Faculty Bibliography

PURPOSE: To subclassify pseudodrusen based on their appearance in the multimodal imaging. DESIGN: Retrospective, observational series. METHODS: The color fundus photographs and infrared scanning laser ophthalmoscope (IR-SLO) images of patients with pseudodrusen were evaluated along with spectral domain optical coherence tomography (SD-OCT) by masked readers. Distinct types of pseudodrusen could be differentiated. RESULTS: There were 140 eyes of 93 patients with a mean age of 82.4 years. Multimodal imaging analysis showed 3 subtypes of pseudodrusen. One principal type was an orderly array of whitish discrete accumulations principally located in the perifovea, termed dot pseudodrusen. They appeared as hyporeflective spots, often with a target configuration, in IR-SLO images. The second type was interconnected bands of yellowish-white material forming a reticular pattern, called ribbon pseudodrusen, which were located in the perifovea. This subtype was faintly hyporeflective in IR-SLO imaging. Dot pseudodrusen were detected more commonly with IR-SLO imaging than color photography (P=. 014) and ribbon pseudodrusen were seen more frequently in color than in IR-SLO images (P<. 001). An uncommon third type of pseudodrusen, yellow-white globules primarily located peripheral to the perifoveal region, appeared hyperreflective in IR-SLO and were called peripheral pseudodrusen. All 3 types were seen as subretinal drusenoid deposits by SD-OCT. CONCLUSION: Pseudodrusen may be classified into at least 3 categories, each with optimal methods of detection and only one that formed a reticular pattern. These findings suggest pseudodrusen could contain differing constituents and therefore, may vary in conferred risk for progression to advanced age-related macular disease.

The authors report the in vivo visualization through multimodal imaging of silicone oil in the vitreous cavity, optic nerve, and retina of a patient 11 years after surgical removal of the main tamponade. Contact B-scan ultrasonography revealed countless small echogenic particles in the vitreous cavity and a hyperreflective structure within the optic nerve head. Swept-source optical coherence tomography showed multiple round hyporeflective spaces within the substance of the prelaminar optic nerve head. Adaptive optics imaging revealed numerous particles in the optic nerve head and within the retina in the same plane as the photoreceptors, with similar size and shape to the silicone oil droplets in the vitreous cavity. The authors hypothesize that in this case, the silicone oil droplets in the retina and optic nerve head may be associated with the patient's otherwise unexplained progressive visual loss.

PURPOSE: To describe previously unreported imaging features of choroidal lymphoma using enhanced depth imaging optical coherence tomography (OCT). METHODS: Enhanced depth imaging OCT was performed before and after the therapy. RESULTS: A 32-year-old white man with a 4-month history of blurred vision in the right eye was found to have a macular fold. There was no visible intraocular tumor. There were no signs of anterior segment inflammation, vascular abnormalities, or infiltrative disease. Visual acuity was 20/150 in the right eye and 20/20 in the left eye. Enhanced depth imaging OCT demonstrated a macular retinal fold and marked thickening of the choroid with striking choroidal surface undulation and folds imparting an appearance similar to a "sea storm" (seasick appearance). Deep choroidal structures could not be visualized, and the sclerochoroidal interface could not be identified. Overlying subretinal fluid and intraretinal fluid was noted. Ultrasonography demonstrated diffuse, relatively smooth thickening of the choroid (4.0-mm thickness) with minor extraocular hypoechoic area. Based on these findings, choroidal lymphoid proliferation was suspected, and fine-needle aspiration biopsy confirmed B-cell lymphoma. Results of systemic evaluation were unremarkable. After external beam radiotherapy with dose of 40 Gy, visual acuity returned promptly to 20/40 and the lymphoid infiltration resolved with flattening of the macular fold and resolution of subretinal and intraretinal fluids. The enhanced depth imaging OCT returned to a more normal appearance with the resolution of the retinal fold and reduction of the choroidal mass with retinal pigment epithelial-choroidal surface features to a "calm sea" appearance. CONCLUSION: Enhanced depth imaging OCT is a useful tool for subclinical monitoring of choroidal infiltration from lymphoma before and after therapy.

PURPOSE: To investigate the effect that panretinal photocoagulation to peripheral areas of retinal vascular nonperfusion has on the visual acuity and injection frequency of ranibizumab in eyes with previous central retinal vein occlusion. METHODS: Patients enrolled in a prospective study of ranibizumab for central retinal vein occlusion were imaged with wide-field angiography using the Optos P200 system. Laser photocoagulation was carried out and the extent of laser photocoagulation was evaluated with repeat wide-field angiography. Injection of ranibizumab was based on an as needed strategy throughout the study. The injection frequency in the 6 months before laser was compared with a 6-month period starting 2 months after the laser photocoagulation. The visual acuity was measured by Early Treatment Diabetic Retinopathy Study protocol refraction at both the end of the 6-month follow-up period and at the time of laser photocoagulation. RESULTS: There were 10 patients treated in this study with a mean number of 1,757 spots of laser photocoagulation in the peripheral retina. The injection frequency in the 6-month lead-in period was 3.4 and in the 6-month follow-up period was 3.1, a difference that was not significant (P = 0.26). The visual acuity at the time of laser photocoagulation was 54.2 letters (approximate Snellen equivalent of 20/80) and at the end of the observation period was 51.4 letters, a difference that was not significant (P = 0.33). CONCLUSION: In this small study, laser photocoagulation to peripheral areas of nonperfusion as visualized by wide-field angiography did not result in either decreased injection frequency or improved visual acuity in eyes with central retinal vein occlusion treated with ranibizumab.

