on OCTA are associated with larger changes on CS than in VA. CS is affected earlier than VA in the course of DR and performed better in discriminating between controls, DMnoDR and across DR stages.

Faricimab in Previously Treated Eyes With Neovascular Age-Related Macular Degeneration: An Assessment of Durability and Treatment Outcomes.

BACKGROUND AND OBJECTIVE: This study evaluated the efficacy and durability of faricimab in patients with neovascular age-related macular degeneration (nAMD) who were previously treated with anti-vascular endothelial growth factor (anti-VEGF) agents. PATIENTS AND METHODS: This retrospective case series was conducted at a single tertiary center in the United States. It focused on nAMD patients who transitioned to faricimab after initial anti-VEGF therapy, with a follow-up period of at least 9 months. "Complete dryness" was defined as the absence of intra- and/or subretinal fluid on optical coherence tomography. Durability was gauged by the extension of treatment intervals relative to the injection frequency of the previous agent. RESULTS: Sixty-two eyes from 62 patients were included. Treatment interval ranged from 5 to 10 weeks; 10 (16%) patients were able to be extended by 2 or more weeks compared to their previous regimen. Median (interquartile range [IQR]) central field thickness was 310 µm (254, 376) on initiating faricimab and declined by the ninth month (P values at 3, 6, and 9 months were 0.01, 0.02, and 0.07, respectively). Median (IQR) visual acuity at initiation of faricimab was 0.4 (0.20, 0.50) and did not change by the ninth month. Complete anatomical dryness was present in 10 (16%) eyes before switching; 90% remained dry at 9 months. Of 52 (84%) incompletely dry eyes before switching, 15% achieved complete dryness by 9 months on faricimab. CONCLUSIONS: Faricimab modestly improved the treatment intervals for a small proportion of previously treated patients on anti-VEGF therapy. [Ophthalmic Surg Lasers Imaging Retina 2024;55:XX-XX.].

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.

Neonatal Macular Hemorrhage in a Term Healthy Infant: 18-Year Follow-up.

Unobstructed binocular vision is required during the critical period of vision development to obtain optimal visual acuity in each eye and binocular stereopsis. In this article, we report 18-year follow-up of a full-term, otherwise healthy infant noted to have dense premacular hemorrhage occluding the visual axis in the left eye on retinal imaging performed 48 hours after birth. Serial examinations by the retina service were performed weekly for 10 weeks as the hemorrhage resolved spontaneously. Shortly thereafter, visual acuity revealed fixation was present, but the mother noted intermittent left eye esodeviation. At 90 days of life, the infant was seen by pediatric ophthalmology and started on 1 to 2 hours patching of the right eye daily for the esotropia, which was maintained through 24 months. At 18 years of age, the patient had orthophoria alignment, no spectacle correction, vision of 20/20 in the right eye and 20/25 in the left eye, and normal binocularity and stereopsis. Intermittent esodeviation on the left eye was observed when the patient was fatigued. Early identification of a visual axis occlusion led to prompt referral for the esotropia and initiation of patching therapy. This patient ultimately achieved a very favorable visual functional outcome that may not have been possible without early detection and management. This case report describes the longest-term published follow-up of a neonatal macular hemorrhage. [Ophthalmic Surg Lasers Imaging Retina 2024;55:354-357.].

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.

Assessment of Microvascular Changes After Rhegmatogenous Retinal Detachment Repair Using Wide-Field Swept-Source Optical Coherence Tomography Angiography.

