Shah, Saumya M.

Purpose: Foveal herniation occurs when neuroretinal tissue protrudes through and above the level of an epiretinal membrane. This study describes the visual symptoms and spectral domain optical coherence tomography findings associated with foveal herniation and evaluates the postoperative visual, anatomical, and surgical outcomes. Methods: A multicenter retrospective review of patients diagnosed with epiretinal membrane identified 59 patients with preoperative foveal herniation on spectral domain optical coherence tomography. Data regarding visual symptoms, preoperative and postoperative best-corrected visual acuity (BCVA), central retinal thickness, macular volume, and size of foveal herniation were collected, and statistical analysis was performed. Results: A total of 58 of the 59 patients with foveal herniation underwent surgical epiretinal membrane peeling, with foveal contour restored in 53.5% of patients after surgery. Average BCVA improved from 20/80 to 20/40 Snellen equivalent at most-recent postoperative visit ( P < 0.0001). The average central retinal thickness decreased from 632 µ m to 432 µ m ( P < 0.0001) and the average macular volume decreased from 11.3 mm 3 to 9.5 mm 3 ( P < 0.0001) at 3 months postoperatively. Preoperatively, greater herniation height was associated with worse BCVA ( P = 0.008), greater central retinal thickness ( P = 0.01), retinoschisis, cystoid macular edema, foveolar detachment, ellipsoid zone abnormality, and external limiting membrane abnormalities ( P < 0.05). Postoperatively, there was a decrease in retinoschisis, cystoid macular edema, foveolar detachment, ellipsoid zone, and external limiting membrane abnormality ( P < 0.05) on spectral domain optical coherence tomography. Conclusion: Patients with larger foveal herniation height had greater preoperative central retinal thickness, worse preoperative and postoperative BCVA, and more intraretinal abnormalities on spectral domain optical coherence tomography. Surgical epiretinal membrane peeling in patients with foveal herniation resulted in a significant improvement in patients' BCVA and microstructural abnormalities.

Abstract Background The coronavirus disease 2019 pandemic has significantly impacted medical education, notably the mandate for all residency programs to implement virtual (rather than in-person) residency interviews. Understanding residency applicants' perceptions and approach to this novel virtual interview season will be beneficial as potential future interview formats are considered. Objective The aim of this study was to examine perceptions of the 2020 to 2021 ophthalmology residency match applicants regarding the virtual interview season prior to the start of the interview season. Patients and Methods Ophthalmology residency applicants during the 2020 to 2021 cycle were invited to complete the survey between October 20 and 29, 2020. Respondent demographic information, perceived importance of various application details in a normal versus virtual interview season, strengths and limitations of virtual interviews, and preferences for optimal virtual interview format were obtained. Results There were 337 survey respondents, with at least 50% of the survey completed by 190 applicants (56%). Of these, 73% of respondents applied to more than 60 ophthalmology residency programs, and 78% felt that the evaluation of candidates would be impacted by the virtual interview format. Regardless of interview format, United States Medical Licensing Examination Step 1 score and letters of recommendation were perceived to be the two most important factors related to matching at an applicant's top ranked programs. The primary limitation of a virtual interview season was the inability to experience a program's culture in person, while largest strength was cost savings. Conclusion The ophthalmology residency match is a competitive process made potentially more complex by a novel virtual interview format. A detailed postcycle analysis will be important to optimize future interview seasons.

