What Is Just the Right Amount of Anti–Vascular Endothelial Growth Factor Treatment for Diabetic Macular Edema?Protocol V and the Goldilocks Principle

Michael A. Grassi, MD

doi : 10.1001/jamaophthalmol.2021.0013

JAMA Ophthalmol. 2021;139(4):381-382

For patients with center-involving diabetic macular edema and good visual acuity, the Diabetic Retinopathy Clinical Research (DRCR) Retina Network’s randomized clinical trial Protocol V found no significant difference in vision loss between prompt anti–vascular endothelial growth factor (anti-VEGF) treatment and initial observation.1 Protocol V raises the important teleological question as to what the goal should be in treating macular edema. For patients with good vision and center-involving diabetic macular edema, is there an anti-VEGF treatment strategy that is not too much or too little but just right?

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Presence of SARS-CoV-2 RNA in the Cornea of Viremic Patients With COVID-19

Maria Casagrande, MD; Antonia Fitzek, MD; Martin S. Spitzer, MD; et al.

doi : 10.1001/jamaophthalmol.2020.6339

JAMA Ophthalmol. 2021;139(4):383-388

Importance  Current recommendations are to avoid tissue for corneal transplant from donors with coronavirus disease 2019 (COVID-19) or those who were recently exposed to COVID-19 owing to the lack of knowledge about the presence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in corneal tissues. Evidence of SARS-CoV-2 in corneal tissue would seem to have clinical relevance for corneal transplant.

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Opportunities to Reduce Potential Bias in Ophthalmic Cost-Utility Analysis

Gary C. Brown, MD, MBA; Melissa M. Brown, MD, MN, MBA; Imtiaz Chaudhry, MD; et al.

doi : 10.1001/jamaophthalmol.2020.6591

JAMA Ophthalmol. 2021;139(4):389-397

Importance  Select research methods in cost-utility analysis (incremental cost-effectiveness analysis) might potentially bias against patient value (quality-adjusted life-year [QALY]) gain and cost-effectiveness associated with common ophthalmic interventions in disabled, elderly, and African American populations.

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Assessment of Trends in the Incidence Rates of Central Retinal Artery Occlusion in Korea From 2002 to 2015

Jongshin Kim, MD, PhD; Seong Jun Byun, MS; Se Joon Woo, MD, PhD; et al.

doi : 10.1001/jamaophthalmol.2020.6860

JAMA Ophthalmol. 2021;139(4):399-405

Importance  Central retinal artery occlusion (CRAO) is associated with and shares common risk factors with cardiovascular diseases. Over the past several decades, the incidence rates of stroke and ischemic heart disease have substantially decreased in high-income industrialized countries. However, little is known regarding current trends in CRAO incidence rates.

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Discordance Rate Among Bilateral Simultaneous and Sequential Temporal Artery Biopsies in Giant Cell ArteritisRole of Frozen Sectioning Based on the Mayo Clinic Experience

Devon A. Cohen, MD; John J. Chen, MD, PhD; Bryan J. Neth, MD, PhD; et al.

doi : 10.1001/jamaophthalmol.2020.6896

JAMA Ophthalmol. 2021;139(4):406-413

Importance  Frozen section temporal artery biopsy (TAB) may prevent a contralateral biopsy from being performed.

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Tuberculosis Exposure With Risk of Behçet Disease Among Patients With Uveitis

Zhenyu Zhong, MD; Guannan Su, PhD; Qingyun Zhou, MD; et al.

doi : 10.1001/jamaophthalmol.2020.6985

JAMA Ophthalmol. 2021;139(4):415-422

Importance  Although experimental studies support the hypothesis that exposure of infectious agents may trigger an aberrant immune response and contribute to noninfectious uveitis, the association of a definite pathogen with human noninfectious uveitis conditions appears not to have been well established in a population.

