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Landon J Rohowetz,1 Sayena Jabbehdari,2 Nicolas A Yannuzzi,1 Jayanth Sridhar,1 William E Smiddy,1 Audina M Berrocal,1 Thomas A Albini,1 Justin H Townsend,1 Jorge A Fortun,1 Harry W Flynn Jr1 1Department of Ophthalmology, Bascom Palmer Eye Institute, Miami, FL, USA; 2Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, AR, USACorrespondence: Harry W Flynn Jr, Tel +1 305 326 6118, Fax +1 351 207 3928, Email hflynn@med.miami.eduPurpose: To evaluate the outcomes and complications in patients with retained lens fragments (RLF) after cataract surgery, comparing those who received pars plana vitrectomy (PPV) on the same day, within 1 week, or later than 1 week after cataract surgery.Patients and Methods: Retrospective case series of all patients who underwent PPV for RLF at Bascom Palmer Eye Institute between January 1, 2012 and September 30, 2020. Individuals with less than 3 months of follow-up, chronic uveitis, congenital cataract, previtrectomy retinal detachment (RD), and severe trauma were excluded. All analyses for categorical and binary variables used chi-squared tests. Analyses for continuous variables were performed using multivariate analyses of covariance, adjusting for differences in baseline visual acuity before cataract surgery.Results: The study included 246 eyes of 246 patients. The timing distribution included the following: 140 (57%) eyes underwent same-day PPV, 33 (13%) eyes underwent same-week PPV, and 73 (30%) eyes underwent PPV after 1 week (up to 90 days). When all eyes were included in analysis, there were no statistically significant differences in mean best-corrected visual acuity (BCVA) between groups at last follow-up examination (P = 0.07). When only eyes without known pre-existing ocular disease (N = 157) were included in analysis, there were no differences in mean BCVA between groups at all postoperative timepoints (P > 0.05). The rate of RD did not differ between groups when eyes with and without pre-existing ocular disease were analyzed (P > 0.05).Conclusion: In the current study, there were no statistically significant differences in postoperative BCVA or rates of RD at last follow-up examination in eyes undergoing PPV for RLF on the same day, within 1 week, or later than 1 week after cataract surgery.Keywords: retained lens fragments, pars plana vitrectomy, cataract surgery, retinal detachment

Jason Fan, Julia L Hudson, Parastou Pakravan, Thomas A Lazzarini, Benjamin R Lin, Kenneth C Fan, Nicolas A Yannuzzi, Jayanth Sridhar, Justin H Townsend, Audina M Berrocal, William E Smiddy, Elizabeth A Vanner, Harry W Flynn Jr Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Miami, FL, USACorrespondence: Harry W Flynn Jr, Bascom Palmer Eye Institute, 900 NW 17th St, Miami, FL, 33136, USA, Email HFlynn@med.miami.eduObjective: The purpose of the current study is to report outcomes of suprachoroidal hemorrhage (SCH) after anterior segment surgery at a single institution, and to identify clinical features associated with visual prognosis.Methods and Analysis: Retrospective consecutive case series of patients with SCH occurring after anterior segment surgery.Results: The study includes 112 eyes of 112 patients between 2014 and 2020. There were 76 cases of non-appositional SCH versus 36 cases of appositional SCH. The mean presenting visual acuity for patients with non-appositional versus appositional SCH was 2.03 logMAR (SD 0.78) versus 2.39 logMAR (SD 0.43), respectively. Visual acuity outcomes generally remained poor at last follow-up: 64 (58%) patients had a visual acuity (VA) of ≤ 20/200, including 19 (17%) with light perception (LP), and 11 (10%) with no light perception (NLP). Regarding management of non-appositional versus appositional SCH, observation was selected in 46 (61%) vs 12 (33%), delayed drainage in 14 (18%) vs 15 (42%), delayed pars plana vitrectomy in 16 (21%) vs 13 (36%), and VA at last follow-up was 1.2 versus 1.86 logMAR (p=0.002). In patients that were observed, both appositional SCH (p=0.01) and duration of apposition (p=0.04) were correlated with worse outcome.Conclusion: Appositional SCH was associated with poorer visual outcomes compared to non-appositional SCH. Observation remains a reasonable management strategy for non-appositional SCH.Keywords: suprachoroidal hemorrhage, non-appositional, appositional, anterior segment surgery

Rehan M Hussain,1 Bilal A Shaukat,1,2 Lauren M Ciulla,3 Audina M Berrocal,4 Jayanth Sridhar4 1Retina Associates Ltd, Elmhurst, IL, USA; 2Department of Ophthalmology, Cook County Hospital, Chicago, IL, USA; 3Northwestern University Feinberg School of Medicine, Chicago, IL, USA; 4Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, 33136,USACorrespondence: Rehan M HussainRetina Associates Ltd, 133 E Brush Hill Road, Suite 300, Elmhurst, IL, 60126, USATel +1 630-288-5540Fax +1 630-571-5679Email Rhussain27@gmail.comAbstract: Neovascular age-related macular degeneration (nAMD) treatment has been revolutionized by the introduction of vascular endothelial growth factor antagonists (anti-VEGF), but the need for frequent intravitreal injections poses a heavy burden to patients and physicians. Evolving anti-VEGF therapies include longer duration agents, approaches that target multiple pathways, topical anti-VEGF agents, sustained-release, and genetic therapies. Abicipar pegol, a designed ankyrin repeat protein (DARPin), demonstrated the ability to maintain stable visual acuity with 12-week dosing, but was not approved by the FDA due to higher than usual rates of intraocular inflammation. Conbercept, a recombinant anti-VEGF fusion protein, has been approved in China, and is in Phase 3 trials globally. KSI-301 is an anti-VEGF antibody biopolymer conjugate that allowed 66% of nAMD patients to maintain at least a 6-month treatment-free interval in Phase 1b studies. OPT-302, an inhibitor of VEGF-C/D, will be tested in phase 3 studies that compare anti-VEGF-A monotherapy against combination therapy with OPT-302. Faricimab is a bispecific anti-VEGF/Ang-2 antibody that upregulates the Tie-2 signaling pathway and promotes vascular stability; it is undergoing phase 3 trials with potential for 12- or 16-week dosing. PAN-90806 is a topical anti-VEGF agent that showed the ability to reduce injection frequency by 79% compared to ranibizumab monotherapy in a phase 1/2a trial. Sustained-release anti-VEGF therapies include the ranibizumab Port Delivery System (in phase 3 studies), GB-102 (Phase 2b), OTX-TKI (phase 1), and Durasert (preclinical). Suprachoroidal delivery of the tyrosine kinase inhibitor, axitinib, is in preclinical studies. Genetic therapies in phase 1 studies include RGX-314 and ADVM-022, which introduce a viral vector that modifies the retina’s cellular apparatus to create an anti-VEGF biofactory, potentially serving as a one-time treatment. Further investigation is warranted for drugs and delivery systems that hope to advance visual outcomes and reduce treatment burden of nAMD.Keywords: ADVM-022, age-related macular degeneration, abicipar pegol, CLS-AX, conbercept, faricimab, GB-102, KSI-301, PAN-90806, OTX-TKI, ranibizumab port delivery system, RGX-314, VEGF