Purpose: New-onset persistent diplopia has become a common complication after glaucoma drainage device (GDD) placement. Understanding the orbital anatomy of such patients may provide information regarding risk of diplopia, GDD selection, and post-operative management. The purpose of this study was to examine the orbital anatomic differences in diplopic and non-diplopic patients after GDD implantation using high-resolution MRI. Methods: Seven eyes ( N = 4 with diplopia and N = 3 without diplopia after GDD placement) of seven patients that had undergone placement of Baerveldt 250 (B250), Baerveldt 350 (B350), or Ahmed FP7 (FP7) GDD were prospectively enrolled at a single institution. All patients underwent a 3.0T orbital MRI with 3D volumetric T1 and T2 weighted sequence. Images were analyzed for orbital volume, axial length, orbital distances, presence of superior rectus-lateral rectus (SR-LR) band, position of GDD, and SR-LR angles. Results: Patients with diplopia had smaller mean ± SD orbital axial (911.5 ± 111.8 mm 3 vs 931.7 ± 79.7 mm 3 ) and coronal volumes (1162.5 ± 145.5 mm 3 vs 1180 ± 34.6 mm 3 ) compared to non-diplopic patients. Average orbital rim distances were larger for the diplopic group. The SR-LR displacement angle for diplopic patients was larger (101.6° ± 8.1 vs 94.7° ± 17.6) while the SR-LR quadrantic angle (86.6° ± 4.2 vs 89.1° ± 4.3) was smaller. SR-LR band was present and intact in all patients. GDD malpositioning was not evident in any patient. Conclusion: The decreased orbital axial and coronal volumes as well as increased orbital rim distances in diplopic patients suggests the need for further studies to understand the role of orbital anatomy in occurrence of diplopia. Dynamic MRI imaging may be helpful in identifying differences in extraocular muscle function that reveal an etiology of diplopia in patients with GDD implantation.

Purpose: To report a case of pigmented paravenous retinochoroidal atrophy in a patient that was found to have an autosomal dominant pathogenic variant of the hexokinase 1 (HK1) gene. Methods: A case report. Results: A 41-year-old White woman with a distant family history of retinitis pigmentosa presented with a 5-year history of bilateral blurry and decreased vision that led to eventual loss of ability to drive. Color funduscopic photographs revealed retinochoroidal atrophy, hyper-reflective spots within the retina, and a paravenous distribution of pigment bilaterally. Given the patient's familial ocular history and workup, she was diagnosed with inherited retinal degeneration with phenotype suggestive of pigmented paravenous retinochoroidal atrophy. Genetic testing revealed a single rare variant, c.2551 G>A in the HK1 gene. Discussion: This case describes a pathogenic variant in HK1, a gene that has been associated with RP, but has not been previously reported in association with the pigmented paravenous retinochoroidal atrophy phenotype. This expands the phenotypes associated with HK1 pathogenic variant, p.Glu851Lys, and the genetic association of pigmented paravenous retinochoroidal atrophy to include HK1. Although pigmented paravenous retinochoroidal atrophy has been previously reported to be associated with CRB1 gene, no previous relationship to the HK1 gene has been described.

Purpose: The relationship between age-related macular degeneration (AMD) and malignancy, especially cutaneous malignancies, is not well studied. We investigated a possible association between AMD and cutaneous malignancies. Methods: A retrospective, consecutive review of all patients who had received at least 1 intravitreal injection for wet AMD between January 1, 2004, and December 31, 2013, was conducted using the Rochester Epidemiology Project in Olmsted County, Minnesota. Age- and sex-matched control groups included 473 pre–anti–vascular endothelial growth factor era wet AMD patients, 504 concurrent time dry AMD patients, and 504 patients with no AMD. The rates of AMD and overall malignancy, cutaneous malignancies, and specific types of cutaneous malignancies were compared between groups of patients. Results: Patients with wet AMD incurred an increased rate of overall malignancies compared to patients with dry AMD {52.8% wet AMD (confidence interval [CI]: 48.3–57.2) vs. 43.7% dry AMD (CI: 39.3–48.1); P= 0.003} or those without AMD (52.8% wet AMD [CI: 48.3–57.2] vs. 35.3% no AMD [CI: 31.1–39.7]; P = <0.001). Patients with dry AMD also had higher rates of malignancy than those without AMD (43.7% dry AMD [CI: 39.3–48.1] vs. 35.3% no AMD [CI: 31.1–39.7]; P = 0.007). Rate of cutaneous malignancies was increased in patients with wet AMD compared to patients with dry AMD (24.4% wet AMD [CI: 20.7–28.4] vs. 14.6% dry AMD [CI: 11.5–17.9]; P = <0.001) and those with no AMD (24.4% wet AMD [CI: 20.7–28.4] vs. 9.7% no AMD [CI: 7.3–12.7]; P = <0.001). Conclusion and Relevance: To the best of our knowledge, this is the first report to establish an association between AMD and cutaneous malignancies, supporting a possible discussion of the association when a patient presents with one of the two conditions.

