Faculty Bibliography

PURPOSE: To describe the natural course, visual outcomes and anatomic changes and provide histological correlates in eyes with intraretinal hyperreflective foci associated with acquired vitelliform lesions. DESIGN: Retrospective cohort study and imaging-histology correlation in a single donor eye. METHODS: Participants: Patients with intraretinal hyperreflective foci and acquired vitelliform lesions from two tertiary referral centers were evaluated from January 2002-January 2014. MAIN OUTCOME MEASURES: The chronology of clinical and imaging features of retinal anatomic changes and the pattern of intraretinal hyperreflective foci migration were documented using spectral-domain optical coherence tomography (OCT). One donor eye with intraretinal hyperreflective foci was identified in a pathology archive by ex vivo OCT and was studied with high-resolution light and electron microscopic examination. RESULTS: Intraretinal hyperreflective foci were associated with acquired vitelliform lesions in 25 of 254 eyes (9.8%) with a strong female preponderance (86% of patients). Focal disruptions to the ellipsoid zone and external limiting membrane overlying the acquired vitelliform lesions were observed prior to the occurrence of intraretinal hyperreflective foci in 75% of cases. Histological evaluation showed that intraretinal hyperreflective foci represent cells of retinal pigment epithelium origin that are similar to those found in the vitelliform lesions themselves and contain lipofuscin granules, melanolipofuscin granules and melanosomes. The occurrence of intraretinal hyperreflective foci was not a significant determinant of final visual acuity (p=0.34), but development of outer retinal atrophy was significant (p=0.003). CONCLUSIONS: Intraretinal hyperreflective foci associated with acquired vitelliform lesions is of retinal pigment epithelium origin, and the natural course and functional changes are described.

PURPOSE: To assess the treatment response to mineralocorticoid antagonists in a pilot study of patients diagnosed with central serous chorioretinopathy using multimodal imaging. METHODS: This retrospective observational case series included 23 eyes of 14 patients with central serous chorioretinopathy treated by a single physician (L.A.Y.) with either spironolactone, eplerenone, or both consecutively over a 12-month period. Choroidal thickness, central macular thickness, and best-corrected visual acuity were measured and compared with baseline values. Twelve eyes of 11 patients demonstrated subretinal fluid before or during the initiated treatment course. Subretinal fluid was measured and compared with baseline values in this subgroup. RESULTS: In all eyes (n = 23), best-corrected visual acuity improved at 12 months of treatment; however, central macular thickness and choroidal thickness showed no improvement. In the subgroup with subretinal fluid (n = 12), subretinal fluid was significantly decreased at 6 months and 12 months of treatment; however, central macular thickness, choroidal thickness, and best-corrected visual acuity showed no significant change. CONCLUSION: Mineralocorticoid antagonists may improve best-corrected visual acuity and decrease subretinal fluid in patients with central serous chorioretinopathy, but do not affect the choroidal or macular thickness. This pilot study demonstrates that mineralocorticoid receptor antagonists may be effective in treating central serous chorioretinopathy but warrants consideration for future research within a randomized clinical trial.

PURPOSE: To evaluate and characterize multiple evanescent white dot syndrome abnormalities with modern multimodal imaging modalities. METHODS: This retrospective cohort study evaluated fundus photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, enhanced depth imaging optical coherence tomography, short-wavelength autofluorescence, and near-infrared autofluorescence. RESULTS: Thirty-four multiple evanescent white dot syndrome patients with mean age of 28.7 years were studied (range, 14-49 years). Twenty-six patients were women, and eight were men. Initial mean visual acuity was 0.41 logMAR. Final mean visual acuity was 0.03 logMAR. Fluorescein angiography shows a variable number of mid retinal early fluorescent dots distributed in a wreathlike pattern, which correlate to fundus photography, fundus autofluorescence, and indocyanine green angiography. Indocyanine green angiography imaging shows the dots and also hypofluorescent, deeper, and larger spots, which are occasionally confluent, demonstrating a large plaque of deep retinal hypofluorescence. Optical coherence tomography imaging shows multifocal debris centered at and around the ellipsoid layer, corresponding to the location of spots seen with photography, indocyanine green angiography, and fluorescein angiography. Protrusions of the hyperreflectant material from the ellipsoid layer toward the outer nuclear layer correspond to the location of dots seen with photography, indocyanine green angiography, and fluorescein angiography. CONCLUSION: Multimodal imaging analysis of the retina in patients with multiple evanescent white dot syndrome shows additional features that may help in the diagnosis of the disease and in further understanding its etiology. Multiple evanescent white dot syndrome is predominantly a disease of the outer retina, centered at the ellipsoid zone, but also involving the interdigitation zone and the outer nuclear layer.

