Faculty Bibliography

PURPOSE/UNASSIGNED:To describe delayed detection of pericentral hydroxychloroquine (HCQ) toxicity. METHODS/UNASSIGNED:67-year-old Dominican woman with rheumatoid arthritis on HCQ presented for examination. RESULTS/UNASSIGNED:Spectral-domain optical coherence tomography (SD-OCT) demonstrated bilateral cystoid macular edema with parafoveal attenuation of the external limiting membrane (ELM) and the ellipsoid zone (EZ). ELM and EZ disruption was present in inferior macula. While subtle superior defects were present on 10-2 visual fields, superior pericentral defects were noted on 24-2 testing. Hyperautofluorescence along inferior arcades corresponded to SD-OCT and visual fields. Examination 2 years prior demonstrated nonspecific points of depression on 10-2 visual fields and normal central SD-OCT findings. EZ and ELM disruption was present in the perifoveal inferior macula. CONCLUSIONS/UNASSIGNED:Early pericentral distribution of HCQ toxicity is not limited to Asian patients. Detecting pericentral HCQ toxicity involves reviewing entire macular cube on OCT. When OCT changes are suspected on parafoveal OCT B-scans, visual field testing with 24-2 may be more sensitive than 10-2.

PURPOSE/OBJECTIVE:To describe the vascular anatomy and intraluminal flow characteristics of segmental retinal arteritis (SRA) using structural and angiographic optical coherence tomography (OCT). METHODS:Retrospective case series of consecutive patients presenting with SRA. All patients were evaluated at presentation with fundus photography, spectral domain OCT, and OCT angiography. One patient was imaged with dense B-scan OCT angiography. RESULTS:Three eyes of three male patients were evaluated. All examinations were consistent with reactivation of ocular toxoplasmosis with an area of active retinochoroiditis adjacent to a focal chorioretinal scar. Spectral domain OCT through areas of SRA noted on clinical examination demonstrated areas of hyperreflectivity circumscribing the affected vessel with a normoreflective lumen. Optical coherence tomography angiography and dense B-scan OCT angiography demonstrated narrowing of the intraluminal flow signal that correlated with areas of segmental hyperreflectivity on spectral domain OCT. Vascular sections proximal and distal to areas of SRA showed normal flow signal. CONCLUSION/CONCLUSIONS:Vessels with SRA demonstrated hyperreflectivity highlighting the vessel wall on spectral domain OCT. Optical coherence tomography angiography showed narrowing of the flow signal within these segments suggesting reduced lumen diameter. Coupling these finding with previous indocyanine green imaging findings in SRA, the collective data suggest the plaques are localized within the vessel wall to either the endothelium or the muscular tunica media without occlusion of the vessel lumen.

PURPOSE/OBJECTIVE:To report a detachment that apparently separated photoreceptor inner segment myoids from inner segment ellipsoids as a manifestation of toxoplasmosis chorioretinitis in a patient with pachychoroid spectrum disease. METHODS:Multimodal imaging including fundus photography, spectral domain and enhanced-depth imaging optical coherence tomography (OCT), indocyanine green angiography, and OCT angiography. RESULTS:A 33-year-old man with a history of toxoplasmosis chorioretinitis reported 1 week of decreased vision to 20/200 in his right eye. Examination of the right eye demonstrated mild vitritis with recurrent chorioretinitis inferior to the fovea and adjacent to a chorioretinal scar. A dome-shaped, foveal photoreceptor layer-splitting detachment was noted on OCT. Because degenerating cone photoreceptors are capable of shedding their inner segments, we inferred the location of the detachment at the level of the inner segment myoid and provided a histological example of such from an unrelated donor case. In addition, multimodal imaging revealed dilated choroidal veins (pachyvessels) with attenuation of the inner choroid in both eyes and asymptomatic findings of central serous chorioretinopathy in the left eye. After 1 month of antibiotic and steroid therapy, the chorioretinitis resolved, as did the detachment. Hyperreflective foci on the vitreoretinal interface were appreciated with en face OCT that appeared to aggregate throughout the course of therapy, induce inner retinal striae, and resolve without inducing epiretinal membrane formation. CONCLUSION/CONCLUSIONS:Patients with preexisting pachychoroid spectrum disease may manifest a more significant retinal fluid accumulation in the setting of superimposed chorioretinal inflammation. In this case of macular toxoplasmosis chorioretinitis, inflammation manifested as a retinal detachment at the level of photoreceptor inner segment myoids that we named as a bacillary layer detachment. In this case, inflammatory sequelae of toxoplasmosis reactivation responded well to oral and intravitreal therapy.