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Predicting Edema Resolution After Descemet Membrane Endothelial Keratoplasty for Fuchs Dystrophy Using Scheimpflug Tomography

Daniel Zander, BSc; Viviane Grewing, MD; Andreas Glatz, MD; et al.

doi : 10.1001/jamaophthalmol.2020.6994

JAMA Ophthalmol. 2021;139(4):423-430

Importance  Predicting the extent of corneal edema resolution after Descemet membrane endothelial keratoplasty (DMEK) may help in preoperative decision-making by identifying patients who may benefit from restoring endothelial function.

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Prevalence and Factors Associated With Eyeglass Wear Compliance Among Preschoolers From Low-Income Families in San Francisco, California

Sabhyta Sabharwal, MPH; April Nakayoshi, MPH, CHES; Christopher R. Lees, BS; et al.

doi : 10.1001/jamaophthalmol.2020.7053

JAMA Ophthalmol. 2021;139(4):433-440

Importance  Despite growing support for early school-based vision screening and eyeglass provision, few studies have rigorously monitored the compliance of eyeglass wear among preschool-aged children who receive eyeglasses through such programs.

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Early Experience With Brolucizumab Treatment of Neovascular Age-Related Macular Degeneration

Ana Bety Enr?quez, MD; Caroline R. Baumal, MD; Ashley M. Crane, MD; et al.

doi : 10.1001/jamaophthalmol.2020.7085

JAMA Ophthalmol. 2021;139(4):441-448

Importance  Outcome data are limited regarding early experience with brolucizumab, the most recently approved anti–vascular endothelial growth factor (VEGF) agent for the treatment of neovascular age-related macular degeneration (nAMD).

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Association of No-Cost Genetic Testing Program Implementation and Patient Characteristics With Access to Genetic Testing for Inherited Retinal Degenerations

Peter Y. Zhao, MD; Kari Branham, MS; Dana Schlegel, MS, MPH; et al.

doi : 10.1001/jamaophthalmol.2021.0004

JAMA Ophthalmol. 2021;139(4):449-455

Importance  The benefits of no-cost genetic testing initiatives have not been characterized. The no-cost My Retina Tracker Genetic Testing Study (MRT-GTS) research registry for inherited retinal degenerations (IRDs) was launched in 2017 in the US.

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Frequency of Urgent or Emergent Vitreoretinal Surgical Procedures in the United States During the COVID-19 Pandemic

Mark P. Breazzano, MD; Archana A. Nair, MD; J. Fernando Arevalo, MD, PhD; et al.

doi : 10.1001/jamaophthalmol.2021.0036

JAMA Ophthalmol. 2021;139(4):456-463

Importance  The American Academy of Ophthalmology (AAO) indicated that urgent or emergent vitreoretinal surgical procedures should continue during the coronavirus disease 2019 (COVID-19) pandemic. Although decreases in the frequency of critical procedures have been reported outside the field of ophthalmology, analyses are limited by volume, geography, and time.

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Overminus Lens Therapy for Children 3 to 10 Years of Age With Intermittent ExotropiaA Randomized Clinical Trial

Angela M. Chen, OD, MS; S. Ayse Erzurum, MD; Danielle L. Chandler, MSPH; et al.

doi : 10.1001/jamaophthalmol.2021.0082

JAMA Ophthalmol. 2021;139(4):464-476

Importance  This is the first large-scale randomized clinical trial evaluating the effectiveness and safety of overminus spectacle therapy for treatment of intermittent exotropia (IXT).

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Calculating Utility Gain by Different Methods for the Treatment of Cataract or Neovascular Age-Related Macular DegenerationWhat Are the Consequences?

Marissa J. Carter, PhD, MA

doi : 10.1001/jamaophthalmol.2020.6584

JAMA Ophthalmol. 2021;139(4):397-398

Cataract surgery and drug treatment for neovascular age-related macular degeneration (NVAMD) are among the most common evidence-based ophthalmic procedures and yield substantial benefit to patients. For vulnerable populations who are elderly or disabled and minority populations who have struggled to obtain access to appropriate care most of their lives, loss of sight is devastating, so providing timely treatment ought to be straightforward. However, cataracts and NVAMD mostly occur late in life, which means that these conditions compete with other concurrent serious comorbidities, and benefits are spread across far fewer years than for younger patients. As a result, quantifying treatment benefits becomes more complicated.

