Faculty Bibliography

PURPOSE/OBJECTIVE:To analyze the changes in visual acuity and subfoveal choroidal thickness in patients with non-neovascular age-related macular degeneration (AMD) and drusenoid pigment epithelium detachments (PED). DESIGN/METHODS:Consecutive observational case series. METHODS:Observational retrospective review of eyes diagnosed with drusenoid PED in a single clinical setting. Demographic and clinical data included age, gender, laterality, best corrected visual acuity (BCVA) and subfoveal choroidal thickness measured at baseline, before and after the collapse of the PED, and at the last available follow-up. The presence of geographic atrophy (GA) was also assessed. RESULTS:Thirty-seven eyes of 25 patients (18 females) were included in the analysis. Mean age at baseline was 71 ± 8.4 years. During a mean follow-up period of 4.9 ±1.9 years, PED collapse was observed in 25 eyes (68%). Mean BCVA, mean maximum PED height and mean subfoveal choroidal thickness significantly decreased from baseline to the last available follow-up (p<0.001) in patients showing PED collapse. Choroidal thinning was faster during the PED collapse (speed rate of 35.9 microns/year). From those, 23 eyes (92%) developed GA. A significant correlation between the area of GA and the decrease in choroidal thickness was found (p=0.010). CONCLUSIONS:Choroidal thickness significantly decreased in eyes showing drusenoid PED collapse, but not in eyes in which the PED persisted. A significant correlation with resultant GA area following PED collapse and the magnitude of choroidal thinning was found. Further studies are warranted to better understand the mechanisms involved in the occurrence of choroidal changes during the lifecycle of drusenoid PEDs.

PURPOSE/OBJECTIVE:To examine the alterations in retinal vascular morphology over an extended follow-up in eyes with macular telangiectasis Type 2 (MacTel2). METHODS:Eyes with high-quality digital photographs were evaluated. The geometric distortion in baseline images required to emulate the follow-up images was determined and vectors were made that represented the direction and magnitude of changes, to create a warp field. Optical coherence tomography and optical coherence tomography angiography evaluation of the retina was performed. RESULTS:There were 7 eyes of 4 patients, who had a mean age of 70.25 years, which were followed for a mean of 8.8 years. The eyes showed increasing grayish opacification in the temporal macula with straightening and displacement of the macular vessels, even those in the nasal macula. The warp field vectors pointed to the temporal juxtafoveal macula. There was never any cavitation at the epicenter of the retinal distortion in any patient, although cavitations were found around this area. Optical coherence tomography imaging showed a circumscribed region of hyperreflectivity in the temporal macula. Optical coherence tomography angiography showed a deep angular condensed network of vessels within the hyperreflective region. One eye showed marked atrophic changes including full-thickness macular hole formation, but no increase in graying of the retina, loss of retinal laminations, pigmentary infiltration, or alteration in the retinal vessels. CONCLUSION/CONCLUSIONS:Tissue contraction with retinal vascular displacement and contortion seem to be integral aspects of disease manifestation in MacTel2. The induced vascular changes may lead to secondary effects that increase morbidity in this disease.

PURPOSE/OBJECTIVE:To describe clinical findings in patients with acute exudative polymorphous vitelliform maculopathy (AEPVM). DESIGN/METHODS:Retrospective, observational, multicenter case series review. PARTICIPANTS/METHODS:Consecutive patients diagnosed with idiopathic AEPVM. METHODS:Review of clinical charts, multimodal imaging, electrophysiologic findings, and genetic findings in previously unpublished patients and review of the literature. MAIN OUTCOME MEASURES/METHODS:Clinical features of idiopathic AEPVM and differential diagnosis. RESULTS:Eighteen patients (age range, 21-74 years) with typical features of AEPVM, including initial localized, serous detachments followed by the development of characteristic yellow-white deposits in the vitelliform space. Over time, this hyperautofluorescent material gravitated within the larger lesions, resulting in typical curvilinear deposits characteristic of later stages. Symptoms and clinical findings lasted from weeks to several years. Some patients showed previously undescribed features such as fluorescein-negative intraretinal cystic changes, choroidal neovascularization, serous retinal elevations mimicking retinal folds, increased choroidal thickness, lack of rapid visual recovery, and recurrence years after complete resolution of initial manifestations. CONCLUSIONS:Acute exudative polymorphous vitelliform maculopathy can present with a more variable natural course than previously described. Paraneoplastic retinopathy and autosomal recessive bestrophinopathy closely resemble AEPVM, necessitating medical and hereditary evaluation to exclude these clinical possibilities. This series of patients with AEPVM expands the clinical spectrum of the disorder, including demographics, clinical manifestations, imaging features, natural course, and visual prognosis.