Supalert Prakhunhungsit,1 Audina M Berrocal2 1Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand; 2Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, FL, USACorrespondence: Audina M BerrocalBascom Palmer Eye Institute, Miller School of Medicine, University of Miami, 900 Northwest 17th Street, Floor 1, Miami, FL 33136, USATel +1 305-326-6000Email aberrocal@med.miami.eduAbstract: Persistent fetal vasculature (PFV), previously known as persistent hyperplastic primary vitreous, is a developmental malformation of the eyes that is caused by a failure of the hyaloid vasculature to regress in utero. PFV has been reported for decades; however, our understanding of the pathophysiology/pathogenesis of PFV, and the diagnostic and treatment modalities for PFV have evolved over time, and these advancements have improved diagnosis, treatment, and outcomes. However and in spite of these advancements, the heterogeneity of this disease continues to make PFV a diagnostic challenge. Here, we review what is currently known about various important aspects of PFV to update and enhance the knowledge of ophthalmologists who encounter and manage PFV in clinical practice.Keywords: diagnostic and management strategies, persistent fetal vasculature, PFV, current insights

Nicolas A Yannuzzi, Nimesh A Patel, Audina M Berrocal, Jayanth Sridhar Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL 33136, USACorrespondence: Jayanth SridharDepartment of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Miller School of Medicine, 900 NW 17th Street, Miami, FL 33136, USATel +1 305 326-6000Email jsridhar119@gmail.comPurpose: To report a modified surgical technique for chandelier endoillumination-assisted scleral buckling using an endolaser.Methods: A three patient case reports for demonstration of surgical technique.Results: All patients underwent successful primary retinal detachment repair without any intraoperative or post-operative complications.Conclusion: Endolaser can be employed during chandelier endoillumination assisted scleral buckling either as a standalone tool or in conjunction with cryopexy, especially in cases with posteriorly located retinal breaks.Keywords: rhegmatogenous retinal detachment, scleral buckle, chandelier, endoillumination, endolaser

Karen Brown, Nicolas A Yannuzzi, Natalia F Callaway, Nimesh A Patel, Nidhi Relhan, Thomas A Albini, Audina M Berrocal, Janet L Davis, Jorge A Fortun, William E Smiddy, Jayanth Sridhar, Harry W Flynn Jr, Justin H Townsend Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USACorrespondence: Justin H TownsendDepartment of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17th Street, Miami, FL 33136, USATel +1305 326-6000Fax +1305 326-6417Email jtownsend@med.miami.eduPurpose: The purpose of this study is to investigate associations with and surgical outcomes of rhegmatogenous retinal detachment (RRD) in young adults.Methods: This is a retrospective consecutive case series of patients aged 18–30 years who underwent surgical repair for RRD between January 1, 2014 and December 1, 2016 at a university referral center.Results: The current study includes 38 eyes with RRD. Documented high or moderate myopia was present in 28 (74%) eyes. Connective tissue disease was present in 3 (8%) eyes. Median pre-operative visual acuity (VA) was 20/70. Surgery was performed via scleral buckle (SB) alone in 27/38 (71%) and via combined SB and pars plana vitrectomy (SB/PPV) in 11/38 (29%) eyes. Single surgery anatomical success (SSAS) rate was 20/27 (74%) for SB and 7/11 (64%) for SB/PPV. The retina was reattached at last follow-up in 25/27 (93%) for SB and 11/11 (100%) for SB/PPV. The median postoperative VA was 20/40.Conclusion: In the current study of young adults with RRD, the most common association was high or moderate myopia. Visual and anatomic outcomes at last follow-up were generally favorable.Keywords: myopia, rhegmatogenous retinal detachment, RRD, young adults

Marianeli Rodriguez, James Lin, Justin H Townsend, William E Smiddy, Thomas A Albini, Audina M Berrocal, Jayanth Sridhar, Harry W Flynn Jr Department of Ophthalmology, Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, FL, USA Purpose: The purpose of this study was to report clinical features and outcomes in patients with giant retinal tears (GRTs) undergoing vitreoretinal surgery and to compare data from this contemporary series to a previous study from the same institution. Materials and methods: A retrospective, interventional, consecutive case series was conducted for all patients diagnosed with GRTs who underwent vitreoretinal surgery between January 2011 and August 2017. Intraoperative data including the use of scleral buckling, perfluorocarbon liquid, and intraocular tamponade were collected and compared according to GRT size. These parameters, along with postoperative anatomic success and best-corrected visual acuity (BCVA), were compared with the previous series. Results: The study included 80 eyes of 79 patients with a presentation of retinal detachment with a GRT. Management approach for repair of GRT-associated retinal detachments included scleral buckle (SB) alone (three eyes, 4%), pars plana vitrectomy (PPV) (16 eyes, 20%), and combined SB/PPV (61 eyes, 76%). Perfluorocarbon liquids were used in 60/77 eyes (78%) undergoing PPV, and silicone oil was used in 54/77 eyes (70%). Single surgery success rate was in 69/80 eyes (86%). Eyes managed with SB (including SB alone and SB/PPV) or PPV without SB had similar rates of recurrent retinal detachment (16% vs 6%; P=0.33). Anatomic success was achieved in 76/80 eyes (95%) with one or more surgical procedures, and 54/80 eyes (68%) achieved postoperative BCVA of ≥20/400. Conclusions: In the current series, repairs of retinal detachment due to GRTs were most commonly managed with combined PPV/SB and perfluorocarbon liquid, resulting in reasonably generally favorable anatomic and visual outcomes. Keywords: giant retinal tears, perfluorocarbon liquid, retinal detachment, vitrectomy

Victor M Villegas,1 Aaron S Gold,1 Audina M Berrocal,2 Timothy G Murray11Ocular Oncology and Retina, Miami, FL, USA; 2Department of Ophthalmology, Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, FL, USAPurpose: To evaluate the impact of intravitreal bevacizumab combined with laser vascular ablation in the management of advanced Coats’ disease presenting with exudative retinal detachment.Methods: This was a retrospective review of 24 children that presented with exudative retinal detachments associated with advanced Coats’ disease. Mean patient age was 62 months (range 9–160 months). Presenting signs included retinal detachment in 24 children (100%), vascular telangiectasia in 24 children (100%), and retinal ischemia in 24 children (100%). Twenty of 24 children presented with elevated, vascular leakage in the fovea (83%). Two children presented with sub-retinal fibrosis associated with presumed long-standing retinal detachment without evidence of rhegmatogenous retinal detachment. Ten patients exhibited vascular alterations in the periphery of the second eye without clinical evidence of exudation. All 24 children were treated with a large-spot-size diode laser directly to areas of abnormal telangiectatic vasculature. All 24 children received intravitreal bevacizumab injection. Results: All 24 children had resolution of exudative retinal detachment, ablation of vascular telangiectasia, and anatomic improvement of the retina. No child exhibited progressive retinal detachment and no eye required enucleation. No cases of neovascular glaucoma were seen. Fellow eyes with peripheral vascular alterations showed no progression to exudative vasculopathy during the observation period. Intravitreal bevacizumab injection was not associated with endophthalmitis or systemically-observed complications.Conclusion: Repetitive intravitreal bevacizumab combined with laser vascular ablation may be utilized effectively for advanced Coats’ disease presenting with exudative retinal detachment.Keywords: Coats’ disease, bevacizumab, anti-VEGF, laser ablation, retina