BACKGROUND AND OBJECTIVE: Our objective was to evaluate retinal microvascular changes and visual outcomes following rhegmatogenous retinal detachment (RRD) repair using wide-field swept-source optical coherence tomography angiography (WF SS-OCTA). PATIENTS AND METHODS: The study included 116 eyes of 111 patients with macula-off (n = 68) or macula-on (n = 48) RRD treated with a single successful procedure, 79 fellow eyes, and 183 eyes of control patients imaged with WF SS-OCTA (3 ×3, 6 ×6, and 12 ×12 mm images). Mixed-effects multiple linear regression models were used for statistical analysis. RESULTS: Vessel density (VD) and vessel skeletonized density (VSD) of the superficial capillary plexus (3 ×3 mm scans) and full-thickness retina (12 ×12 mm) were significantly reduced in RRD eyes compared to fellow and control eyes. Decreased VSD and VD in all layers (3 ×3 mm and 6 ×6 mm) were significantly associated with greater preoperative extent of retinal detachment (P < 0.05) and poorer postoperative best-corrected visual acuity (BCVA) in RRD eyes (P < 0.05). Macula-off status was associated with increased foveal avascular zone irregularity (12 ×12 mm, P = 0.02). CONCLUSIONS: Decreased VD on WF SS-OCTA is associated with poorer postoperative BCVA following RRD repair. [Ophthalmic Surg Lasers Imaging Retina 2024;55:310-317.].

Widefield, Swept-Source Optical Coherence Tomography Angiography Metrics as Predictors of Anti-VEGF Treatment in Retinal Vein Occlusions.

BACKGROUND AND OBJECTIVE: Our objective was to assess baseline widefield swept-source optical coherence tomography angiography (WF SSOCTA) microvascular metrics as predictors for the number of anti-vascular endothelial growth factor (VEGF) injections and visual acuity (VA) at 12-months follow-up in patients with retinal vein occlusion (RVO). PATIENTS AND METHODS: This was a prospective study including 49 RVO eyes from 49 patients who had not received an anti-VEGF injection for at least 3 months prior to imaging. Microvascular metrics from 6×6-mm and 12×12-mm angiograms were assessed using linear regression models, adjusting for age. RESULTS: Reductions in the vessel density (VD) and vessel skeletonized density (VSD) vascular metrics were associated both with a higher number of anti-VEGF injections at all follow-up time points and reduced VA 12 months after imaging in all RVO eyes. CONCLUSIONS: WF SS-OCTA VD and VSD micro-vascular metrics at baseline can prognosticate VA and number of anti-VEGF injections required at 3, 6, and 12 months in RVO eyes. [Ophthalmic Surg Lasers Imaging Retina 2024;55:xx-xx.].

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.].

Changes in wider field swept-source OCT angiography vascular metrics with anti-vascular endothelial growth factor therapy in central retinal vein occlusion.

PURPOSE: To investigate the impact of anti-VEGF therapy on vascular metrics in eyes with macular edema secondary to central retinal vein occlusion (CRVO) using wider field swept-source OCT angiography (WF SS-OCTA). METHODS: We included 23 eyes with macular edema associated with non-ischemic CRVO from 22 patients treated with anti-VEGF therapy (median number of injections: 5 [2-9]). Changes in vessel density (VD), vessel skeletonized density (VSD), and foveal avascular zone (FAZ) parameters were measured using WF SS-OCTA. Visual acuity (VA) and central subfield thickness (CST) were also measured. RESULTS: Median CST decreased significantly from 369 µm (305-531) to 267 µm (243-300, p < 0.001). VD and VSD parameters in 12 × 12 mm images showed significant reductions. For instance, VSD in the whole retina decreased from a median of 13.37 (11.22-13.74) to 11.29 (9.36-12.97, p = 0.013). Additionally, a significant increase in FAZ circularity was found, suggesting improved microvascular integrity. Significant inverse correlations were found between the number of anti-VEGF injections and all VSD and VD parameters on the 12 × 12 mm images (p < 0.05). Notably, the reductions in VSD and VD on 12 × 12 mm angiograms in the deep capillary plexus (DCP) after each injection significantly correlated with increased logMAR VA (worse VA). CONCLUSION: Anti-VEGF therapy in CRVO patients not only mitigates macular edema but also alters the overall microvascular morphology and functionality as revealed by WF SS-OCTA.

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.

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.].

