Faculty Bibliography

PURPOSE: To investigate the spectral-domain optical coherence tomography findings in four cases of acute macular neuroretinopathy and identify features that may differentiate acute macular neuroretinopathy from similar conditions in the acute zonal occult outer retinopathy complex. METHODS: Patients with acute macular neuroretinopathy underwent complete periodic examinations, including ophthalmoscopy, color photography and autofluorescence photography, visual field testing, and spectral-domain optical coherence tomography. Abnormalities as seen by multimodal imaging were documented and evaluated during follow-up. RESULTS: The average age of the 4 patients was 32 years (range, 28-34 years), and 2 had bilateral involvement. The follow-up ranged from 1 month to 5 months. In each patient, dark lobular areas seen by ophthalmoscopy corresponded to the measured visual field defect and what appeared to be a regional loss of outer segments of the photoreceptors. The outer segment defect resolved in two patients and improved in the other two during follow-up. All patients showed a colocalizing regional thinning of the outer nuclear layer, which did not change during the follow-up period. There were no associated abnormalities in the fundus appearance. CONCLUSION: Acute macular neuroretinopathy causes reddish brown patches that are because of outer retinal damage as documented by spectral-domain optical coherence tomography. Unlike other entities in the acute zonal occult outer retinopathy complex, acute macular neuroretinopathy has no other synchronic ophthalmoscopically visible fundus abnormalities. These lesions may resolve over time, with restoration of the outermost retinal architecture, but persistent thinning of the overlying outer nuclear layer remained.

PURPOSE: To evaluate the subfoveal choroidal thickness in Vogt-Koyanagi-Harada (VKH) disease using enhanced depth imaging optical coherence tomography. METHODS: Retrospective observational study. Subfoveal choroidal thickness was measured using enhanced depth imaging optical coherence tomography, in which the optical coherence tomography instrument was placed close enough to the eye to obtain an inverted image, which was averaged for 100 scans. All patients were diagnosed as having the ocular findings of VKH disease with or without extraocular disorders. The patients were followed during their initial treatment with corticosteroids. RESULTS: All 8 patients (16 eyes) with acute phase VKH disease presented with thickening of the choroid. The serous retinal detachment disappeared in 1 month after corticosteroid treatment. The mean choroidal thickness in 16 eyes decreased from 805 +/- 173 mum at the first visit to 524 +/- 151 mum at 3 days (P < 0.001) and 341 +/- 70 mum by 2 weeks (P < 0.001). CONCLUSION: Patients with active VKH disease have markedly thickened choroids, possibly related not only to inflammatory infiltration but also to increased exudation. Both the choroidal thickness and the exudative retinal detachment decreased quickly with corticosteroid treatment. Enhanced depth imaging optical coherence tomography can be used to evaluate the choroidal involvement in VKH disease in the acute stages and may prove useful in the diagnosis and management of this disease noninvasively.

PURPOSE: To investigate spectral domain optical coherence tomography and autofluorescence findings in eyes with pseudoxanthoma elasticum. METHODS: A retrospective analysis of visual acuity, spectral domain optical coherence tomography, and autofluorescence findings of consecutive patients with pseudoxanthoma elasticum was performed. The spectral domain optical coherence tomography was evaluated for retinal architecture, subretinal accumulations, and photoreceptor layer thickness. Autofluorescence and near-infrared reflectance images were reviewed for correlative findings. RESULTS: There were 21 patients (42 eyes) with pseudoxanthoma elasticum with a mean age of 56.1 +/- 12.4 years. Subretinal fluid was found in 14 eyes, 7 of which had no signs of choroidal neovascularization. In six of the seven eyes with a history of choroidal neovascularization controlled with antivascular endothelial growth factor injections, there were areas of subretinal fluid that were not contiguous with the choroidal neovascularization and did not seem responsive to antivascular endothelial growth factor injections. Two types of formed material were observed in the subretinal space and outer retina. The first was hypoautofluorescent deposits above the retinal pigment epithelium resembling subretinal drusenoid deposits (reticular pseudodrusen). The second was yellow to brown hyperautofluorescent aggregates in the subretinal space and outer retina similar to those seen in pattern dystrophies and was found in 19 eyes. There was an apparent association between the presence of subretinal fluid and pattern dystrophy-like findings. CONCLUSION: Subretinal fluid in patients with pseudoxanthoma elasticum is not always indicative of active leakage from underlying choroidal neovascularization and can be resistant to antivascular endothelial growth factor injections. This fluid is associated with pattern dystrophy-like findings and may indicate abnormal retinal pigment epithelial function.