Marco A Gonzalez, Harry W Flynn Jr, William E Smiddy, Thomas A Albini, Audina M Berrocal, Paul Tenzel Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA Purpose: To evaluate management strategies and outcomes for patients with giant retinal tear (GRT)-associated retinal detachment (RD) that had undergone previous pars plana vitrectomy (PPV). Methods: A noncomparative consecutive case series between January 2005 and July 2010. Patients with a preceding history of PPV undergoing retinal reattachment surgery for GRTs were identified. Results: Using International Classification of Diseases 9 codes for GRTs, 227 cases were identified. A total of eight eyes in eight patients were identified as having had preceding PPV for non-RD-related pathology. The mean age was 45.5 (range of 10–79) years. The mean time between PPV and diagnosis of GRT was 2.4 months. The mean follow-up after RD surgery was 24.3 months. Presenting visual acuity was 20/400 or better in four of eight patients (50%). All patients underwent repeat PPV with either gas or oil tamponade. A scleral buckling procedure was performed in seven patients (88%). Perfluorocarbon liquid was used during reattachment surgery in four patients (50%). Although anatomic success was achieved in all patients, visual acuity at last follow-up was 20/400 or better in 6 patients (75%). Conclusion: GRTs are an uncommon complication of PPV. The majority of patients underwent repeat PPV, scleral buckling procedure, perfluorocarbon liquid use and silicone-oil tamponade. Patients presenting with GRT-associated RD after PPV undergoing additional surgery achieved high rates of anatomic success, but visual outcomes were variable. Keywords: giant retinal tear, retinal detachment, vitrectomy, perfluorocarbon liquids

Jonathan H Tzu, Harry W Flynn Jr, Audina M Berrocal, William E Smiddy, Timothy G Murray, Yale L FisherDepartment of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USAPurpose: The purpose of this retrospective study was to evaluate the characteristic features, including spectral-domain optical coherence tomography (SD-OCT), clinical course, and outcome of treatment if given for patients with optic disc pit maculopathy.Methods: We investigated a consecutive series of patients with a diagnosis of optic pit maculopathy treated between 2001 and 2012 at the Bascom Palmer Eye Institute. Patients were divided into two main groups, ie, patients who were observed without surgery and patients who received surgical intervention. The main outcome measures were presenting and final visual acuity, and changes in SD-OCT imaging were recorded. Other data including age, gender, eye, age of onset, length of follow-up, location of optic pit, and location of fluid by OCT were also recorded.Results: On OCT, 67% (12/18) of the eyes showed schisis-like cavities, 22% (4/18) had only subretinal fluid, and 17% (3/18) had only a schisis-like cavity without subretinal fluid. In the patients managed by observation, visual acuity was ≥20/200 in 6/8 eyes initially and 6/8 eyes at last follow-up. Ten of 18 patients received either focal laser, surgery or both. Six of 10 eyes undergoing surgery had initial visual acuity ≥ 20/200, and 8 of 10 eyes undergoing surgery had a visual acuity of ≥20/200 at last follow-up.Conclusion: In this study, many eyes were observed and remained stable during follow-up. In eyes with reduced vision, surgical intervention produced variable outcomes, and persistent intraretinal/subretinal fluid was a common occurrence.Keywords: optic pit maculopathy, spectral-domain optical coherence tomography

Hassan A Aziz1, Audina M Berrocal1,2, Robert A Sisk1, Kristin Hartley1, Magaly Diaz-Barbosa2, Rose A Johnson2, Ditte Hess1, Sander R Dubovy1, Timothy G Murray1, Harry W Flynn Jr11Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 2Jackson Memorial Hospital, Miami, FL, USABackground: The purpose of this study was to report on the incidence and treatment outcomes of endogenous endophthalmitis among newborns in the neonatal intensive care unit (NICU) of a single medical center.Methods: This was a noncomparative, retrospective case series of endogenous endophthalmitis among infants at the Jackson Memorial Hospital NICU treated between March 1, 2002 and March 1, 2007.Results: Of 4323 infants admitted to the NICU, seven eyes of six (0.139%) infants (two males, four females) were diagnosed with endophthalmitis during the study period. Four patients were born prematurely with a mean gestational age of 27.5 weeks and a mean birth weight of 1153 g. Retinopathy of prematurity was reported in two of the six patients. Mean follow-up was 3.5 years. The diagnosis was confirmed by positive cultures or polymerase chain reaction testing at a median age of 34 postnatal days. Positive cultures included Candida albicans (n = 4), Pseudomonas aeruginosa (n = 1), and Herpes simplex type 2 (n = 1). All patients received systemic treatment and five received adjunctive ophthalmic interventions, including intravitreal antibiotics in five eyes of four patients and vitrectomy with pars plana lensectomy in three eyes. One patient underwent primary enucleation and another had delayed evisceration. In the remaining five eyes, there was a normal appearing posterior segment and normal intraocular pressures at last follow-up.Conclusion: Endogenous endophthalmitis is a rare complication in infants in the NICU, but may occur in patients with candidemia, bacteremia, retinopathy of prematurity, and low birth weight. Despite early and appropriate treatment, involved eyes may have poor outcomes.Keywords: endogenous endophthalmitis, neonatal intensive care unit

David J Jacobs,1 Julia Sein,1 Audina M Berrocal,1 Alana L Grajewski,1,2 Elizabeth Hodapp11Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, 2Department of Ophthalmology, University of Minnesota, Minneapolis, MN, USAAbstract: The purpose of this report is to describe the fluorescein angiography findings in a case of Rubinstein-Taybi syndrome. Fundus photography and fluorescein angiography were performed on a 6-year-old male with Rubinstein-Taybi syndrome due to CREB binding protein gene mutation. Fundus photography showed glaucomatous cupping and diffusely attenuated retinal vasculature. Choroidal vasculature was prominent due to diffuse retinal atrophy with scattered focal retinal pigment epithelial changes. Fluorescein angiography showed retinal vascular attenuation, prolonged arteriovenous transit time with delayed venous filling, late small vessel leakage, and 360 degrees of peripheral avascularity. Peripheral retinal avascularity and retinal vascular inflammation evidenced by late small vessel leakage can be demonstrated by fluorescein angiography in the retinal dystrophy of Rubinstein-Taybi syndrome.Keywords: Rubinstein-Taybi syndrome, CREB binding protein, gene mutation

Purpose: To report the clinical features and outcomes in eyes that underwent vitreoretinal surgery for complications of concurrent sickle cell retinopathy and diabetic retinopathy., Methods: Retrospective, consecutive case series of all eyes that underwent vitreoretinal surgery for complications secondary to concurrent sickle cell retinopathy and diabetic retinopathy between January 01, 2014, and December 31, 2021., Results: The study included 20 eyes of 14 patients. Indications for surgery included tractional retinal detachment in 12 eyes (60%), combined tractional retinal detachment/rhegmatogenous retinal detachment in 6 eyes (30%), and vitreous hemorrhage in 2 eyes (10%). Pars plana vitrectomy was performed in all eyes. One eye received a scleral buckle at the same time as pars plana vitrectomy. There was no change in mean best-corrected visual acuity at the last follow-up examination (1.5 [20/678]) when compared with mean preoperative best-corrected visual acuity (1.6 [20/762], P = 0.83). Preoperative best-corrected visual acuity correlated with postoperative best-corrected visual acuity at the last follow-up examination in eyes with retinal detachment (r = 0.49, P = 0.04). Single operation anatomic success was achieved in 11 of 17 eyes (65%) with retinal detachment., Conclusion: Functional and anatomic outcomes after surgery in eyes with concurrent sickle cell retinopathy and diabetic retinopathy are relatively poor.