PURPOSE: To report a posterior segment phenotypic manifestation of Coxsackie virus infection that has not been previously appreciated. METHODS: The clinical course and multimodal imaging findings, including spectral domain optical coherence tomography, fluorescein angiography, near infrared reflectance, and fundus autofluorescence of two patients with Coxsackie virus infections were documented. RESULTS: A neurosensory macular detachment was present in both patients on baseline examination. Fluorescein angiography demonstrated pooling within this lesion and spectral domain optical coherence tomography identified thickening of the retinal pigment epithelial band with variable degrees of attenuation of the ellipsoid zone and interdigitation zone. Fundus autofluorescence and near infrared reflectance imaging revealed multiple satellite lesions adjacent to the neurosensory detachment. These lesions were not seen on fluorescein angiography or color photography. Satellite lesions were hyporeflective on near infrared reflectance imaging and hyperautofluorescent on fundus autofluorescence imaging. The satellite lesions correlated with sites of ellipsoid disruption on spectral domain optical coherence tomography. Both patients were observed and their condition improved over the course of time. There was total resolution of satellite lesions, reconstitution of the ellipsoid zone and interdigitation zone, and return of retinal pigment epithelial thickness to the normal range. A bull's eye pattern of macular retinal pigment epithelial disturbance persisted on color and near infrared reflectance images, despite good visual acuity. CONCLUSION: Posterior segment Coxsackie virus infection may concurrently express the clinical characteristics of acute idiopathic maculopathy and multifocal retinitis. The visual prognosis in this variant is usually favorable. The multimodal imaging features that characterize this entity should be recognized to avoid confusion with other diseases that have a similar presentation.

The clinical presentation of an intraocular nematode unusually affixed to the posterior lens capsule is described. A 64-year-old female patient presented with a 7-year history of gradually declining vision and enlarging central scotoma, but no inflammation. On follow-up 2 years later, vision had further declined and a non-motile, 8-mm nematode was seen affixed to the posterior lens capsule that remained unchanged through final follow-up. The patient disclosed having resided in Africa as a child. Systemic review revealed no evidence of extraocular involvement. Nematode carcasses may remain preserved in the human eye for extended periods without ongoing inflammation.

PURPOSE: To correlate postmortem histology with previously recorded multimodal imaging from a patient with type 1 neovascularization associated with an acquired vitelliform lesion in the setting of age-related macular degeneration (AMD). DESIGN: Case study. METHODS: Multimodal imaging that was obtained ante mortem was matched with ex vivo and high-resolution histological images of the preserved donor macula. Anatomic correlates for multimodal imaging findings were then defined. RESULTS: Spectral-domain optical coherence tomography (OCT) revealed a split in the retinal pigment epithelial-Bruch's membrane band. Type 1 NV in this case was comprised of 6 layered components: (1) Retinal pigment epithelium, (2) Basal laminar deposits, (3) Fibrovascular membrane, (4) Fibrocellular scar, (5) Hemorrhage, and (6) Bruch's membrane. The anatomic correlates for the hyporeflective band on spectral-domain OCT included a thick basal laminar deposit. Not all structures could be readily separated on the basis of their reflectivity patterns. CONCLUSIONS: This is an important clinicopathologic correlation of NV secondary to AMD in the spectral-domain OCT era. Our findings of 6 layers include and extend the anatomical framework encapsulated by the double-layer and triple-layer signs. The resolution of current devices does not always permit distinction of the different layers of NV tissue. Thick basal laminar deposits may appear hyporeflective on spectral-domain OCT and may be confused with fluid from a neovascular process. It will be important to perform a larger clinicopathologic series to aid our anatomical interpretation of spectral-domain OCT images.