Purpose: To report a case of adult-onset vitelliform macular dystrophy in a patient who was found to have a previously unreported variant of the IMPG2 gene. Methods: Case report. Results: A 65-year-old white woman with no significant medical or ocular history presented with a complaint of persistent wavy vision for 10 months. On funduscopic examination, bilateral vitelliform lesions of approximately 1 mm in the right eye and 0.5 mm in the left eye were evident, with no choroidal neovascularization in either eye. The patient was diagnosed with adult-onset vitelliform macular dystrophy. Genetic testing revealed a single likely pathogenic variant of the IMPG2 gene that may explain the examination findings. Conclusion: Adult-onset vitelliform macular dystrophy is a common and relatively benign condition occurring in approximately 1 in 8,000 individuals. Although vitelliform lesions can be a manifestation of systemic diseases or be idiopathic, in a minority of patients, genetic predisposition may play a role. Mutations in four particular genes BEST1, PRPH2, IMPG1, and IMPG2 have been associated with some cases of adult-onset vitelliform macular dystrophy, with this particular gene variant of IMPG2 being previously unreported.

Introduction This manuscript describes data from an original study, simulating a tele-glaucoma programme in an established clinic practice with an interdisciplinary team. This is a ‘real life’ trial of a telemedicine approach to see a follow-up patient. The goal is to evaluate the accuracy of such a programme to detect worsening and/or unstable disease. Such a programme is attractive since in-clinic time could be reduced for both the patient and provider. This study evaluates agreement between in-person and remote assessment of glaucoma progression. Methods A total of 200 adult glaucoma patients were enrolled at a single institution. The in-person assessment by an optometrist or glaucoma specialist at time of enrolment was used as the gold standard for defining progression. Collated clinical data were then reviewed by four masked providers who classified glaucoma as progression or non-progression in each eye by comparing data from enrolment visit to data from the visit immediately prior to enrolment. Agreement of glaucoma progression between the masked observer and the in-person assessment was determined using Kappa statistics. Intra-observer agreement was calculated using Kappa to compare in-person to remote assessment when both assessments were performed by the same provider ( n = 279 eyes). Results A total of 399 eyes in 200 subjects were analysed. Agreement between in-person versus remote assessment for the determination of glaucoma progression was 63%, 62%, 69% and 68% for each reader 1–4 (kappa values = 0.19, 0.20, 0.35 and 0.33, respectively). For intra-observer agreement, reader 1 agreed with their own in-person assessment for 65% of visits (kappa = 0.18). Discussion Intra-observer agreement was similar to the agreement for each provider who did not see the patient in person. This similarity suggests that telemedicine may be equally effective at identifying glaucomatous disease progression, regardless of whether the same provider performed both in-clinic and remote assessments. However, fair agreement levels highlight a limitation of using only telemedicine data to determine progression compared with clinical detail available during in-patient assessment.

Purpose: Glaucoma specialists and optometrists who work in a team model at a single institution utilize a common definition of glaucoma progression and treatment algorithm. The purpose of this study was to assess the consistency of agreement in identifying glaucoma progression among glaucoma specialists and optometrists of 1 team. Methods: In total, 399 eyes of 200 patients age 18 or older with glaucoma were enrolled over 2 years. Clinical data, disc photographs, optical coherence tomography (OCT), and visual fields were independently reviewed by 2 masked optometrists and 2 masked fellowship-trained glaucoma specialists. Each eye was judged as progression or no progression of glaucomatous disease. The following were assessed: (1) agreement among optometrists; (2) agreement among glaucoma specialists; and (3) agreement among optometrists and glaucoma specialists. The frequency of use of testing modality to determine progression was also studied. κ statistics were used to evaluate agreements. Results: Optometrists agreed with each other for 74.2% of the eyes assessed (κ=0.42), whereas glaucoma specialists agreed with each other for 78.7% of eyes (κ=0.39). All 4 providers agreed with each other for 54.4% of the eyes evaluated (κ=0.37). Providers had the highest agreement when the progression decision was based on disc hemorrhage (92%) and the lowest agreement when based on OCT progression analysis (36%). Compared with optometrists, glaucoma specialists used OCT (P≤0.01) more frequently to determine disease progression. Conclusions: Fair to moderate agreement levels were found among providers in their assessment of glaucoma progression, suggesting that a team approach to glaucoma management may be effective. Further work is needed to investigate ways to optimize consistency within the glaucoma team.