Purpose: To report the clinical presentation and outcomes in patients who underwent surgery for proliferative sickle cell retinopathy (PSCR)., Design: Retrospective, consecutive case series., Subjects: All patients who underwent vitreoretinal surgery for complications secondary to PSCR between January 1, 2014, and December 31, 2021, at a university referral center., Methods: Retrospective consecutive case series., Main Outcome Measures: Best-corrected visual acuity (BCVA), single operation anatomic success rate., Results: The study included 65 eyes of 61 patients. Disease distribution included 24 (44.4%) eyes with hemoglobin SC disease, 14 (25.9%) with hemoglobin SS disease, 13 (24.1%) with sickle cell trait, and 3 (5.6%) with sickle cell-β thalassemia. Preoperative transfusion was not performed in any study patients. Regional anesthesia with monitored anesthesia care (RA-MAC) was utilized in 58 (89.2%) eyes and general anesthesia in 7 (10.8%). In eyes that underwent surgery for retinal detachment (RD; N = 52) the rate of single operation anatomic success was 72.4% with combined scleral buckling/pars plana vitrectomy (SB/PPV; N = 29) compared with 47.8% with PPV alone (N = 23; P = 0.07). Mean BCVA at the last follow-up examination was 1.27 (20/372) in the SB/PPV group and 1.05 (20/226) in the PPV group (P = 0.48). In all SB cases, an encircling band was utilized and there were no known cases of anterior segment ischemia. All eyes that had surgery for vitreous hemorrhage (N = 13) underwent PPV with endolaser and mean BCVA improved from 1.67 (20/944) preoperatively to 0.45 (20/56) at last follow-up examination (P < 0.001). Mean preoperative BCVA, indication for surgery, single operation success rate, and mean BCVA at last follow-up examination did not differ based on sickle cell disease type (P > 0.05)., Conclusions: In patients with RD, SB/PPV achieved slightly higher rates of single operation anatomic success compared with PPV alone. Visual acuity outcomes were similar in the 2 groups. The majority of patients received RA-MAC anesthesia and preoperative transfusions were not performed. There were no cases of postoperative anterior segment ischemia. Hemoglobin SC disease was the most common disease type in the current study and surgical outcomes did not differ between sickle cell disease types., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

This report describes a unique case of a Coats-like presentation of familial exudative vitreoretinopathy in an 11-year-old girl. The patient was originally referred for evaluation of presumed Coats disease and presented with telangiectatic vessels, perivascular exudates, diffuse peripheral exudation, and intraretinal hemorrhages. Clinical and angiographical findings were consistent with familial exudative vitreoretinopathy, while genetic testing identified variants of uncertain significance in two associated genes, LRP5 and ZNF408 . In silico analysis predicts the LRP5 variant to be pathogenic. Retinal vasculopathies often have phenotypic overlap, warranting angiographic examination of both eyes and genetic testing to uncover the correct diagnosis and guide proper treatment. [ Ophthalmic Surg Lasers Imaging Retina 2024;55:462-466.] .

Introduction: Familial Exudative Vitreoretinopathy (FEVR) is a heritable retinal vascular disease characterized by incomplete vascularization of the peripheral retina resulting in ischemia. Fifty percent of FEVR cases 10 are due to known pathogenic genetic variants, and disease phenotype can vary greatly. FEVR is a clinical diagnosis, however, genetic testing can play a key role in screening for FEVR in genetically susceptible populations, thus leading to early treatment and improved patient outcomes., Case: A 2-year-old male with no known past ocular or medical history was diagnosed with FEVR upon examination under anesthesia and multimodal retinal imaging. Genetic testing identified a Jagged 1 ( JAG1 ) variant of uncertain significance, 15 which has been linked to FEVR in recent studies. Despite close follow-up and treatment, the patient experienced a funnel retinal detachment in the right eye approximately one year after diagnosis., Discussion: This case in conjunction with recent literature suggests that JAG1 variants are likely associated with FEVR. Further investigations are necessary to identify the frequency of JAG1 variants among patients with FEVR. Robust understanding of FEVR's heterogenous genetic profile will lead to improved treatment modalities 20 and patient outcomes.

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., (© 2024 Published by Elsevier Inc. on behalf of the American Academy of Ophthalmology.)

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.] .

Background: Choroidal neovascularization (CNV) is a rare complication of choroideremia that occurs secondary to relative atrophy of the retinal pigment epithelium and eventual rupture of Bruch's membrane. The ideal management of CNV in choroideremia is unclear., Materials and Methods: Case report., Observations: A 14-year-old male with no known ocular history presented to the eye emergency department complaining of a central scotoma in the right eye for 4 days. He had no past medical history and family history was unremarkable for known ocular disease. Visual acuity was 20/70 in the right eye and 20/30 in the left eye. Posterior segment exam revealed chorioretinal atrophy extending from the outer macula to the midperiphery in both eyes. There was CNV with associated subretinal hemorrhage in the right eye. Optical coherence tomography demonstrated the presence of CNV with subretinal fluid in the right eye and parafoveal outer retinal atrophy in both eyes. Genetic testing revealed a hemizygous exon 2 deletion on the CHM gene, pathogenic for choroideremia. The patient received a total of 3 injections 4 weeks apart followed by 1 injection 6 weeks later with resolution of the subretinal hemorrhage and reduction in CNV size with improvement in visual acuity to 20/20 at last follow-up exam., Conclusions and Importance: Choroidal neovascularization is a rare cause of central vision loss in patients with choroideremia. In this report, we demonstrate a good functional and anatomic response to intravitreal bevacizumab in a 14-year-old patient with undiagnosed choroideremia who presented with CNV-induced central vision loss.

