Faculty Bibliography

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 To evaluate the diagnostic yield and clinical impact of the SPARK/Invitae 248 gene panel in patients with known or suspected inherited degenerative retinal disease, in comparison with traditional clinical assessments including ERG testing. Methods Patients were drawn from the clinical practices of the authors. Genetic screening was provided by means of the SPARK/Invitae 248-gene panel at no charge to patients. Over the past year, results are available for 18 patients. Clinical diagnoses were Best vitelliform dystrophy (definite in one patient, suspected in three patients); retinitis pigmentosa (five affected patients, one likely female carrier); possible Stargardt disease (five patients); and one each with Leber congenital amaurosis, choroideremia, nyctalopia, and Norrie disease. Results Of the 18 studies returned, results were deemed by Invitae ''Positive'' in nine and ''Uncertain'' in nine. The genetic diagnosis agreed with the clinical diagnosis in eight cases (including two patients diagnosed with retinitis pigmentosa with homozygous mutations in USH2A, and one patient with the diagnosis of retinitis pigmentosa with compound heterozygotic mutations in NR2E3, which is also associated with enhanced S-cone syndrome). One patient with macular degeneration of uncertain etiology had a deletion in TPP1, which codes for ceroid lipofuscinosis. The remaining nine patients all returned heterozygous mutations considered ''variations of uncertain significance'' (VUS) ranging from two to eight different genes. Heterozygous VUS in ABCA4 were noted in two patients with the clinical diagnosis of Stargardt disease and in one with the clinical diagnosis of Best disease. Conclusion The SPARK/Invitae 248-gene panel provided a genetic diagnosis consistent with the clinical diagnosis in about half of cases and was diagnostic in one case where the clinical diagnosis was unclear. Retinitis pigmentosa and Stargardt disease were the most common genetic diagnoses. No cases of Leber congenital amaurosis were detected by genetic testing which had been misdiagnosed clinically.

PURPOSE/OBJECTIVE:To report a rare case of vitreous cavity-Tenon capsule fistula formation after removal of a symptomatic hydrogel scleral buckle. METHODS:Case report. RESULTS:A 43-year-old man presented with chronic headache and involuntary gaze deviation for over 1 year after hydrogel scleral buckle surgery 25 years prior. After removal of the scleral buckle, the patient developed a fluid-filled inflation of the buckle capsule, surrounding a previously noted area of severe scleral thinning. Ocular ultrasonography suggested a fistulous connection between the vitreous cavity and the sub-Tenon space in the area of scleral thinning. There was resolution of diplopia and headache postoperatively, with stability of the fluid collection on clinical examination. Because of high risk of further surgery and resolution of the patient's symptoms, conservative management was elected. CONCLUSION/CONCLUSIONS:This is the first report, to the best of our knowledge, of Tenon capsule-vitreous cavity fistula formation after scleral buckle explantation. Because of innate ability to expand, as well as tendency to become friable, hydrogel buckles have a higher risk of requiring removal and of complications from explantation, respectively. Our patient experienced relief of symptoms, without complication from the fistula, and was successfully managed conservatively.

BACKGROUND:Inadequate screening of treatment-warranted retinopathy of prematurity (ROP) can lead to devastating visual outcomes. Especially in resource-poor communities, the use of an affordable, portable, and easy to use smartphone-based non-contact fundus photography device may prove useful for screening for high-risk ROP. This study evaluates the feasibility of screening for high-risk ROP using a novel smartphone-based fundus photography device, RetinaScope. METHODS:Retinal images were obtained using RetinaScope on a cohort of prematurely born infants during routine examinations for ROP. Images were reviewed by two masked graders who determined the image quality, the presence or absence of plus disease, and whether there was retinopathy that met predefined criteria for referral. The agreement between image-based assessments was compared to the gold standard indirect ophthalmoscopic assessment. RESULTS:Fifty-four eyes of 27 infants were included. A wide-field fundus photograph was obtained using RetinaScope. Image quality was acceptable or excellent in 98% and 95% of cases. There was substantial agreement between the gold standard and photographic assessment of presence or absence of plus disease (Cohen's κ = 0.85). Intergrader agreement on the presence of any retinopathy in photographs was also high (κ = 0.92). CONCLUSIONS:RetinaScope can capture digital retinal photographs of prematurely born infants with good image quality for grading of plus disease.