PURPOSE: To define the range and life cycles of cuticular drusen phenotypes using multimodal imaging and to review the histologic characteristics of cuticular drusen. DESIGN: Retrospective, observational cohort study and experimental laboratory study. PARTICIPANTS: Two hundred forty eyes of 120 clinic patients with a cuticular drusen phenotype and 4 human donor eyes with cuticular drusen (n = 2), soft drusen (n = 1), and hard drusen (n = 1). METHODS: We performed a retrospective review of clinical and multimodal imaging data of patients with a cuticular drusen phenotype. Patients had undergone imaging with various combinations of color photography, fluorescein angiography, indocyanine green angiography, near-infrared reflectance, fundus autofluorescence, high-resolution OCT, and ultrawide-field imaging. Human donor eyes underwent processing for high-resolution light and electron microscopy. MAIN OUTCOME MEASURES: Appearance of cuticular drusen in multimodal imaging and the topography of a cuticular drusen distribution; age-dependent variations in cuticular drusen phenotypes, including the occurrence of retinal pigment epithelium (RPE) abnormalities, choroidal neovascularization, acquired vitelliform lesions (AVLs), and geographic atrophy (GA); and ultrastructural and staining characteristics of druse subtypes. RESULTS: The mean age of patients at the first visit was 57.9+/-13.4 years. Drusen and RPE changes were seen in the peripheral retina, anterior to the vortex veins, in 21.8% of eyes. Of eyes with more than 5 years of follow-up, cuticular drusen disappeared from view in 58.3% of eyes, drusen coalescence was seen in 70.8% of eyes, and new RPE pigmentary changes developed in 56.2% of eyes. Retinal pigment epithelium abnormalities, AVLs, neovascularization, and GA occurred at a frequency of 47.5%, 24.2%, 12.5%, and 25%, respectively, and were significantly more common in patients older than 60 years of age (all P < 0.015). Occurrence of GA and neovascularization were important determinants of final visual acuity in eyes with the cuticular drusen phenotype (both P < 0.015). Small cuticular drusen typically demonstrated a homogenous ultrastructural appearance similar to hard drusen, whereas fragmentation of the central and basal contents was seen frequently in larger cuticular drusen. CONCLUSIONS: Although the ultrastructural characteristics of cuticular drusen appear more similar to those of hard drusen, their lifecycle and macular complications are more comparable with those of soft drusen. Cuticular drusen phenotype may confer a unique risk for the development of GA and neovascularization.

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:The authors describe a woman diagnosed with hemophagocytic lymphohistiocytosis and found to have retinal examination findings consistent with Purtscher retinopathy. METHODS:A 52-year-old woman underwent multimodal imaging, including color fundus photography and spectral-domain optical coherence tomography, to confirm the diagnosis. RESULTS:The ophthalmic examination and imaging confirmed the findings of Purtscher retinopathy with significant inner retinal thickening on spectral-domain optical coherence tomography. Throughout a hospital course complicated by multi-organ failure, she continued to have profoundly limited visual acuity, likely resulting from inner retinal ischemia affecting the posterior pole of both eyes. CONCLUSION/CONCLUSIONS:The authors describe a patient with hemophagocytic lymphohistiocytosis, a disease characterized by disruption of normal natural killer cell activity with subsequent uncontrolled cytokine release, who presented with Purtscher retinopathy confirmed with spectral-domain optical coherence tomography.

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/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.