PURPOSE: To report a recently observed association of macular vitelliform detachment and subretinal drusenoid deposits (reticular pseudodrusen). METHODS: Clinical and multimodal imaging data of patients with acquired vitelliform lesions in association with subretinal drusenoid deposits were reviewed. Acquired vitelliform lesions were defined as subretinal accumulations of yellow material that developed in adulthood. Subretinal drusenoid deposits were diagnosed as being present if there were drusen-like accumulations that colocalized with aggregates of subretinal material as seen by multimodal imaging including spectral-domain optical coherence tomography, autofluorescence, and near-infrared imaging. RESULTS: Seven eyes of 6 patients with a mean age of 85 years, all of whom were white, were found to have vitelliform material in association with subretinal drusenoid deposits. The median visual acuity was 20/30. The vitelliform material was hyperautofluorescent and was in all eyes located in the subretinal space between the inner segment/outer segment junction and the retinal pigment epithelium. This material had the same color, autofluorescence, and optical coherence tomographic characteristics as the vitelliform material seen in association with cuticular drusen. CONCLUSION: Acquired vitelliform lesions can occur in association with subretinal drusenoid deposits. Subretinal drusenoid deposits might be mistaken for cuticular drusen because of their similar appearance in color fundus photography but can be easily distinguished with multimodal imaging because they lie above the retinal pigment epithelium. Subretinal drusenoid deposits may reflect abnormalities in the function of the retinal pigment epithelium and their presence may interfere with photoreceptor outer segment turnover, leading to an accumulation of vitelliform material.

PURPOSE: To examine the posterior anatomic structure of eyes with dome-shaped macula using enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT). DESIGN: Retrospective observational case series. METHODS: Patients with dome-shaped macula, a condition defined as convex elevation of the macula as compared with the surrounding staphylomatous region in a highly myopic eye, were identified through routine examinations using optical coherence tomography (OCT). EDI-OCT was used to examine their posterior anatomic changes. The scleral thickness was measured from the outer border of the choroid to the outer scleral border under the fovea and 3000 mum temporal to the fovea. RESULTS: The mean age of the 15 patients (23 eyes) was 59.3 (+/- 12.2) years, and the mean refractive error was -13.6 (+/- 5.0) diopters. The best-corrected visual acuity ranged from 20/15 to 20/800 (median: 20/30). Eight patients (53%) had dome-shaped macula bilaterally. The mean subfoveal scleral thickness in 23 eyes with dome-shaped macula was 570 (+/- 221) mum, and that in 25 eyes of 15 myopic patients with staphyloma but without dome-shaped macula was 281 (+/- 85) mum (P < .001) even though both groups had similar myopic refractive error. The scleral thickness 3000 mum temporal to the fovea was not different in the 2 groups. CONCLUSIONS: Dome-shaped macula is the result of a relative localized thickness variation of the sclera under the macula in highly myopic patients, and it cannot be categorized into any of the known types of staphyloma. This finding suggests the ocular expansion in myopia may be more complex than previously thought.

PURPOSE: To correlate clinical observations with multimodal imaging analysis in acquired vitelliform lesions (AVLs) and to elucidate their nature, pathogenesis, and natural course. METHODS: Clinical examination, color fundus photography, fluorescein angiography, near-infrared reflectance, fundus autofluorescence, and spectral-domain optical coherence tomography (SD-OCT) data were retrospectively reviewed for a consecutive series of 90 eyes of 67 patients with an AVL secondary to a variety of diagnoses. RESULTS: In all 90 eyes with AVLs, SD-OCT helped localize the clinically apparent yellowish material to the subretinal space above the retinal pigment epithelial (RPE) band. Only 19 eyes (21.1%) had SD-OCT evidence of subretinal fluid. All eyes exhibited abnormal hyperautofluorescence corresponding to the material seen clinically. In 18 of 72 eyes (25.0%) imaged simultaneously with SD-OCT and near-infrared reflectance imaging, near-infrared hyperreflectivity corresponding to presumed collections of pigment-laden macrophages and RPE cells was observed. Resolution of some AVLs appeared to coincide with progressive thinning of the outer nuclear layer, indicating a gradual loss of photoreceptors. Visual acuity at baseline was best predicted by the subfoveal integrity of the external limiting membrane (P = 0.001) and the inner segment/outer segment junction (P = 0.0002) on SD-OCT. Fluorescein angiography revealed a pattern often mimicking poorly defined (Type 1) choroidal neovascularization. CONCLUSION: Acquired vitelliform lesions occur in a variety of different clinical entities that share common features with multimodal imaging analyses. We propose that both dysfunctional RPE and loss of apposition between the photoreceptor tips and the RPE can interfere with the phagocytosis of shed outer segments. Both this material and pigment-laden macrophages and RPE cells appear to contribute to the yellowish appearance of AVLs. Ongoing photoreceptor loss may in some cases be associated with the spontaneous resolution of an AVL