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Is It Always Better to Get Faster Results in Temporal Artery Biopsies?

Patricia Chévez-Barrios, MD

doi : 10.1001/jamaophthalmol.2020.6895

JAMA Ophthalmol. 2021;139(4):413-414

Diagnosis and treatment of giant cell arteritis (GCA) have evolved throughout the years. Before the 1990s, the diagnosis relied entirely on temporal artery biopsy (TAB)–proven disease to start the eyesight-saving steroid treatment. Pathologists used to do frozen sections to rapidly render a diagnosis that would inform the decision of whether or not to have the patient start corticosteroid treatment. This sounds like an ideal practice for patient care, so why have we stopped favoring this approach? Primarily, it is because we have reached a better understanding of GCA physiopathology, which has balanced the accuracy vs speed of diagnosis.

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Toward a Paradigm Shift in the Therapeutic Approach to Fuchs Endothelial Corneal Dystrophy

Viridiana Kocaba, MD, PhD; Silke Oellerich, PhD; Gerrit R. J. Melles, MD, PhD

doi : 10.1001/jamaophthalmol.2020.7041

JAMA Ophthalmol. 2021;139(4):431-432

Over the past decade, Descemet membrane endothelial keratoplasty (DMEK) has become the standard for the treatment of endothelial diseases, with Fuchs endothelial corneal dystrophy (FECD) being the most common indication.1 Descemet membrane endothelial keratoplasty in eyes with FECD usually results in successful clinical outcomes with low postoperative complication rates.2 Because of the fast and reliable visual rehabilitation after DMEK, the visual threshold for undergoing corneal transplantation has decreased and DMEK is performed in a wide range of FECD stages.3 Currently, surgical indications are based mainly on best-corrected visual acuity, central corneal thickness, and patients’ subjective symptoms.

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Overminus Lens Therapy for Children With Intermittent Exotropia

Boon Long Quah, MBBS, MMed(Ophth)

doi : 10.1001/jamaophthalmol.2021.0081

JAMA Ophthalmol. 2021;139(4):476-477

Conservative management of intermittent exotropia in children includes fusion or convergence exercises, antisuppression eye patch therapy, overminus lens therapy, or observation alone. Overminus lenses improve strabismus control in children with intermittent exotropia by stimulating accommodative convergence, thereby reducing exotropia and facilitating sensory fusion. They have been recommended as nonsurgical treatment for children with intermittent exotropia associated with a high accommodative convergence/accommodation (AC/A) ratio,1 because standard strabismus surgery is associated with risk of consecutive esotropia for near.

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White Macula After Pars Plana Vitrectomy in a 71-Year-Old Woman

Chris Y. Wu, MD; Darren Knight, MD; William Freeman, MD

doi : 10.1001/jamaophthalmol.2020.4616

JAMA Ophthalmol. 2021;139(4):478-479

A 71-year-old woman with non-Hodgkin lymphoma receiving maintenance obinutuzumab (anti-CD20 monoclonal antibody) infusions underwent uneventful pars plana vitrectomy of the right eye for vitreomacular traction. Diluted triamcinolone acetonide (TA) was used to stain the posterior hyaloid. After removal of 25-gauge trocar cannulas, subconjunctival TA and ceftazidime was injected. Owing to cystoid changes in the central macula, a short-acting gas tamponade with sulfur hexafluoride was used to tamponade any cystoid abnormalities that may have been unroofed. Despite unremarkable postoperative day 1 (POD1) examination findings, the patient presented on postoperative week 3 with a painless central scotoma and worsening floaters. Her visual acuity was 20/400 OD and intraocular pressure was normal. There was no external hyperemia or chemosis, and the anterior chamber was deep and quiet with no cells or flare. On dilated fundus examination, there were 15 to 20 cells per high-power field (using a 1?×?1-mm2 high-intensity incidental slit beam) and a white macular lesion with disc edema and scattered intraretinal and peripapillary hemorrhages and mixed venous and arterial sheathing. Optical coherence tomography of the macula showed atrophy, full-thickness hyperreflectivity, and a residual epiretinal membrane (Figure).

