Faculty Bibliography

Purpose/UNASSIGNED:To describe features of neovascularization in proliferative diabetic retinopathy (PDR) using optical coherence tomography angiography (OCTA). Methods/UNASSIGNED:A retrospective case series was performed in 23 eyes from 21 patients who underwent OCTA of neovascular complexes (NVCs) due to PDR. Eyes were imaged with the DRI Triton swept-source OCTA, Avanti RTVue XR or Cirrus HD-OCT 5000 as part of routine clinical examination. Segmentation was adjusted to include vasculature between the vitreous cavity and the internal limiting membrane (ILM). The presence of NVCs was confirmed by clinical examination and multimodal imaging such as color or red-free fundus photography, fluorescein angiography, multicolor imaging or near-infrared reflectance. Results/UNASSIGNED:Thirty-five NVCs were imaged, of which, 34% were neovascularization of the disc (NVD) and 66% were neovascularization elsewhere (NVE). On structural OCT B-scans, NVE appeared as medium to highly reflective tissue that breached the ILM, while NVD showed highly reflective tissue protruding from the disc in a sea fan configuration. Flow signal was seen on OCTA in all cases of NVE and in 67% of NVD lesions. Areas with minimal or absent retinal flow signal identified retinal nonperfusion areas and were found adjacent to 87% of NVE. Intraretinal microvascular abnormalities (IRMAs) were noted next to 70% of NVE. Absent flow signal was seen in 4 NVD cases showing posterior shadowing and were considered inactive. Conclusion/UNASSIGNED:OCTA appears useful for imaging NVCs, IRMAs, and retinal nonperfusion areas in eyes with diabetic retinopathy. This imaging modality enables noninvasive screening and monitoring of PDR and can obviate the need for additional testing in certain clinical settings.

Purpose : Age-related macular degeneration (AMD), a leading cause of blindness, is characterized by the formation of drusen. Subretinal drusenoid deposits (SDD) sit over the retinal pigment epithelium, are a proven risk factor for AMD, and can be visualized on cross-sectional spectral domain optical coherence tomography (SD-OCT). We are evaluating the relationship between SDD and serum risk factors for cardiovascular disease (CVD) in AMD. Methods : 58 subjects (ages of 50-90), diagnosed with AMD on two successive examinations, were recruited prospectively, provided blood samples and underwent SDOCT. The presence of SDD was determined on SD-OCT by an experienced examiner, and a panel of serum risk factors performed: Total Cholesterol (TC), Triglycerides (TG), HDL, VLDL, LDL Direct and high sensitivity C-reactive protein (hs-CRP). Independent t-tests were performed on these results. Results : 25 of 58 subjects were found to have SDD present on SD-OCT. Independent ttests demonstrated a significant association (p<0.05) of high TC (>149 mg/dL) (p=0.01) and low HDL (<39mg/dL) (p=0.02) in subjects with SDD compared to subjects without SDD. Post hoc analysis showed that of patients with high TC, 52% had SDD and 22% did not have SDD present (p=0.02); of patients with low HDL, 4% had SDD, 12% did not (p=0.04); of patients with high LDL-direct, 72% had SDD and 55% did not have SDD (p=0.05). Results for TG and hsCRP were inconclusive. Conclusions : The relationship of TC and AMD is inconsistently reported in the literature, with no demonstrated difference between the phenotypes of AMD. Our results demonstrate that high TC is a specific and significant risk factor for AMD with SDD compared to AMD without SDD. High TC is also a strong risk factor for CVD. Therefore, the leading cause of blindness (AMD) can now be connected to the leading cause of death (CVD) through its SDD phenotype, which may be an effective ophthalmic predictor for future CVD. High LDL is a strong risk for CVD as well, and the risk for SDD also approached significance. High HDL is a known risk for AMD, and per our study, may confer even greater risk for SDD. One limitation of our study is that the groups were not matched for other independent CVD risk factors such as hypertension and smoking history, which

