Faculty Bibliography

PURPOSE/OBJECTIVE:To describe a variant of acute zonal occult outer retinopathy (AZOOR) that has concentric involvement of the peripheral retina with centripetal progression toward the posterior pole. METHODS:Three patients with AZOOR were reported to show peripheral concentric zonal involvement with centripetal progression of their disease from the periphery to the posterior fundus. RESULTS:All three cases involved elderly hyperopic women with a history of autoimmune disease. All six eyes showed bilateral central peripapillary AZOOR lesions that progressed in a centrifugal manner to the periphery. Five of the six eyes showed the presence of concentric peripheral zonal abnormalities that progressed in a centripetal manner to the posterior pole. In one case, the peripheral and central zonal abnormalities became confluent, leaving only a small island of normal retina temporal to the fovea. CONCLUSION/CONCLUSIONS:A variant of AZOOR may involve the peripheral retina, causing concentric zonal atrophy with centripetal progression, with central peripapillary zonal abnormalities that have centrifugal progression. This may eventually lead to widespread atrophic degeneration with severe visual field loss. Wide-field imaging of the peripheral retina and monitoring of the visual fields are important to document this rare atypical presentation of AZOOR and any subsequent disease progression.

PURPOSE/OBJECTIVE:To evaluate multimodal imaging including volume-rendered angiographic and structural optical coherence tomography of macular telangiectasia Type 2 (MacTel2) for right-angle vein complexes, macular cavitations, and signs of deeper retinal vascular invasion. METHODS:Retrospective review of imaging performed in a community-based retinal referral center. The eyes were scanned using optical coherence tomography using split-spectrum amplitude-decorrelation techniques to derive flow information. These data were extracted and used to create volume-rendered images of the retinal vasculature with integrated structural information derived from the component optical coherence tomographic images. RESULTS:There were 24 eyes of 16 patients who had a mean age of 61.8 years. Right-angle veins seemed in association with vascular proliferation external to the deep vascular plexus. The origin of a right-angle vein was surrounded by a stellate arrangement of radiating retinal vessels apparently caused by contraction of surrounding tissue in the temporal macula. Cavitations were found in the fovea and varied in size and configuration from one examination to the next. Many smaller cavitations, called microcavitations, were seen in the surrounding macula. Vascular invasion occurred into the subretinal space. CONCLUSION/CONCLUSIONS:There are contractile features of the tissue in the temporal macula and the number, size, and temporal variations in the cavitations have not been in not mentioned in previous published descriptions of MacTel2. Vascular invasion of deeper layers occurred in the temporal macula through the outer nuclear layer. Volume-rendered angiographic and structural optical coherence tomography offers unprecedented ability to examine the vascular interrelationships their associations with cavitations in the macula.

PURPOSE: To study the cross-sectional and en face optical coherence tomography angiography (OCTA) findings in Type 3 neovascularization (NV). METHODS: Optical coherence tomography angiography imaging of 27 eyes of 23 patients with Type 3 NV was analyzed with 9 eyes having consecutive follow-up OCTA studies. RESULTS: Type 3 NV appeared as a linear high-flow structure on cross-sectional OCTA corresponding to a high-flow tuft of vessels seen on en face OCTA. Cross-sectional OCTA seemed to enable the distinction between vascular and nonvascular intraretinal hyperreflective foci. Two patterns of flow were observed; Pattern 1 (11%): a flow signal confined to the neurosensory retina and Pattern 2 (74%): a flow signal extending through the retinal pigment epithelium. No definitive retinal-choroidal anastomosis was observed; however, projection artifacts confounded the interpretation of deeper structures. An increase in the intensity of the high-flow tuft was seen during the progression or recurrence of Type 3 NV. Intravitreal anti-vascular endothelial growth factor therapy caused a reduction in the intensity of the high-flow tuft which was not sustained. CONCLUSION: Compared with conventional imaging, OCTA may improve detection and delineation of vascular changes occurring in Type 3 NV. Cross-sectional and en face OCTA may prove useful in studying the pathogenesis and guiding the management of these lesions.

PURPOSE: To report a case of unilateral stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) in a young male showing structural changes induced by a Valsalva maneuver. METHODS: Case report of a 26-year-old oboist with SNIFR, including multimodal imaging. Eye-tracked spectral-domain optical coherence tomography (SD-OCT) was used to compare the retinal architecture at rest and during a Valsalva maneuver. RESULTS: Spectral-domain optical coherence tomography showed macular and peripapillary retinoschisis with no signs of pathologic myopia, optic pit, or vitreoretinal traction. A full-field electroretinogram showed supranormal responses in the eye studied. Magnetic resonance imaging of the brain showed no abnormalities. Eye-tracked SD-OCT scans showed an increase in retinal thickness reaching 28 microns superior to the disc during an induced Valsalva maneuver. CONCLUSION: Stellate nonhereditary idiopathic foveomacular retinoschisis is a diagnosis made when other known causes of retinoschisis have been excluded. In this patient with unilateral SNIFR, an increase in retinal thickness during a Valsalva maneuver was demonstrated. Further study would be needed to determine the mechanism producing this change and to assess its potential influence on visual prognosis.