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Nonclearing Corneal Edema After Phacoemulsification and Intraocular Lens Implantation

Shruti Aggarwal, MD; Ghada Al Bayyat, MD; Carol L. Karp, MD

doi : 10.1001/jamaophthalmol.2020.4660

JAMA Ophthalmol. 2021;139(4):480-481

An 82-year-old man with an ocular history significant for bilateral nuclear sclerotic cataracts presented to the Cornea Service at Bascom Palmer Eye Institute, Miami, Florida, with a history of blurred vision in the right eye after phacoemulsification cataract extraction 8 weeks earlier. Current ophthalmic medications included topical prednisolone acetate, 1%, in the right eye 6 times per day. The patient reported no improvement with the treatment. On examination, his best-corrected visual acuity was 20/80 OD and 20/20 OS. Pupils were reactive without relative afferent pupillary defect, and intraocular pressures were 18 mm Hg in the right eye and 11 mm Hg in the left eye. A slitlamp biomicroscopic examination of the right eye revealed grade 2 to 3 diffuse central corneal edema and folds (Figure 1). The anterior chamber was deep and the posterior chamber intraocular lens was in good position within the capsular bag. Although the view to the posterior pole of the right eye was hazy owing to corneal edema, dilated fundus examination findings were unremarkable. Examination of the left eye revealed a few guttate. The central corneal thickness by ultrasound pachymetry was 957 ?m in the right eye and 547 ?m in the left eye.

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A 47-Year-Old Woman Urgently Referred for Proliferative Diabetic Retinopathy Management

Tianyu Liu, MD; Ayman G. Elnahry, MD, PhD; Benjamin J. Kim, MD

doi : 10.1001/jamaophthalmol.2020.4670

JAMA Ophthalmol. 2021;139(4):482-483

A 47-year-old woman with a history of hypertension and type 2 diabetes for more than 15 years was referred for proliferative diabetic retinopathy management. Her chief complaint was acute onset of blurry vision in the right eye. She described intermittent foggy vision and the appearance of “a hair moving across the vision of her right eye” for the past few weeks. Her most recent hemoglobin A1c level was 8.3%, and 2 months prior to her visit, she had restarted diabetes medications. Her medications included metformin and glimepiride. Her corrected visual acuity was 20/20 OU. Her pupil, color vision, and anterior segment examinations had normal results bilaterally. A posterior segment examination of the right eye showed mild temporal elevation of the optic disc with blurred margins, mild peripapillary hemorrhages, a small amount of preretinal hemorrhage, and small-caliber branching vessels at the temporal disc with no fibrosis (Figure 1A). The left eye showed a normal optic disc, a cup-disc ratio of 0.1, and some microaneurysms at the macula. There were a few scattered dot-blot hemorrhages bilaterally. Fluorescein angiography of the right eye showed mild leakage from the abnormal vessels at the temporal optic disc (Figure 1B).

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Unilateral Frosted Branch Angiitis in a Patient With Immunosuppression

Narine Viruni, MD; Amde Selassie Shifera, MD, PhD; J. Fernando Arevalo, MD, PhD

doi : 10.1001/jamaophthalmol.2020.4684

JAMA Ophthalmol. 2021;139(4):484-485

A 49-year-old African American man who was hospitalized with hypervolemia due to supraventricular tachycardia presented with acute unilateral painless vision loss. His active medical problems included atrial flutter on anticoagulation, antisynthetase syndrome (which was diagnosed based on the presence of autoantibodies against aminoacyl-tRNA synthetase), fever with negative infectious workup results, and interstitial lung disease that was managed with azathioprine. Two weeks before the onset of sudden vision loss, the patient developed a rapidly evolving purpuric rash on his hands and feet that progressed to digital ischemia (Figure 1A). He was treated with intravenous methylprednisolone, which was completed 3 days before the onset of loss of vision and transitioned to treatment with azathioprine, 150 mg, and prednisone, 80 mg, that he was taking at the time of his vision loss.