To describe the ocurrence of Bartonella-associated neuroretinitis secondary to non-feline pet exposure, we retrospectively reviewed medical records and imaging from patients with a clinical and serologic diagnosis of Bartonella henselae (BH). Retinal imaging included color fundus photography, optical coherence tomography (OCT) and fluorescein angiography (FA). Four eyes of two patients with cat-scratch disease were included in this study, with a mean age of 35 years. The mean follow-up was 13 months, after presentation of infectious neuroretinitis. Both patients suffered from bilateral neuroretinitis after direct contact with family pets (ferret and guinea pig). All patients were treated with a long-term systemic antimicrobial therapy. Visual acuity in all improved to 20/30 or better at six months. In conclusion, humans may develop cat-scratch disease when they are exposed to Bartonella henselae (BH) in the saliva of infected cats or BH-containing flea feces reaching the systemic circulation through scratches or mucous membranes. As the cat flea (Ctenocephalides felis) may reside on non-feline mammals, Bartonella-associated neuroretinitis may result from contact with other furred family pets.

PURPOSE: To report two cases of choroidal nevi associated with focal choroidal excavation (FCE) and polypoidal choroidal neovascularization (PCN). METHODS: Report of two patients with choroidal nevi showing FCE and PCN who underwent multimodal imaging including color fundus photography, fluorescein angiography, indocyanine green angiography, fundus autofluorescence, spectral domain optical coherence tomography, swept-source optical coherence tomography, and optical coherence tomography angiography. RESULTS: Two patients presented with choroidal nevi associated with FCE and PCN. In the first case, a 74-year-old woman, the nevus had sharp margins, a deep FCE, surrounding drusen, and subretinal exudation at its inferior edge due to PCN that responded well to intravitreal anti-vascular endothelial growth factor therapy. In the second case, a 64-year-old woman, the nevus had ill-defined margins, a shallow FCE, and angiographic evidence of PCN without associated exudation. CONCLUSION: There have been several reports showing an association of either choroidal nevi or FCE with PCN. To our knowledge, there have been no previous reports of FCE identified within choroidal nevi, with or without associated PCN. Since, in one of our cases, the FCE was not apparent on clinical examination, the prevalence of FCE within nevi may be underdiagnosed.

PURPOSE: Congenital retinal macrovessels are large aberrant retinal blood vessels that cross the horizontal raphe and can traverse the central macula. Using multimodal imaging and optical coherence tomography angiography, we describe 2 cases of congenital retinal macrovessel associated with macroaneurysms. METHODS: Two patients presented for evaluation and were found to have congenital retinal macrovessels associated with macroaneurysms. Color photography, optical coherence tomography, fundus autofluorescence fluorescein angiography, and optical coherence tomography angiography were performed and used to establish the diagnosis and monitor resolution at follow-up visits. RESULTS: The first patient presented with central vision loss in the right eye and was noted to have a ruptured macroaneurysm and scattered microaneurysms along the course of a venous macrovessel. After 3 months of observation, the patient's vision improved. The second patient presented for evaluation of a cataract in her left eye and was incidentally found to have an arterial macrovessel in her right eye with an associated macroaneurysm. Both cases demonstrated an intricate capillary network in the central macula best visualized on optical coherence tomography angiography. CONCLUSION: Macroaneurysms can occur on both arterial and venous macrovessels. After rupture of these lesions, hemorrhage and exudation can resolve with observation alone. Macrovessels can also present with microaneurysms. Optical coherence tomography angiography can effectively image the complex capillary network associated with these vascular anomalies.