PURPOSE/OBJECTIVE:To characterize the true position of in-the-bag intraocular lenses (IOLs) relative to the limbus using ultrasound biomicroscopy (UBM) and estimate scleral sutured IOL positioning. METHODS:This prospective single center study included 70 eyes of 41 patients with in-the-bag posterior chamber IOLs. Four vertical UBM captures were performed in each eye in the superior, inferior, nasal and temporal quadrants. Post-operative biometric data were collected. The primary outcome was the vertical distance of the in-the bag IOL from the sclero-corneal limbus. Secondary outcomes included anterior shift and refractive change of a theoretical scleral sutured IOL using sclerotomies at 2.5mm and 3mm posterior to the limbus. RESULTS:A total of 265 UBM images were analyzed, including 64 superior, 69 inferior, 66 nasal and 66 temporal. The true in-the-bag IOL position measured as distance posterior to the sclero-corneal limbus was 4.23 mm ± 0.56 mm superiorly, 4.22 ± 0.46 mm inferiorly, 3.95 ± 0.48 mm nasally and 3.86 ± 0.52 mm temporally. The anterior shift of a theoretical scleral sutured IOL was 0.60mm for a 3mm sclerotomy and 0.93mm for a 2.5mm sclerotomy, resulting in a theoretical myopic shift of 0.45 D and 0.79 D respectively, assuming a 15 D IOL. Larger biometric measurements correlated to a more posterior in-the-bag position. CONCLUSIONS:True in-the-bag IOL position was found to be more posterior than estimates of scleral sutured IOLs. Additional corrections in scleral sutured IOL calculations may improve refractive outcomes.

OBJECTIVE:To identify the role of gender and other factors in influencing ophthalmologists' compensation. DESIGN/METHODS:Cross-sectional study. PARTICIPANTS/METHODS:U.S. practicing ophthalmologists. METHODS:Between January and March 2020, an anonymous survey was sent to U.S. residency program directors and practicing ophthalmologists who recently completed residency training. Respondents who completed residency ≤10 years ago and responded to questions about gender, fellowship training, state of practice, and salary were included. Propensity score match (PSM) analysis was performed with age, academic residency, top residency, fellowship, state median wage, practice type, ethnicity and number of work days. Multivariate linear regression (MLR) analysis controlled for additional factors along with the aforementioned variables. MAIN OUTCOME MEASURES/METHODS:Base starting salary with bonus (SWB) received in the first year of clinical position was the main outcome measure. A multiplier of 1.2 (20%) was added to the base salary to account for bonus. RESULTS:Of 684 respondents, 384 (56% female, 44% male) from 68 programs were included. Female ophthalmologists received a mean initial SWB that was $33,139.80 less than their male colleagues (12.5%, p=0.00). PSM analysis showed a SWB difference of -$27,273.89 (10.3% gap, p=0.0015). Additionally, SWB differences were calculated with the number of work days substituted by OR days [-$19727.85 (8.60% gap, p=0.0092)] and clinic days [--$27793.67 (10.5% gap, p=0.0013)] in separate PSM analyses. The SWB differences between genders were significant using MLR analyses, which also controlled for work, clinic, and OR days separately (-$22261.49, $-18604.65, and $-16191.26, respectively; p=0.017, p=0.015, p=0.002). Gender independently predicted income in all 3 analyses (p<0.05). Although an association between gender and the attempt to negotiate was not detected, a greater portion of men subjectively reported success in negotiation (p=0.03). CONCLUSION/CONCLUSIONS:Female ophthalmologists earn significantly less than their male colleagues in the first year of clinical practice. Salary differences persist after controlling for demographic, educational, and practice type variables with MLR and PSM analyses. These income differences may lead to a substantial loss of accumulated earnings over an individual's career.

Purpose : To evaluate the diagnostic yield and clinical impact of the SPARK/Invitae gene panel in patients with known or suspected inherited degenerative retinal disease, in comparison with traditional clinical assessments. Methods : Patients of the authors' clinical practices obtained genetic screening at no charge via the SPARK/Invitae ID your IRD genetic testing panel, which ranged from 248 genes to 293 genes. Over 16 months, tests were submitted for 87 patients and results were available for 70 patients. Clinical diagnoses prior to submitting the gene panel . By continuing to use our website, you are agreeing to included retinitis pigmentosa; Stargardt disease; Best vitelliform dystrophy; Leber our privacy policy. congenital amaurosis; choroideremia; achromatopsia; cone-rod dystrophy; congenital stationary night blindness; occult macular dystrophy; and familial dominant drusen in addition to patients with normal clinical findings and unclear diagnoses. Results : Of 70 patients, SPARK/Invitae considered the results ''Positive or Potentially Positive'' in 24 cases (34.3%), Carrier in 16 cases (22.9%) and ''Uncertain'' in 30 cases (42.9%). Uncertain results comprised patients with only Variants of Uncertain Significance. Patients categorized as a Carrier by SPARK/Invitae but who demonstrated pathogenic genetic changes correlating to the clinical diagnosis were considered to be in agreement with the clinical impression. The genetic diagnosis agreed with the clinical diagnosis in 30/70 (42.9%) total patients. Test results were consistent with the clinical impression in 13/26 (50.0%) retinitis pigmentosa cases, 6/8 (75.0%) Stargardt patients, 3/7 (42.9%) cone-rod dystrophy cases, and 2/4 (50.0%) Best vitelliform dystrophy patients. Gene testing helped elucidate diagnoses in two patients with unclear clinical impressions: one panel showed autosomal recessive achromatopsia and the other showed a carrier state for autosomal recessive retinitis pigmentosa. Of four patients with normal clinical exams, none had diagnostic results: all showed Uncertain findings. Conclusions : The SPARK/Invitae gene panel provided a genetic diagnosis consistent with the clinical impression in about 40 percent of patients. Retinitis pigmentosa and Stargardt disease were the most common clinical diagnoses and the diagnoses most often confirmed by testing. Genetic screening also assisted in clarifying unknown diagnoses for two patients