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Association of Macular Optical Coherence Tomography Measures and Deficits in Facial Recognition in Patients With Glaucoma

Sabine S. Khan, MD; Sitara H. Hirji, MD; Donald C. Hood, PhD; et al.

doi : 10.1001/jamaophthalmol.2021.0137

JAMA Ophthalmol. 2021;139(4):486-487.

We recently reported that glaucomatous macular damage, as measured by 10-2 perimetry, is directly correlated with diminished facial recognition in eyes with good central vision.1 However, it is possible that glaucomatous macular damage impairs several dimensions of visual function required for facial recognition and is not limited to impairment related to Humphrey visual field defects. This study investigates the direct association between facial recognition and macular structural damage, as measured by spectral-domain ocular coherence tomography (SD-OCT) retinal ganglion cell and inner plexiform layer (RGC+). Because global damage is more likely to impair central visual tasks, such as contrast and vernier acuity, we also determined if the pattern of glaucomatous macular damage had a differential association with facial recognition.

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Cutaneous Malignant Melanoma Metastatic to the Intraocular Lens

Jérôme Chartier, MD; Pierre-Jean Pisella, MD, PhD; Raoul Kanav Khanna, MD

doi : 10.1001/jamaophthalmol.2021.0017

JAMA Ophthalmol. 2021;139(4):e210017.

A 77-year-old man, who had a stage 4 cutaneous malignant melanoma located on his back that was first treated with nivolumab and switched to ipilimumab, was examined for ocular redness and reduced visual acuity to light perception OD. An examination revealed iris neovascularization, pigmented cells in the anterior chamber, and several pigmented lumps on the posterior chamber intraocular lens (Figure). Cytological analysis of the aqueous humor revealed human melanoma black–positive cells, confirming the melanocytic origin. Few cases of secondary ocular,1 crystalline,2 or pseudophakic lens or capsule3 localization of malignant cutaneous melanoma have been described. No additional treatment was possible, and the patient died of brain metastases 1 month later.

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Perforation of Presumed Papillary Gliosis Resulting in an Optic Pit in a Child

Aaron W. Winter, MD; Christopher J. Lyons, MD

doi : 10.1001/jamaophthalmol.2021.0246

JAMA Ophthalmol. 2021;139(4):e210246

A healthy 16-month-old boy had a translucent, gliotic-appearing membrane overlying the left optic nerve. Grayish discoloration was apparent inferotemporally (Figure, A). By age 8 years, the presumed gliosis had thinned to a translucent, pulsatile membrane. One year later, the membrane had perforated (Figure, B). An adjacent papillomacular nerve fiber layer defect was evident, associated with visual acuity of 20/70 OS. There was no subretinal fluid on optical coherence tomography.

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Ocular Relapse of B-Cell Acute Lymphoblastic Leukemia

Kevin Ferenchak, MD; Linda N. Dao, MD; Lauren A. Dalvin, MD

doi : 10.1001/jamaophthalmol.2021.0514

JAMA Ophthalmol. 2021;139(4):e210514

A young adult presented with left-eye redness and pain, which was unresponsive to corticosteroids for presumed uveitis. The patient’s visual acuity was 20/50 OS, with thickened pink conjunctiva, corneal edema, and anterior chamber cell, hypopyon, and diffuse, white vascular iris infiltration. A history revealed B-cell acute lymphoblastic leukemia/lymphoma (B-ALL) in remission after a bone marrow transplant. No B-ALL recurrence or posttransplant lymphoproliferative disorder was identified via a bone marrow biopsy, blood work, and Epstein-Barr virus testing.1 Anterior segment fluorescein angiography (Figure, A) showed an area of maximal vascularity that guided a fine-needle biopsy. Histologic sections showed blasts in the iris, aqueous humor, and conjunctiva (Figure, B). Blasts were positive for terminal deoxynucleotidyl transferase and CD19, CD20, and CD22 immunoperoxidase stains, consistent with B-ALL.2 Systemic chemotherapy and ocular radiation were started.