PURPOSE/OBJECTIVE:To report the pathogenic factors that account for cystoid macular edema and cystoid macular degeneration in chronic central serous chorioretinopathy (CSC). METHODS:The clinical course and multimodal imaging findings, including fundus color photography, fundus autofluorescence, spectral-domain optical coherence tomography, and fluorescein angiography, of one eye with cystoid macular edema due to chronic CSC was documented. RESULTS:A 44-year old woman with a history of chronic CSC presented with progressive visual decline in the right eye. Best-corrected visual acuity was 20/40. Funduscopic examination revealed diffuse retinal pigment epithelial changes and macular edema. Fluorescein angiography demonstrated perifoveal microaneurysms and leakage in a petaloid configuration. Spectral-domain optical coherence tomography demonstrated cysts at the level of the inner nuclear layer, an epiretinal membrane, vitreomacular traction, and an attenuated retinal pigment epithelial band. Central subfield thickness was 486 μm. Three intravitreal injections of aflibercept were administered over 16 weeks following which there was resolution of leakage, release of vitreomacular traction, and resolution of microaneurysms. Central subfield thickness reduced to 379 μm, but persistent intraretinal cysts were observed. There was subjective improvement in visual symptoms, but Snellen acuity remained at 20/40. CONCLUSION/CONCLUSIONS:Intraretinal cystic changes in chronic CSC may be the result of multifactorial pathogenic factors and may represent the coexistence of cystoid macular edema and cystoid macular degeneration. Anti-vascular endothelial growth factor may play an important role in the treatment of cystoid macular edema caused by CSC.

PURPOSE: To report novel observations of previously described solitary circumscribed retinal astrocytic proliferation using spectral domain optical coherence tomography that suggests this tumor does not arise in the nerve fiber layer as initially believed, but arises within deep retinal or retinal pigment epithelial structures. METHODS: Retrospective review of four cases. RESULTS: Patient age ranged from 46 to 75 years. The tumor was pearl white or yellow-white (n = 4, 100%), located in the macula (n = 1, 25%) or macula to equator (n = 3, 75%) regions, and with mean tumor base of 1.2 mm and thickness of 0.8 mm. There were no feeding vessels, intrinsic vessels, subretinal fluid, or vitreoretinal traction. Mild surrounding retinal pigment epithelial hyperplasia and atrophy rimmed each tumor (n = 4, 100%). Fluorescein angiography depicted the mass with early hypofluorescence (n = 3/3, 100%) and late hypofluorescence (n = 2/3, 67%). Spectral domain optical coherence tomography demonstrated the mass with an abruptly elevated "snowball" configuration (n = 4, 100%), with smooth or slightly irregular surface (n = 4, 100%), and originating from deep retina or retinal pigment epithelial (n = 4, 100%), with overlying compression and draping of retinal tissue (n = 4, 100%). CONCLUSION: This previously described small yellow-white retinal tumor appears to arise in the outer retinal layers and not from the inner retinal layers as formerly believed. This tumor may not be astrocytic as initially believed since it arises deep within the retina, but it could represent a deep glial or pigment epithelial fibrous mass. The pathogenesis and pathology of this rare lesion remain unknown.

PURPOSE: To quantify the temporal properties of the acquired vitelliform lesion (AVL) lifecycle, define the clinical characteristics of choroidal neovascularization (NV) in this setting and determine the predictors of long-term visual outcomes. DESIGN: Retrospective cohort study METHODS: Clinical and imaging data from 199 eyes of 124 consecutive patients with AVLs associated with age-related macular degeneration (AMD) and adult-onset foveomacular vitelliform dystrophy (AOFVD) were analyzed. Volumetric calculations of vitelliform material were determined using spectral-domain optical coherence tomography and the temporal properties of the AVL lifecycle were quantified. The clinical characteristics of NV were assessed as were the predictors of final best-corrected visual acuity (BCVA) and change in BCVA. RESULTS: Mean age was 79.2+/-12.1 years. AVLs grew and collapsed at approximately the same rate (P = 0.275). Fifteen eyes (7.5%) developed NV of which all were type 1. In 13 of these eyes, NV occurred during the collapse phase of the AVL lifecycle, after the peak AVL volume was reached. The risk of NV (P = 0.006) and the decline in BCVA (P = 0.001) were both significantly greater among eyes with AMD. Foveal atrophy was the characteristic most significantly associated with final BCVA and change in BCVA from baseline (both P < 0.0005). The development of NV was not predictive of long-term visual outcomes (all P = 0.216). CONCLUSIONS: Complications associated with AVLs typically occur during the collapse phase of the AVL lifecycle. Visual outcomes and risk of NV are related to the underlying disease associated with AVLs.

PURPOSE/OBJECTIVE:To describe and compare the clinical and imaging characteristics of pigment epithelial detachments (PEDs) in age-related macular degeneration (AMD), polypoidal choroidal vasculopathy (PCV), and central serous chorioretinopathy (CSC) as seen in a clinical setting of a tertiary retinal practice. DESIGN/METHODS:A perspective supported by clinical and imaging characteristics of a consecutive cohort of patients with strictly defined PEDs. RESULTS:One hundred seventy-four eyes of 113 patients with PEDs were studied with comprehensive clinical retinal examination and multimodal imaging; PEDs were differentiated into nonvascularized and vascularized forms with 3 main underlying etiologies: AMD (76%), PCV (9%), and CSC (3%). AMD was the most common diagnosis, with both nonvascularized PEDs (drusenoid and serous) and vascularized PEDs (type 1 and type 3 neovascularization) associated with drusen and a thin choroid. PCV patients had large, vascularized, peaked PEDs associated with polyps and a variable choroidal thickness, while CSC patients had a thick choroid and predominantly nonvascularized, serous PEDs with an overlying neurosensory detachment. The combined clinical and imaging characteristics form a profile for each PED subtype related to their underlying disease. However, atypical features noted in 11% of patients may complicate the underlying diagnosis. CONCLUSION/CONCLUSIONS:Typical phenotypic manifestations of PEDs and other features seen with multimodal imaging were associated with specific underlying etiologies. As suggested by our study, identification of these features help clinicians to determine the precise underlying etiology and manage both vascularized PEDs, where evidence-based treatment exists, and nonvascularized PEDs, where current treatment is not supported by convincing evidence.