PURPOSE/OBJECTIVE:To report a retinal pigment epithelium (RPE) tumor with exudative maculopathy, originating from an atypical RPE lesion presumed to represent congenital hypertrophy of the RPE or RPE hyperplasia. METHODS:Multimodal imaging including fundus autofluorescence, optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. RESULTS:A 76-year-old West African man noted visual acuity reduction to count fingers in the right eye and 20/400 in the left eye. Features of chronic glaucoma were noted. In addition, there was a fairly well-circumscribed darkly pigmented RPE lesion in the paramacular region in the right eye, measuring 4 mm in diameter and flat and consistent with atypical congenital hypertrophy of the RPE or RPE hyperplasia. On the posterior margin of this mass was an RPE tumor, presumed to represent RPE adenoma, producing exudative maculopathy and cystoid macular edema. Multimodal imaging was used to distinguish the RPE tumor from macular neovascularization. A similar atypical congenital hypertrophy of the RPE without retinopathy measuring 3.5mm in diameter was noted in the temporal macular region in the left eye. After six monthly doses of intravitreal bevacizumab (1.25 mg/0.05 mL) in the right eye, the maculopathy resolved and the RPE mass showed partial involution with visual acuity return to baseline 20/200. CONCLUSION/CONCLUSIONS:Congenital hypertrophy of the RPE and RPE hyperplasia can produce RPE adenoma with related exudative maculopathy. In this case, the maculopathy responded to bevacizumab.

Importance/UNASSIGNED: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. Objective/UNASSIGNED:To evaluate whether the frequency of ophthalmic surgical procedures deemed urgent or emergent by the AAO changed across the United States during the COVID-19 pandemic. Design, Setting, and Participants/UNASSIGNED:Vitreoretinal practices from 17 institutions throughout the US participated in this multicenter cross-sectional study. The frequency of 11 billed vitreoretinal Current Procedural Terminology (CPT) codes across respective weeks was obtained from each practice between January 1, 2019, and May 31, 2020. Data were clustered into intravitreal injections (code 67028), lasers and cryotherapy (codes 67141, 67145, and 67228), retinal detachment (RD) repairs (codes 67107, 67108, 67110, and 67113), and other vitrectomies (codes 67036, 67039, and 67040). Institutions were categorized by region (Northeast, Midwest, South, and West Coast), practice setting (academic [tax-exempt] or private [non-tax-exempt]), and date of respective statewide stay-at-home orders. Main Outcomes and Measures/UNASSIGNED:Nationwide changes in the frequency of billing for urgent or emergent vitreoretinal surgical procedures during the COVID-19 pandemic. Results/UNASSIGNED:A total of 526 536 CPT codes were ascertained: 483 313 injections, 19 257 lasers or cryotherapy, 14 949 RD repairs, and 9017 other vitrectomies. Relative to 2019, a weekly institutional decrease in injections was observed from March 30 to May 2, 2020, with a maximal 38.6% decrease (from a mean [SD] of 437.8 [436.3] to 273.8 [269.0] injections) from April 6 to 12, 2020 (95% CI, -259 to -69 injections; P = .002). A weekly decrease was also identified that spanned a longer interval, at least until study conclusion (March 16 to May 31, 2020), for lasers and cryotherapy, with a maximal 79.6% decrease (from a mean [SD] of 6.6 [7.7] to 1.5 [2.0] procedures) from April 6 to 12, 2020 (95% CI, -6.8 to -3.3 procedures; P < .001), for RD repairs, with a maximal 59.4% decrease (from a mean [SD] of 3.5 [4.0] to 1.6 [2.2] repairs) from April 13 to 19, 2020 (95% CI, -2.7 to -1.4 repairs; P < .001), and for other vitrectomies, with a maximal 84.3% decrease (from a mean [SD] of 3.0 [3.1] to 0.4 [0.8] other vitrectomies) from April 6 to 12, 2020 (95% CI, -3.3 to -1.8 other vitrectomies; P < .001). No differences were identified by region, setting, or state-level stay-at-home order adjustment. Conclusions and Relevance/UNASSIGNED:Although the AAO endorsed the continued performance of urgent or emergent vitreoretinal surgical procedures, the frequency of such procedures throughout the country experienced a substantial decrease that may persist after the COVID-19 pandemic's initial exponential growth phase. This decrease appears independent of region, setting, and state-level stay-at-home orders. It is unknown to what extent vitreoretinal intervention would have decreased without AAO recommendations, and how the decrease is associated with outcomes. Although safety is paramount during the COVID-19 pandemic, practices should consider prioritizing availability for managing high-acuity conditions until underlying reasons for the reduction are fully appreciated.