Purpose: Advancements in retinal imaging have augmented our understanding of the pathology and structure-function relationships of retinal disease. No single diagnostic test is sufficient; rather, diagnostic and management strategies increasingly involve the synthesis of multiple imaging modalities. Methods: This literature review and editorial offer practical clinical guidelines for how the retina specialist can use multimodal imaging to manage retinal conditions. Results: Various imaging modalities offer information on different aspects of retinal structure and function. For example, optical coherence tomography (OCT) and B-scan ultrasonography can provide insights into the microstructural anatomy; fluorescein angiography (FA), indocyanine green angiography (ICGA), and OCT angiography (OCTA) can reveal vascular integrity and perfusion status; and near-infrared reflectance and fundus autofluorescence (FAF) can characterize molecular components within tissues. Managing retinal vascular diseases often includes fundus photography, OCT, OCTA, and FA to evaluate for macular edema, retinal ischemia, and the secondary complications of neovascularization (NV). OCT and FAF play a key role in diagnosing and treating maculopathies. FA, OCTA, and ICGA can help identify macular NV, posterior uveitis, and choroidal venous insufficiency, which guides treatment strategies. Finally, OCT and B-scan ultrasonography can help with preoperative planning and prognostication in vitreoretinal surgical conditions. Conclusions: Today, the retina specialist has access to numerous retinal imaging modalities that can augment the clinical examination to help diagnose and manage retinal conditions. Understanding the capabilities and limitations of each modality is critical to maximizing its clinical utility., Competing Interests: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr. Berrocal has financial interests in Aerie Pharmaceuticals, ProQR therapeutics, Oculus Surgical, Alcon, Allergan, Bayer, DORC, Phoenix Clinical, Vizunex Medical Systems, Zeiss, and Novartis. Dr. Modi is a consultant to Alimera, Alcon, Allergan/Abbvie, Bausch + Lomb, DORC, EyePoint, Genentech, Iveric Bio, Regeneron, Thea, and Zeiss. Dr. Wykoff receives consulting fees/honoraria for ongoing services provided as a consultant to 4DMT, AbbVie, Adverum, Aerie, AGTC, Alcon, Annexon, Apellis, Arrowhead, Bausch + Lomb, Boehringer Ingelheim, Cholgene, Clearside, Curacle, EyePoint, Foresite, Frontera, Genentech, Iveric Bio, Janssen, Kato, Kiora, Kodiak, Kriya, Merck, Nanoscope, NGM, Notal Vision, Novartis, Ocular Therapeutix, OcuTerra, ONL, Opthea, Oxular, Palatin, PerceiveBio, Perfuse, Ray, RecensMedical, Regeneron, RegenXBio, Resonance, Roche, SciNeuro, Stealth, Surrozen, Suzhou Raymon, THEA, TissueGen, and Valo; received grants paid to his institution for ongoing research support as a principal investigator for trials sponsored by 4DMT, Adverum, AffaMed, Alexion, Alimera, Alkahest, Allgenesis, Amgen, Annexin, Annexon, Apellis, Asclepix, Bayer, Boehringer Ingelheim, Chengdu Kanghong, Clearside, Curacle, EyePoint, Gemini, Genentech, GlaxoSmithKline, Gyroscope, IONIS, iRENIX, Iveric Bio, Kodiak, LMRI, Nanoscope, Neurotech, NGM, Novartis, Ocular Therapeutix, Ocuphire, OcuTerra, Ophthotech, Opthea, Oxurion, Oxular, Oyster Point, PerceiveBio, Regeneron, RegenXBio, Roche, SamChunDang Pharm, Sandoz, UNITY, Verily, and Xbrane; and has stock options (not owner) from ongoing relationships as a consultant from the following private for-profit entities: ONL, PolyPhotonix, RecensMedical, TissueGen, Visgenx, and Vitranu. None of the other authors declared potential conflicts of interest with respect to the research, authorship, and/or publication of the article., (© The Author(s) 2024.)

Participants: This article includes 7293 infants (14 586 eyes) screened for ROP across 5 centers in the United States (Austin Retina Associates, Austin, TX; Bascom Palmer Eye Institute, Miami, FL; Beaumont Eye Institute, Royal Oak, MI; Massachusetts Eye and Ear, Boston, MA; and Stanford Byers Eye Institute, Stanford, CA)., Purpose: To analyze the incidence and timing of treatment requiring retinopathy of prematurity (ROP) in extremely small premature infants. We hypothesize that the smaller the infant by gestational age and birthweight, the higher their likelihood of requiring treatment for ROP., Design: Premature infants screened for Retinopathy of Prematurity from 2002-2022 were divided into cohorts based on the following criteria based on gestational age (GA) and birth weight (BW). "Micropremature infants" are infants born between 24-26 weeks GA and between 600-799 g BW. "Nanopremature infants" are born ≤ 24 weeks GA and ≤ 600 g BW., Methods: Retrospective chart review., Main Outcome Measures: The incidence and timing of treatment-requiring ROP., Results: We found that infants defined as nanopremature had a ∼63% chance of requiring treatment at an average postmenstrual age (PMA) of 36.6 weeks, whereas those defined as micropremature had a 30% chance of requiring treatment at an average PMA of 36.3 weeks. This significantly contrasts with the risk of all screened babies for ROP where the risk of requiring treatment was 8.5%., Conclusion: Micropremature and nanopremature infants are significantly more likely to require treatment for ROP. With demographic data matched to all 5 major US regions spanning the last decade, these results have the potential to inform neonatologists, pediatricians, and ophthalmologists of an important shift in the landscape of prematurity in the United States., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: Classify the appearance and quantify the growth rate of chorioretinal atrophy in patients who received voretigene neparvovec-rzyl (VN) for RPE65-mediated retinal degeneration., Design: Multicenter retrospective analysis., Subjects: Patients who underwent subretinal VN injection at 5 institutions and demonstrated posterior-pole chorioretinal atrophy., Methods: Ultrawidefield scanning laser ophthalmoscopy or color fundus photos were assessed before and after subretinal VN. Atrophy was defined as regions with ≥ 2 of the following: (1) partial or complete retinal pigment epithelial depigmentation; (2) round shape; (3) sharp margins; and (4) increased visibility of choroidal vessels. Atrophy was qualitatively classified into different subtypes. All atrophy was manually segmented. Linear mixed-effects models with random slopes and intercepts were fit using atrophy area and square root of atrophy area., Main Outcome Measures: Number of eyes with each atrophy pattern, and slopes of linear mixed-effects models., Results: Twenty-seven eyes from 14 patients across 5 centers developed chorioretinal atrophy after subretinal VN. A mean of 5.8 ± 2.7 images per eye obtained over 2.2 ± 0.8 years were reviewed, and atrophy was categorized into touchdown (14 eyes), nummular (15 eyes), and perifoveal (12 eyes) subtypes. Fifteen eyes demonstrated > 1 type of atrophy. Thirteen of 14 patients demonstrated bilateral atrophy. The slopes of the mixed-effects models of atrophy area and square root of atrophy area (estimate ± standard error) were 1.7 ± 1.3 mm 2 /year and 0.6 ± 0.2 mm/year for touchdown atrophy, 5.5 ± 1.3 mm 2 /year and 1.2 ± 0.2 mm/year for nummular atrophy, and 16.7 ± 1.8 mm 2 /year and 2.3 ± 0.2 mm/year for perifoveal atrophy. The slopes for each type of atrophy were significantly different in the square root of atrophy model, which best fit the data (P < 0.05)., Conclusions: Chorioretinal atrophy after subretinal VN for RPE65-mediated retinal degeneration developed according to a touchdown, nummular, and/or perifoveal pattern. Perifoveal atrophy grew the most rapidly, while touchdown atrophy grew the least rapidly. Understanding the causes of these findings, which are present in a minority of patients, merits further investigation., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To describe abnormalities of the optic nerve microvasculature in patients with persistent fetal vasculature (PFV) and their fellow eyes using OCT angiography (OCTA)., Design: Cross-sectional study., Subjects: Fifty eyes of 25 patients with PFV who underwent prospective imaging using supine OCTA during examination under anesthesia at Bascom Palmer Eye Institute from March 1, 2019, to December 31, 2022., Methods: OCT angiography images of the optic nerve of the included patients were analyzed with a primary focus on blood flow. Demographic, clinical, and treatment factors were compared with morphologic changes in the optic disc microvasculature., Main Outcome Measures: Prevalence of optic nerve microvascular abnormalities on OCTA in the affected and fellow eyes of patients with PFV., Results: A total of 50 eyes from 25 patients were reviewed, and 28% (7/25) met image quality criteria for OCTA analysis. Optic nerve OCTA showed a persistent hyaloid artery (PHA) in all (7/7) PFV eyes analyzed. Of these, flow on OCTA was detectable in 57% (4/7). A Bergmeister papilla was evident in 100% (25/25) fellow eyes, of which flow was detected in 68% (17/25). Fluorescein angiography (FA) demonstrated blood flow within the stalk in 40% (10/25) of PFV eyes and within the Bergmeister papilla in 25% (6/25) of fellow eyes. Similar findings of abnormal blood flow and presence of fibrovascular stalk were seen in both treatment-naïve and treated groups., Conclusions: OCT angiography allows for high-resolution visualization of subtle vascular abnormalities that are not readily apparent using RetCam FA and may serve as a useful noninvasive test to confirm the patency of the PHA and Bergmeister papilla in children. The results of the present study suggest that PFV may be a bilateral and asymmetric process., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim of this study was to describe two cases of multiple epithelial detachments (pigment epithelial detachments [PEDs]) occurring in otherwise young, healthy patients., Methods: The medical and imaging records of two cases were reviewed retrospectively., Results: Multiple serous nonvascularized PEDs were present in two young, healthy patients who underwent multimodal imaging. The first patient, a 38-year-old woman, was incidentally found to have a visual acuity of 20/20 in both eyes and multiple bilateral PEDs. Imaging and a systemic work-up was negative for secondary causes, and at 36 months, vision remained stable although some of the PEDs had coalesced. The second patient, a 32-year-old woman, presented with distortion and perceived scotomas in both eyes progressive over the course of 2 years. The visual acuity was 20/20, and multimodal imaging confirmed the presence of bilateral PEDs., Conclusion: Bilateral idiopathic multifocal retinal PEDs in otherwise healthy young adults is a rare condition. Longer term follow-up is needed to evaluate secondary complications and visual outcomes., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Purpose: This case report describes a central retinal vein occlusion in a healthy 12-year-old girl who developed retinal neovascularization at 24 years of age. To the knowledge of the authors, this is the longest time between a reported pediatric central retinal vein occlusion event and neovascularization., Methods: The patient underwent a full history, physical examination, and laboratory workup to determine potential risk factors contributing to the vascular event. Fundus photographs, optical coherence tomography, and fluorescein angiography were performed throughout the patient's treatment course., Results: Family history was noncontributory, but laboratory testing revealed a mildly elevated homocysteine level and homozygous C677T mutation in methylenetetrahydrofolate reductase. As a result, she was started on folate supplementation. The patient has had no further ocular or systemic thrombotic events to date., Conclusion: Pediatric patients presenting with central retinal vein occlusion should undergo a systemic workup and require long-term follow-up to avoid complications, such as intraocular hemorrhage, tractional retinal detachments, and neovascular glaucoma.