PURPOSE: To evaluate the vascular structure of eyes with macular telangiectasia type 2 (MacTel2) using volume-rendered optical coherence tomography angiography (OCTA). DESIGN: Retrospective cross-sectional study. PARTICIPANTS: A total of 14 consecutive patients (20 eyes) with MacTel2 who had a signal strength score >/=55 and could maintain fixation during the scan process. METHODS: The eyes were scanned using optical coherence tomography with split-spectrum amplitude decorrelation techniques to derive flow information. Data were extracted and used to create volume-rendered images of the retinal vasculature that could be rotated about 3 different axes for evaluation. MAIN OUTCOME MEASURES: Descriptive appraisal of the vascular abnormalities associated with MacTel2. RESULTS: Vessels posterior to the outer boundary of the deep retinal plexus were secondary to retinal thinning, vascular invasion, or a combination of both. These vessels had the same shape and distribution as the late staining seen during conventional fluorescein angiography. Lateral contraction in the temporal macula in 5 eyes created an appearance of vessels radiating from a central locus, which was the site of a right angle vein. Loss of macular tissue as part of the disease process led to a central amalgamation of the inner vascular plexus and the deep vascular plexus, which appeared to be in a state of decline. Subretinal neovascularization originated from the retinal circulation but involved not only the subretinal space but also could infiltrate the remaining, thinned, retina. CONCLUSIONS: Volume rendering of OCTA information preserves the 3-dimensional relationships among retinal vascular layers and provides opportunities to visualize retinal vascular abnormalities in unprecedented detail. The retinal vascular leakage and invasion in MacTel2 may arise as a consequence of loss of control with depletion of Muller cells and exposure of the remaining retinal vessels to the more hypoxic environment near the inner segments of the photoreceptors.

PURPOSE: To use volume-rendered optical coherence tomography angiography to investigate vascular proliferation in macular telangiectasia type 2 (MacTel2), extending beyond the retinal pigment epithelium (RPE). METHODS: Six eyes of four patients with MacTel2 with neovascularization proliferating external to the RPE confines were studied. Eyes were scanned using optical coherence tomography using split-spectrum amplitude-decorrelation techniques to derive flow information (RTVue XR; Optovue). These data were extracted and used to create volume rendered images of the area of vascular proliferation. RESULTS: Mean age was 66.2 years. There was demonstrable vascular proliferation in the sub-RPE space observable by both optical coherence tomography and optical coherence tomography angiography. Fibrovascular RPE detachments were identified in all eyes. The topographic distribution of abnormal vessels located below the plane of the deep retinal vascular plexus and above the RPE closely matched the pattern of hyperfluorescence and leakage on fluorescein angiography. Vessels under the RPE demonstrated different branching patterns and larger diameter lumens than those above the RPE, but anastomosis with the choroidal circulation was difficult to demonstrate. CONCLUSION: This study provides evidence that sub-RPE vascular proliferation may be a complication of MacTel2. Retinal pigment epithelium abnormalities are known to occur in MacTel2 and may provide a conduit for abnormal vessels in the subretinal space to proliferate into the sub-RPE compartment. The authors have no reason to exclude the possibility that the choroid contributes to the deep proliferation.

Macular holes in the setting of macular telangiectasia type 2 can be difficult to manage. The rates of anatomical closure after macular hole surgery are less favorable in patients with macular telangiectasia than in those with idiopathic macular holes. These differences may be due to the influence of unique pathogenic mechanisms that modulate macular hole dynamics in patients with macular telangiectasia. In this report, the authors document the multimodal imaging findings of a patient with macular telangiectasia demonstrating spontaneous macular hole closure. These findings may improve the understanding of the natural course of this entity and may have relevance for clinical management. [Ophthalmic Surg Lasers Imaging Retina. 2015;46:883-886.].

PURPOSE: To present a case of an HIV-positive patient with acute exudative polymorphous vitelliform maculopathy (AEPVM) and evaluate the presence of specific spectral-domain optical coherence tomography (SD-OCT) findings. METHODS: Case report. RESULTS: We reviewed the AEPVM cases reported in the literature and compared those to our patient to determine if there is a correspondence between the etiology that leads to the onset of AEPVM and clinical and SD-OCT findings. CONCLUSIONS: Acute exudative polymorphous vitelliform maculopathy is a disease that involves the outer retinal layers with lipofuscin deposits and serous detachment of the neuroepithelium with or without intraretinal cysts. Not much is known about the etiology and pathogenesis, and not many cases have been described. A review of the few clinical cases reported in the literature does not show a specific correspondence between etiology and SD-OCT findings.