A Cost-Effectiveness Analysis of Pegcetacoplan for the Treatment of Geographic Atrophy.

PURPOSE: To evaluate the cost-effectiveness of the treatment of geography atrophy (GA) with intravitreal pegcetacoplan and to identify utility-measurement surrogates. DESIGN: Cost analysis based on data from a published study. SUBJECTS: None; based on data from published sham control compared with 2 treatment groups in the index study. METHODS: Costs were based on 2022 Medicare reimbursement data. Specific outcomes were extrapolated from the DERBY and OAKS trials. Assumptions were made for the lifetime analysis based on a theoretical logistic growth model of the atrophy. OUTCOME MEASURES: Cost, cost utility, cost per quality-adjusted life-year, and cost per area of GA (in US$). RESULTS: The costs to treat GA in every month (EM) and every-other-month (EOM) treatment groups over the 2 years as reported were $70 000 and $34 600, respectively. The costs per area of delaying GA for 2 years in all patients were $87 300/mm2 (EM) and $49 200/mm2 (EOM), and in initially extrafoveal patients, $53 900/mm2 (EM) and $32 100/mm2 (EOM). The costs per day of delaying GA for 2 years were $295 (EM) and $170 (EOM); the marginal cost (EM vs. EOM) per retinal pigment epithelium cell saved was $30. The modeled lifetime costs were $350 000 (EM) and $172 000 (EOM), or $309 000/mm2 (EM) and $180 000 (EOM) /mm2. The modeled time to 95% atrophy at 13 years was delayed by 2.5 years (EM) and 2.1 years (EOM). The costs/quality-adjusted life-year gained based on modeled visual loss with 95% atrophy were $706 000 (EM) and $397 000 (EOM). CONCLUSION: Treatment of GA with intravitreal pegcetacoplan EOM was more cost effective than EM. Treatment of extrafoveal lesions yielded greater utility than the treatment of the entire group. As atrophy progression approaches an upper limit, the marginal cost/benefit ratios increase. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Longitudinal Assessment of Macular Thickness and Microvascular Changes in Children with Sickle Cell Disease.

PURPOSE: To longitudinally assess macular thickness and microvascular changes in children with sickle cell disease (SCD). DESIGN: A retrospective consecutive series. SUBJECTS: Children with SCD aged ≤ 18 years who had an ophthalmic examination at Boston Children's Hospital between January 1998 and August 2022. METHODS: Qualitative and quantitative analyses of both OCT and OCT angiography (OCTA) images were performed. MAIN OUTCOME MEASURES: Total retinal thickness measured on macular OCT, superficial capillary plexus and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6- × 6-mm OCTA scans. RESULTS: International Classification of Diseases, 10th Revision, code search identified 303 pediatric SCD patients who underwent ophthalmic examination during the study period. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit, respectively. Overall, temporal retinal thinning was noted qualitatively in 35.6% of SCD patients at presentation and 39.6% at final visit. Of those patients with macular thinning, 94.6% and 90.5% had peripheral sickle cell retinopathy (SCR) at presentation and final visit. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in the fovea and temporal parafovea compared with HbSC (P < 0.05). Eyes with peripheral SCR had a larger FAZ at presentation compared with eyes without peripheral SCR (P = 0.004), a lower DCP VD at final visit in the inferior temporal macula (P = 0.03), and a higher DCP VD at final visit in the superior nasal macula (P = 0.01). Eighty eyes of 40 patients had OCT, and 34 eyes of 20 patients had both OCT and OCTA images acquired at both initial and final visits. At final visit, retinal thickness decreased at the fovea, inferior perifovea, and temporal perifovea compared with presentation (P < 0.05). In parallel, VD DCP in the superonasal quadrant increased at final visit (P = 0.03). CONCLUSIONS: Macular retinal thinning was progressive and observed in eyes with and without peripheral SCR. Over time, there was a compensatory increase in DCP VD in the nasal macula on OCTA. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.