Background and Objective: To describe the multimodal imaging and histopathological features of patients with dragged optic disc vessels (DODV)., Patients and Methods: This is a retrospective, observational analysis using multimodal imaging of eyes with DODV in patients with retinal vascular diseases including familial exudative vitreoretinopathy, inflammatory disease, and others. In addition, two additional enucleated eyes with DODV underwent histopathological analysis., Results: Of the 13 patients, eight were girls and five were boys. Mean age was 5 years (ranging from 4 months to 10 years old). Of the 15 eyes, 12 (80%) demonstrated temporal dragging and three (20%) had nasal dragging. Retinal vascular abnormalities were present in 11 of the fellow eyes. Multimodal imaging demonstrated features of DODV including direction of traction, outer retinal thickening, increased flow, and other features. Only two (13.3%) eyes required surgical intervention. Mean follow-up was 14 (range 3 to 30 months) months. At last follow-up all eyes remained stable. Last visual acuity ranged from 20/100 to counting fingers. As well, two enucleated globes of adults with retinopathy of prematurity underwent histopathologic evaluation, showing optic nerve fibers that extended from the optic nerve into the DODV., Conclusions: DODV is a sign of various late-stage retinal vascular diseases, associated with poor visual function. Multimodal imaging and histopathology can assist in understanding the disease pathology. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:634-642.] .

Retinopathy of prematurity (ROP) is a leading cause of preventable vision loss in preterm infants. While appropriate screening is crucial for early identification and treatment of ROP, current screening guidelines remain limited by inter-examiner variability in screening modalities, absence of local protocol for ROP screening in some settings, a paucity of resources and an increased survival of younger and smaller infants. This review summarizes the advancements and challenges of current innovative technologies, artificial intelligence (AI), and predictive biomarkers for the diagnosis and management of ROP. We provide a contemporary overview of AI-based models for detection of ROP, its severity, progression, and response to treatment. To address the transition from experimental settings to real-world clinical practice, challenges to the clinical implementation of AI for ROP are reviewed and potential solutions are proposed. The use of optical coherence tomography (OCT) and OCT angiography (OCTA) technology is also explored, providing evaluation of subclinical ROP characteristics that are often imperceptible on fundus examination. Furthermore, we explore several potential biomarkers to reduce the need for invasive procedures, to enhance diagnostic accuracy and treatment efficacy. Finally, we emphasize the need of a symbiotic integration of biologic and imaging biomarkers and AI in ROP screening, where the robustness of biomarkers in early disease detection is complemented by the predictive precision of AI algorithms., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Purpose: To report the surgical approaches and outcomes in patients undergoing surgery for retinal detachment associated with retinal dialysis., Design: Retrospective, consecutive case series., Subjects: All patients who underwent surgery for retinal detachment secondary to retinal dialysis between January 1, 2012, and January 1, 2022., Methods: Retrospective consecutive case series., Main Outcome Measures: Best-corrected visual acuity (BCVA), single-operation success rate., Results: The study cohort included 60 eyes of 58 patients with a mean age of 26.4 (standard deviation, 13.0) years. Males comprised 49 (84.5%) patients. Known trauma occurred in 35 (61.4%) cases. Initial surgical management included scleral buckling (SB) in 49 (81.7%) eyes and combined SB and pars plana vitrectomy (PPV) in 11 (18.3%) eyes. Preoperative BCVA correlated with BCVA at last follow-up visit (r = 0.66; P < 0.001). At last visit, the SB group had a mean logarithm of the minimum angle of resolution BCVA of 0.36 (20/46) and a single-operation success rate of 76.9% at 6 months, whereas the SB/PPV group had a mean logarithm of the minimum angle of resolution BCVA of 1.08 (20/238) and single-operation success rate of 77.8% (P = 0.04 and P = 0.96, respectively). Six eyes in the SB/PPV group received silicone oil tamponade. In eyes with at least 1 year of follow-up, 4 (14.8%) in the SB group and 6 (100%) in the SB/PPV group developed a visually significant cataract requiring cataract surgery (P < 0.001)., Conclusions: Retinal detachment associated with retinal dialysis is typically associated with trauma and more often occurs in young male patients. The current study confirms that SB without PPV is an effective initial treatment strategy for most patients with retinal dialysis and has a low rate of cataract formation., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background and Objective: Retinal detachments (RDs) are a complication of X-linked retinoschisis (XLRS) with a poor prognosis. This study aims to report outcomes of XLRS-RD repair in pediatric patients., Materials and Methods: The study is a retrospective analysis of pediatric patients undergoing vitreoretinal surgery for XLRS-RDs from 2000 to 2022., Results: Nine patients (11 eyes) met inclusion criteria. All patients were boys, with a mean age of 8 years. Most RDs arose inferiorly (64%). Seven (64%) detachments were macula-involving. Seven (64%) detachments were repaired with combined scleral buckling and vitrectomy, two (18%) detachments were repaired with vitrectomy alone, one (9%) detachment underwent a primary scleral buckling procedure, and one (9%) asymptomatic detachment with a pigment demarcation line was observed. Silicone oil was used in eight of nine (89%) eyes undergoing vitrectomy. Final visual acuity was 20/200 or better in eight (73%) eyes. One eye that underwent surgical intervention remained unattached at follow-up., Conclusion: Surgical repair in patients with XLRSRDs was associated with successful reattachment, although visual prognosis varied. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:574-579.] .