PURPOSE: We describe the long-term follow-up of a patient with multifocal Best disease with chronic bilateral serous macular detachment and unusual peripheral findings associated with a novel mutation in the BEST1 gene. METHODS: Case report. RESULTS: A 59-year-old white woman was referred for an evaluation of her macular findings in 1992. There was a family history of Best disease in the patient's mother and a male sibling. Her medical history was unremarkable. Best-corrected visual acuity was 20/20 in her right eye and 20/25 in her left eye. The anterior segment examination was normal in both eyes. Funduscopic examination showed multifocal hyperautofluorescent vitelliform deposits with areas of subretinal fibrosis in both eyes. An electrooculogram showed Arden ratios of 1.32 in the right eye and 1.97 in the left eye. Ultra-widefield color and fundus autofluorescence imaging showed degenerative retinal changes in areas throughout the entire fundus in both eyes. Optical coherence tomography, including annual eye-tracked scans from 2005 to 2016, showed persistent bilateral serous macular detachments. Despite chronic foveal detachment, visual acuity was 20/25 in her right eye and 20/40 in her left eye, 24 years after initial presentation. Genetic testing showed a novel c.238T>A (p.Phe80Ile) missense mutation in the BEST1 gene. CONCLUSION: Some patients with Best disease associated with chronic serous macular detachment can maintain good visual acuity over an extended follow-up. To our knowledge, this is the first report of Best disease associated with this mutation in the BEST1 gene.

PURPOSE/OBJECTIVE:To describe two cases of retinal detachment with hydration folds and discuss the possible cause of these outer retinal abnormalities. METHODS:The medical and imaging records of two patients with retinal detachment and hydration folds were examined. PATIENTS/METHODS:A 43-year-old myopic woman who developed a retinal detachment secondary to a macular hole and a 35-year-old man referred with a rhegmatogenous retinal detachment masquerading as an exudative detachment were each found to have retinal hydration folds. RESULTS:On near-infrared reflectance imaging, the hydration folds appeared similar to eddy currents, and these corresponded to curvilinear outer retinal plications on optical coherence tomography. The photoreceptor outer segments appeared thickened and elongated, and there was apparent lateral expansion of the outer retinal layers. CONCLUSION/CONCLUSIONS:Hydration folds are found in rhegmatogenous retinal detachment and demonstrate reproducible imaging characteristics on near-infrared imaging and optical coherence tomography. The cause for such outer retinal plications is currently unknown. We suspect that they form as a result of hydration of the glycosaminoglycans in the interphotoreceptor matrix, which lies between the photoreceptors. Additional studies are warranted to explore this pathophysiology.

PURPOSE: To report an unusual case of an elderly patient with transient outer retinal disruption resembling bilateral multiple evanescent white dot syndrome. METHODS: Observational case report. Fundus photographs, fluorescein angiography, standard and ultra-widefield fundus autofluorescence, and cross-sectional and en face optical coherence tomography were used to characterize and describe the clinical findings. RESULTS: A 67-year-old woman presented with decreased vision and floaters in her left eye. Best-corrected visual acuity was 20/20-3 in the right eye and 20/80-2 in the left eye. Funduscopic examination showed small deep white dots and foveal granularity of the left eye corresponding to hyperautofluorescent spots on fundus autofluorescence and ellipsoid zone disruption on spectral domain optical coherence tomography. The asymptomatic right eye had evidence of subretinal deposits on spectral domain optical coherence tomography but was otherwise unremarkable. At 4-week follow-up, the patient noted resolution of her symptoms in the left eye but had developed floaters and blurry vision in her right eye. The left eye showed resolving white spots and ellipsoid zone disruption. However, the right eye had new evidence of white spots corresponding to hyperautofluorescent spots on fundus autofluorescence. Spectral domain optical coherence tomography demonstrated subretinal deposits overlying areas of ellipsoid zone disruption. At 8-week follow-up, the patient was asymptomatic in both eyes with best-corrected visual acuity of 20/20 in both eyes. The hyperautofluorescent spots on ultra-widefield fundus autofluorescence had faded with restoration of ellipsoid zone disruption in both eyes and disappearance of subretinal deposits. CONCLUSION: Our case demonstrates multimodal retinal imaging findings resembling multiple evanescent white dot syndrome in an elderly patient. The bilateral presentation, presence of subretinal deposits before symptom onset, and older age of the patient were atypical features for this entity.