PURPOSE: To characterize the known appearance of cuticular drusen, subretinal drusenoid deposits (reticular pseudodrusen), and soft drusen as revealed by multimodal fundus imaging and to create an explanatory model that accounts for these observations. METHODS: Reported color, fluorescein angiographic, autofluorescence, and spectral domain optical coherence tomography images of patients with cuticular drusen, soft drusen, and subretinal drusenoid deposits were reviewed, as were actual images from affected eyes. Representative histological sections were examined. The geometry, location, and imaging characteristics of these lesions were evaluated. A hypothesis based on the Beer-Lambert law of light absorption was generated to fit these observations. RESULTS: Cuticular drusen appear as numerous, uniform, round, yellow-white punctate accumulations under the retinal pigment epithelium (RPE). Soft drusen are larger, yellow-white dome-shaped mounds of deposit under the RPE. Subretinal drusenoid deposits are polymorphous light-gray interconnected accumulations above the RPE. Based on the model, both cuticular and soft drusen appear yellow because of the removal of shorter wavelength light by a double pass through the RPE. Subretinal drusenoid deposits, which are located on the RPE, are not subjected to short-wavelength attenuation and therefore are more prominent when viewed with blue light. The location and morphology of extracellular material in relationship to the RPE, and associated changes to RPE morphology and pigmentation, appeared to be the primary determinants of druse appearance in different imaging modalities. CONCLUSION: Although cuticular drusen, subretinal drusenoid deposits, and soft drusen are composed of common components, they are distinguishable by multimodal imaging because of differences in location, morphology, and optical filtering effects by drusenoid material and the RPE.

PURPOSE: To determine the prevalence and significance of subretinal drusenoid deposits (reticular pseudodrusen) among patients with age-related macular degeneration (AMD). DESIGN: A prospective study with a nested case-control study of consecutive patients with AMD seen in a referral retinal practice. PARTICIPANTS: There were 153 patients with AMD, 131 of whom had > or =1 eye with late AMD, which was defined as either central geographic atrophy or choroidal neovascularization. The control group consisted of 101 patients who did not have AMD as their primary diagnosis, central serous chorioretinopathy, high myopia, retinal detachment, or laser treatment in the macular area. METHODS: The presence of subretinal drusenoid deposits was determined by 2 methods, using the blue channel of color fundus photograph and the spectral domain optical coherence tomography (SD-OCT) sections. Soft drusen were determined from color fundus photographs and confirmed by SD-OCT. MAIN OUTCOME MEASURES: Prevalence of ocular risk factors and subretinal drusenoid deposits in eyes with AMD and their association with late AMD. RESULTS: There were 153 patients who had any form of AMD, with a mean age of 80.3 years. Subretinal drusenoid deposits were diagnosed in the case group in 13 (8.7%) of right and 18 (12.0%) of left eyes using the blue channel of the color photograph and in 58 (38.4%) of right and 54 (35.8%) of left eyes using SD-OCT. Soft drusen and subretinal drusenoid deposits detected by SD-OCT were found to be independently correlated with late AMD (soft drusen odds ratio = 16.66 [P<0.001]; subretinal drusenoid deposits as detected by OCT odds ratio = 2.64 [P = 0.034]). In the control group, subretinal drusenoid deposits were diagnosed in 6 (6.5%) of right and 6 (6.3%) of left eyes using SD-OCT. CONCLUSIONS: Both soft drusen and subretinal drusenoid deposits occur in patients with AMD and both are significantly associated with late AMD. These findings suggest that detection and classification of drusen and consequently assignment of risk should be based on a methodology that includes SD-OCT.

PURPOSE: To evaluate the subfoveal choroidal thickness after treatment of central serous chorioretinopathy (CSC) visualized by enhanced depth imaging spectral-domain optical coherence tomography (EDI OCT) and indocyanine green angiography (ICGA). DESIGN: Retrospective, comparative series. PARTICIPANTS: Twenty patients (20 eyes). METHODS: The subfoveal choroidal thickness and height of the serous retinal detachment before and after treatment was measured using EDI OCT. Areas of choroidal vascular hyperpermeability were visualized with ICGA. Eyes with classic CSC were treated with laser photocoagulation (LP), whereas eyes with chronic CSC, which are not amenable to LP, were treated with half-dose verteporfin photodynamic therapy (PDT). MAIN OUTCOME MEASURES: Change in choroidal thickness and height of the serous retinal detachment after treatment. RESULTS: There were 12 eyes in the LP group and 8 eyes in the PDT group. The serous subretinal fluid resolved in both groups after treatment. In the LP group, the mean choroidal thickness was 345+/-127 microm at baseline and 340+/-124 microm at 4 weeks, a difference that was not significant (P = 0.2). The mean choroidal thickness in the PDT group increased significantly from 389+/-106 microm at baseline to 462+/-124 microm (P = 0.008) by 2 days after treatment, and then reduced rapidly to 360+/-100 microm (P = 0.001) at 1 week and 330+/-103 microm (P<0.001) after 4 weeks as compared with baseline. Indocyanine green angiography showed decreased hyperpermeability in the PDT group after treatment. CONCLUSIONS: The subretinal fluid resolved in both disease groups; however, the choroidal thickness and hyperpermeability seen during ICGA was reduced after PDT. These findings suggest that PDT reduces the choroidal vascular hyperpermeability seen in CSC and may work by a different mechanism than LP.