We report the case of a 28-year-old man with X-linked retinoschisis (XLRS) and type I diabetes mellitus. The patient had bilateral foveoschisis with a tractional retinal fold in the right eye. Optical coherence tomography (OCT) revealed hyperreflective material within the inner nuclear and outer plexiform layers, photoreceptor atrophy, and retinal pigment epithelium irregularities in both eyes. Fluorescein angiography showed hyperfluorescent foveal spots corresponding to the hyperreflective material observed on OCT. This is a unique presentation of XLRS, with concurrent foveoschisis and photoreceptor atrophy in both eyes. The hyperreflective material on OCT serves as a distinctive feature of XLRS. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:603-606.] .

Introduction: An 18-year old highly myopic woman presented with bilateral retinoschisis associated with a unilateral macular hole in the right eye and vitreomacular traction in the left eye., Methods: Genetic studies disclosed a heterozygous pathogenic variant in the KCNJ13 gene was identified (c.484C>T (p.Arg162Trp)), consistent with a diagnosis of snowflake vitreoretinal degeneration (SVD)., Results: While there were no corneal guttata, juvenile cataracts, or perivascular sheathing in this case, salient features of SVD included a fibrillar vitreous structure, crystalline retinopathy, and flattened optic nerves. The patient developed a FTMH in the left eye at 17 months follow up, followed by a rhegmatogenous retinal detachment (RRD) requiring 2 surgical repairs., Conclusion: This case expands on the spectrum of clinical features in SVD, including retinoschisis and FTMH. It also characterizes optical coherence tomography findings in this rare disease entity. We emphasize the importance of using panel-based genetic testing to clinically distinguish and further define atypical vitreoretinopathies.

Background and Objective: Inducing a posterior vitreous detachment (PVD) may be challenging, especially in pediatric patients with firm vitreo-retinal adherence. This case report will present an alternative method of PVD induction using adjunctive perfluorooctane (PFO)., Materials and Methods: An 11-year-old boy underwent scleral buckle placement and 23-gauge pars plana vitrectomy for macula-off retinal detachment. Triamcinolone acetonide was used for vitreous staining. A flexible loop was used to peel the hyaloid membrane from attached retina nasal to the optic nerve. A small hole was created in the posterior hyaloid, and a small PFO bubble was injected under the detached vitreous to further dissect it from the retina and propagate the vitreous detachment anteriorly. The vitrector was used to lift and complete the PVD peripherally., Results: PVD induction was obtained with no iatrogenic retinal breaks or postoperative complications., Conclusion: PVD induction using a stepwise PFO approach is a safe and effective alternative to conventional techniques. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:543-546.] .

Objective: To report the management and outcomes of vitreous hemorrhage in pediatric patients with X-linked retinoschisis (XLRS)., Materials and Methods: Retrospective case series of pediatric patients with XLRS with vitreous hemorrhage between January 2000 and January 2022 at the Bascom Palmer Eye Institute., Results: Nine patients (12 eyes) met inclusion criteria. The average age at presentation was 5.75 years and mean follow-up time was 6 years. All eyes (58.3%) that underwent fluorescein angiography exhibited peripheral capillary dropout. Six of 12 eyes (50%) were observed without intervention. Four of 12 eyes (33.3%) underwent intravitreal injection of bevacizumab and 2 (16.6%) underwent vitreoretinal surgery. Seven of 12 eyes (58.3%) had documented recurrence of vitreous hemorrhage during follow-up. All retinas were attached and had clear media at final follow-up., Conclusion: Fluorescein angiography is a helpful tool to evaluate the vascular phenotype in XLRS. Favorable anatomic outcomes were noted in this cohort, but recurrence of vitreous hemorrhage was common. [ Ophthalmic Surg Lasers Imaging Retina 2023;54:513-518.] .