This is a rare, multimodal imaging report spanning a decade of monitoring in a patient with chronic solar retinopathy showing the natural course of the disease. Spectral-domain optical coherence tomography (SD-OCT) showed mild widening of subfoveal loss of ellipsoid and interdigitation zones bilaterally, progressive retinal pigment epithelial thinning in the right eye, and hyperplasia in the left eye. Structural en face OCT showed subfoveal tissue loss bilaterally. There was no leakage on fluorescein angiography and OCT angiography (OCTA), and dense B-scan OCTA images were unremarkable. Microperimetry revealed bilateral decreased central sensitivity and eccentric fixation in the left eye. Vision remained stable throughout. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:388-392.].

PURPOSE/OBJECTIVE:To assess whether publication of Comparison of Age-related macular degeneration Treatment Trial (CATT) results and introduction of aflibercept to the marketplace affected intravitreal bevacizumab and ranibizumab utilization. DESIGN/METHODS:Retrospective analysis of treatment patterns. METHODS:We calculated weekly bevacizumab and ranibizumab utilization during 3 timeframes: (1) before CATT publication, (2) between CATT publication (4/28/2011) and assignment of a unique aflibercept billing code (1/1/2013), and (3) afterwards for 164188 Medicare beneficiaries with neovascular macular degeneration receiving ≥1 anti-Vascular Endothelial Growth Factor injections from 1/1/2008 to 12/31/2014. We identified ophthalmologists who predominantly (≥80%) administered bevacizumab or ranibizumab, and evaluated changes in preferences over the 3 periods. We replicated analyses on 881381 commercially-insured beneficiaries. RESULTS:Among 317 ophthalmologists administering predominantly ranibizumab to Medicare beneficiaries pre-CATT, 221 (69.7%) reduced ranibizumab utilization post-CATT, whereas 96 (30.3%) continued using ranibizumab ≥80% of the time. Findings were reversed among 1041 ophthalmologists who predominantly administered bevacizumab pre-CATT-777 (74.6%) continued bevacizumab-predominant use while 264 (25.4%) reduced bevacizumab utilization post-CATT. Among the 145 ophthalmologists who predominantly administered ranibizumab before aflibercept's availability, 77 (53.1%) reduced ranibizumab utilization and 68 (46.9%) continued using ranibizumab ≥80% of the time after aflibercept became available. Corresponding numbers among the 909 ophthalmologists who predominantly administered bevacizumab pre-aflibercept were 381 (41.9%) reducing and 528 (58.1%) continuing bevacizumab-predominant use. Similar results were observed for commercially-insured patients. CONCLUSIONS:Many ophthalmologists who favored ranibizumab switched to bevacizumab after CATT publication while most who favored bevacizumab prior to CATT publication continued favoring it afterwards. Aflibercept's introduction had little impact on preferences for ranibizumab or bevacizumab.

Purpose/UNASSIGNED:An important, unmet clinical need is for cost-effective, reliable, easy-to-use, and portable retinal photography to evaluate preventable causes of vision loss in children. This study presents the feasibility of a novel smartphone-based retinal imaging device tailored to imaging the pediatric fundus. Methods/UNASSIGNED:Several modifications for children were made to our previous device, including a child-friendly 3D printed housing of animals, attention-grabbing targets, enhanced image stitching, and video-recording capabilities. Retinal photographs were obtained in children undergoing routine dilated eye examination. Experienced masked retina-specialist graders determined photograph quality and made diagnoses based on the images, which were compared to the treating clinician's diagnosis. Results/UNASSIGNED:Dilated fundus photographs were acquired in 43 patients with a mean age of 6.7 years. The diagnoses included retinoblastoma, Coats' disease, commotio retinae, and optic nerve hypoplasia, among others. Mean time to acquire five standard photographs totaling 90-degree field of vision was 2.3 ± 1.1 minutes. Patients rated their experience of image acquisition favorably, with a Likert score of 4.6 ± 0.8 out of 5. There was 96% agreement between image-based diagnosis and the treating clinician's diagnosis. Conclusions/UNASSIGNED:We report a handheld smartphone-based device with modifications tailored for wide-field fundus photography in pediatric patients that can rapidly acquire fundus photos while being well-tolerated. Translational Relevance/UNASSIGNED:Advances in handheld smartphone-based fundus photography devices decrease the technical barrier for image acquisition in children and may potentially increase access to ophthalmic care in communities with limited resources.