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A Spiderweb-Like Fibrinous Reaction

Isha Agarwalla, MBBS; Mohit Garg, MBBS; Ankit Ahir, MBBS, DO

doi : 10.1001/jamaophthalmol.2021.0697

JAMA Ophthalmol. 2021;139(4):e210697.

A man in his 60s had a dense nuclear sclerotic cataract, a normal pupil, and no substantial medical or ocular history. He underwent phacoemulsification and intraocular lens implantation in the left eye. He presented on the first postoperative day with foggy vision. His visual acuity was 20/40 OS.

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Do Racial/Ethnic Disparities in Rates of Enucleation for Uveal Melanoma Indicate Poor Quality of Care or Financial Obstacles?

Bertil Damato, MD, PhD

doi : 10.1001/jamaophthalmol.2020.6828

JAMA Ophthalmol. 2021;139(4):487-488

To the Editor Rajeshuni et al1 examined the association of race/ethnicity and socioeconomic status with uveal melanoma treatment by performing a retrospective analysis of Surveillance, Epidemiology, and End Results (SEER) registries in the US. They reported that treatment consisted of primary enucleation in 27% of non-White individuals compared with 20% of non-Hispanic White individuals and in 27% of individuals in a lower socioeconomic group compared with 14% of those in a higher socioeconomic group. They concluded, “Given the consequences of enucleation, including postoperative vision loss, decreased quality of life, decreased functionality, and increased perceived stigma, efforts should be made to appropriately reduce differences in enucleation rates across populations.”1

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Do Racial/Ethnic Disparities in Rates of Enucleation for Uveal Melanoma Indicate Poor Quality of Care or Financial Obstacles?—Reply

Talhah Zubair, MD; Nitya Rajeshuni, MD, MS; Prithvi Mruthyunjaya, MD, MHS

doi : 10.1001/jamaophthalmol.2020.6831

JAMA Ophthalmol. 2021;139(4):488-489

In Reply We appreciate Damato’s interest in our work.1 We concur that there are a multitude of factors that impact treatment decisions in cancer care from all sides, including the patient, the medical team, and the structure of the health care system.2 It is our opinion that enucleation in uveal melanoma may, in many cases, be the most appropriate treatment for a particular tumor and may not represent a last-resort treatment even during a pandemic.3 The underlying premise behind our work is that there are no guidelines indicating that patients of different racial/ethnic or socioeconomic status (SES) groups should have their uveal melanoma managed differently. Yet on examination we found racial/ethnic and SES differences in enucleation rates. These incongruencies call for further investigation and may point to health care system–wide issues, such as access to care, health care costs, and treatment burden for different populations, as noted by our work and others.1,4 We marveled at the facilities provided by the British National Health System to afford transportation and accommodation for patients with eye cancer—a service generally not available to patients in the US. We would be interested to learn whether any of the treatment decisions could be attributed to race/ethnicity or SES in that population.

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Replacement of Nonauthor Collaborator Names

doi : 10.1001/jamaophthalmol.2021.0124

JAMA Ophthalmol. 2021;139(4):489

in the Original Investigation titled “Association of Sex With Frequent and Mild ABCA4 Alleles in Stargardt Disease,”1 that published in the October 1, 2020, issue, the nonauthor collaborator names were omitted from the PubMed listing. The article has been corrected by adding Supplement 2 containing all names.

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JAMA Ophthalmology

doi : 10.1001/jamaophthalmol.2020.3755

JAMA Ophthalmol. 2021;139(4):379

Mission Statement: To be the indispensable source of ophthalmic knowledge by promptly publishing innovative, clinically relevant research through consistent and authoritative peer review and, thereby, to be the first choice of authors for their important manuscripts.

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