Purpose: This study aims to report the association of optic nerve hypoplasia (ONH), peripheral retinal nonperfusion, and secondary complications in pediatric patients., Design: Retrospective case series., Methods: The study was conducted between January 2015 and January 2022 at the Bascom Palmer Eye Institute. Inclusion criteria were clinical diagnosis of optic disc hypoplasia, age <18 years, and a fluorescein angiography (FA) of acceptable quality., Results: Seven patients (11 eyes) met inclusion criteria. Average age at presentation was 3.5 years (range 1 month-8 years) and the mean follow-up time was 34.28 months (range 2-87 months). Four patients (57.14%) showed bilateral optic disc hypoplasia. All eyes exhibited peripheral retina nonperfusion on FA, in which mild severity was found in 7 eyes (63.63%), moderate in 2 eyes (18.18%), severe in 1 eye (9.09%) and extreme in 1 eye (9.09%). Eight eyes (72.72%) showed evidence of 360 degrees of retinal nonperfusion. Two patients (18.18%) were diagnosed with concurrent retinal detachment that were deemed inoperable at the time of diagnosis. All cases were observed without intervention. None of the patients were observed to have complications during follow-up., Conclusion: Among pediatric patients with ONH, there appears to be a high rate of concurrent retinal nonperfusion. In these cases, FA is a helpful tool to detect peripheral nonperfusion. Retinal findings are subtle in some cases and may not be detectable in children with suboptimal imaging performed without examination under anesthesia., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: To assess the rates of retinopathy of prematurity (ROP) and treatment-warranted ROP in a modern set of patients meeting 0 or 1 of the current ROP screening criteria., Design: Retrospective cohort study., Methods: Single-center study of 9350 infants screened for ROP from 2009 to 2019. Rates of ROP and treatment-warranted ROP were evaluated in group 1 (birth weight [BW] <1500 g and gestational age [GA] ≥30 weeks), group 2 (BW ≥1500 g and GA <30 weeks), and group 3 (BW ≥1500 g and GA ≥30 weeks)., Results: Of 7520 patients with reported BW and GA, 1612 (21.4%) patients met the inclusion criteria. The number of patients in groups 1, 2, and 3 was 466 (6.19%), 23 (0.31%), and 1123 (14.93%), respectively. The number of patients diagnosed with ROP was 20 (4.29%) in group 1, 1 (4.35%) in group 2, and 12 (1.07%) in group 3 (P < .001). The mean interval between birth and ROP diagnosis was 36.25 days (range 12-75 days) in group 1, 47 days in group 2, and 23.33 days (range 10-39 days) in group 3 (P = .05). No cases of stage 3, zone 1, or plus disease were recorded. No patients met the treatment criteria., Conclusions: Patients meeting 1 screening criterion had a low rate of ROP (<5%), with no stage 3, zone 1, or plus disease. No patients required treatment. We propose a possible algorithm (TWO-ROP) in appropriate neonatal intensive care units, with an amendment in screening protocol for this low-risk population to include only an outpatient screening examination within 1 week of discharge, or at 40 weeks if inpatient, to decrease the inpatient ROP screening burden while maintaining safety. Further external validation of this protocol would be required., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the retinal vasculature in pediatric patients with X-linked retinoschisis (XLRS)., Design: Retrospective consecutive case series., Subjects: Pediatric patients with a diagnosis of XLRS who had undergone widefield fluorescein angiography (FA)., Methods: The electronic medical records of pediatric patients with XLRS at a tertiary referral eye center were reviewed from January 2015 to December 2021. Fluorescein angiography images were reviewed for anomalies of the retinal vasculature., Main Outcomes Measures: Vascular anomalies on FA were recorded, including capillary dropout/ischemia, terminal supernumerary vessels, vascular leakage, abnormal vascular loops, straightening of vessels, aberrant circumferential vessels, and neovascularization., Results: In total, 29 eyes of 15 patients were included in the study (1 patient had a phthisical eye). On FA, the most common findings were capillary dropout/ischemia (21 of 29 eyes, 72.4%), terminal supernumerary vessels (21 eyes, 72.4%), abnormal vascular loops (20 eyes, 69%), and vascular leakage (17 eyes, 58.6%). Of the 17 eyes with leakage, the most posterior zone of involvement was zone 1 in 11 eyes (64.7%) and zone 2 in 6 eyes (35.3%). All eyes demonstrated ≥ 1 vascular anomaly on FA. Among the 29 eyes, 23 (79.3%) demonstrated peripheral bullous schisis or retinal detachment (RD) with a mean of 5.6 clock hours of involvement. The presence of either RD or bullous retinal schisis was associated with the incidence of capillary dropout (91.3% in schisis/RD eyes vs. 0% in nonschisis/RD eyes, P < 0.001). Among those with RD or bullous schisis, a higher degree of involvement correlated with more severe capillary dropout (Pearson 0.49, P = 0.025)., Conclusion: The present study demonstrates consistent vascular changes in pediatric patients with XLRS using widefield FA. Although the presence of capillary ischemia was associated with the severity of bullous schisis or RD, other vascular anomalies were observed in patients both with and without peripheral schisis. Although further research is needed to understand the etiology of these vascular anomalies, FA should be considered in the evaluation of these patients., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2023 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate the visual outcomes with unexplained vision loss during or after silicone oil (SO) tamponade. Methods: This multicenter retrospective case series comprised patients with unexplained vision loss associated with SO tamponade or its removal. Eyes with other clear secondary identifiable causes of vision loss were excluded. Results: Twenty-nine eyes of 28 patients (64% male) were identified. The mean age was 50 ± 13 years (range, 13-78 years). The mean duration of SO tamponade was 148 ± 38 days. Eighteen eyes (62%) developed unexplained vision loss while under SO; 11 (38%) had vision loss after SO removal. The most common optical coherence tomography (OCT) finding was ganglion cell layer (GCL) thinning (55%). Eyes with vision loss after SO removal had a mean logMAR best-corrected visual acuity (BCVA) of 0.6 ± 0.7 (Snellen 20/85) before SO tamponade and 1.2 ± 0.4 (20/340) before SO removal. By the last follow-up after SO removal, the BCVA had improved to 1.1 ± 0.4 (20/235). In eyes with vision loss after SO removal, the BCVA before SO removal was 0.7 ± 0.7 (20/104), which deteriorated to 1.4 ± 0.4 (20/458) 1 month after SO removal. By the last follow-up, the BCVA had improved to 1.0 ± 0.5 (20/219). Conclusions: Unexplained vision loss can occur during SO tamponade or after SO removal. Vision loss was associated with 1000-centistoke and 5000-centistoke oil and occurred in macula-off and macula-on retinal detachments. The duration of tamponade was 3 months or longer in the majority of eyes. Most eyes had GCL thinning on OCT. Gradual visual recovery can occur yet is often incomplete., Competing Interests: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr. Yannuzzi is on Advisory Board of Genentech and RegenXbio. Dr. Mantopoulos is a consultant to Alimera Sciences. Dr. Goduni has served on the Alimera Fellows Advisory Board and is an independent contractor for Clover Therapeutics. Dr. Jung is a consultant to Carl Zeiss Meditec, Inc., (© The Author(s) 2023.)

Background: Familial exudative vitreoretinopathy (FEVR) is a rare inherited disease characterized by abnormal retinal angiogenesis that leads to incomplete vascularization of the peripheral retina and ischemia. The disease demonstrates complex genetics and can be inherited in an autosomal recessive, autosomal dominant, or X-linked recessive fashion. All presently identified pathogenic genetic variants account for about 50% of all FEVR cases worldwide. Genetic testing can confirm the diagnosis., Materials and Methods: Case report., Case: A 7-year-old female who was born prematurely at 33 weeks gestation and was thought to have progression of bilateral retinopathy of prematurity (ROP) was referred to a pediatric-retina specialist for management. Upon initial examination under anesthesia with multimodal imaging, the diagnosis of FEVR was suspected. Genetic testing identified a FZD4 variant involving a novel complex interchromosomal rearrangement involving chromosomes 2 and 11 associated with microarray-defined deletion of 11q14. The patient was conceived via IVF and has a fraternal twin without FEVR. This is the first report of familial exudative vitreoretinopathy associated with this combination of genetic findings., Conclusion: Autosomal dominant FEVR involves abnormalities in several genes, including FZD4 at the chromosome 11q. We recommend that patients with microarray-defined deletions of 11q have careful review of the allelic deletions in Chromosome 11 to determine if FZD4 is included because a loss of function variant of a single copy of FZD4 is sufficient to cause the FEVR phenotype. It is essential to differentiate FEVR from other pediatric retinal diseases in children, including ROP, persistent fetal vasculature, and Coats disease.

Purpose: To study the effect of the pandemic-related lockdown (physical distance measures and movement restrictions) on the characteristics and management of retinopathy of prematurity (ROP)., Methods: In this controlled, multicenter cohort study, the medical records of patients born prematurely and screened for ROP in the neonatal intensive care unit during four time periods were reviewed retrospectively: (1) November 1, 2018, to March 15, 2019; (2) March 16, 2019, to August 2, 2019 (lockdown control period); (3) November 1, 2019, to March 15, 2020; and (4) March 16, 2020-August 2, 2020., Results: A total of 1,645 patients met inclusion criteria. Among the 1,633 patients with complete data, mean gestational age (GA) at birth was 28.2, 28.4, 28.0, and 28.3 weeks across time periods 1 to 4, respectively (P = 0.16). The mean birth weight of all patients was 1079.1 ± 378.60 g, with no significant variation across time periods (P = 0.08). There were fewer patients screened during the lockdown period (n = 411) compared with the period immediately before (n = 491) and the same period in the prior year (n = 533). Significantly more patients were screened using indirect ophthalmoscopy, compared to digital imaging (telemedicine), during the lockdown (P < 0.01). There were 11.7%, 7.7%, 9.0%, and 8.8% of patients requiring treatment in each time period, respectively (P = 0.42), with a median postmenstrual age at initial treatment of 37.2, 36.45, 37.1, and 36.3 weeks, respectively (P = 0.32)., Conclusions: We recorded a decrease in the number of infants meeting criteria for ROP screening during the lockdown. The GA at birth and birth weight did not differ. Significantly more infants were screened with indirect ophthalmoscopy, compared to digital imaging, during the lockdown., (Copyright © 2023 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)