PURPOSE: To analyze the outer retinal layers using spectral domain optical coherence tomography (SD-OCT) in patients with cone-rod dystrophy. METHODS: The diagnosis of cone-rod dystrophy was determined by primary cone involvement or concomitant loss of both cones and rods. Electroretinography showed implicit time shift at 30-Hz flicker response and prevalent decrease of photopic over scotopic responses. Using SD-OCT, the outer retina was retrospectively evaluated in 24 eyes of 12 patients with cone-rod dystrophy. From the innermost to the outermost, the four studied hyperreflective outer retinal bands were labeled Band 1, the external limiting membrane; Band 2, the ellipsoid zone; Band 3, the interdigitation zone between the cone outer segments and the apical processes of the retinal pigment epithelium; and Band 4, the retinal pigment epithelium complex. RESULTS: The mean age of study patients was 30 years, and the median visual acuity was 20/30. A ring maculopathy appearance involving the fovea area was observed in all study eyes. There was an absence of interdigitation zone in the entire length of SD-OCT scan, including the foveal area, in all 24 study eyes. Outside the foveal area, the external limiting membrane and ellipsoid zone were intact in all study eyes. The intensity of the ellipsoid zone was decreased in the entire length of SD-OCT scan in all study eyes. Within the foveal area, there was loss of the external limiting membrane and ellipsoid zone in 20 (83%) and 22 eyes (92%), respectively. The retinal pigment epithelium complex was identified in all study eyes. None of the study eyes revealed cystoid macular edema. CONCLUSION: SD-OCT scans demonstrated complete absence of the interdigitation zone in patients with cone-rod dystrophy. Consistent with the known histology of animal models of cone dystrophy, this finding may represent abnormal outer retinal morphology, including an absence of the outer segments themselves or a defective or absent interdigitation between the apical processes of the retinal pigment epithelium with the cone outer segments.

PURPOSE: To investigate the posterior segment in cases of clinically evident intraocular inflammation for punctate reflections consistent with that expected to arise from inflammatory cells. METHODS: Patients with ocular inflammatory diseases imaged with spectral-domain optical coherence tomography (SD-OCT) were retrospectively reviewed. RESULTS: There were 7 patients with mean age of 66.7 years, and the diagnosis was toxoplasmosis in 5 eyes, multiple evanescent white dot syndrome in 1 eye, and posttraumatic outer retinitis in 1 eye. At baseline, the SD-OCT showed vitreous cells as numerous punctate spots in the vitreous in all seven eyes. The SD-OCT also showed similar-sized hyperreflective dots in the retina in all seven eyes. During follow-up, reduced vitreous cellular infiltration was correlated with a decrease in the number of the punctate spots visible by SD-OCT. CONCLUSION: Eyes with intraocular inflammation had SD-OCT images of the vitreous containing punctate spots of a size consistent with that expected from inflammatory cells. Similarly sized punctate spots were seen within the retina in regions of retinitis. Additional characterization of the optical section should permit stereological estimations of actual cell counts per unit volume.

PURPOSE: To evaluate optic disc drusen, extracellular protein deposits known to contain numerous aggregates of mitochondria, using multimodal modalities featuring optical coherence tomography (OCT) and autofluorescence imaging. DESIGN: Retrospective observational case series. METHODS: Eyes with optic nerve drusen were examined with enhanced depth imaging (EDI)-OCT, swept source OCT, and fundus autofluorescence using a fundus camera. RESULTS: Twenty-six eyes of 15 patients with optic disc drusen were evaluated. EDI-OCT and swept source OCT showed multiple optic disc drusen at different levels; most were located immediately anterior to the lamina cribrosa. The drusen were ovoid regions of lower reflectivity that were bordered by hyperreflective material, and in 12 eyes (46.2%) there were internal hyperreflective foci. The mean diameter of the optic disc drusen as measured in OCT images was 686.8 (standard deviation +/- 395.2) mum. There was a significant negative correlation between the diameter of the optic disc drusen and the global retinal nerve fiber layer thickness (r = -0.61, P = .001). There was a significant negative correlation between proportion of the optic disc drusen area occupied by optic nerve drusen as detected by autofluorescence imaging and the global retinal nerve fiber layer thickness (r = -0.63, P = .001). CONCLUSIONS: Deeper-penetration OCT imaging demonstrated the internal characteristics of optic disc drusen and their relationship with the lamina cribrosa in vivo. This study also showed that both the larger the drusen and the more area of the optic canal occupied by drusen, the greater the associated retinal nerve fiber layer abnormalities.

PURPOSE: To examine histomorphometrically the macular region of highly myopic eyes. METHODS: On horizontal anterior-posterior histological sections, we examined the posterior pole of 138 human globes (axial length: 20-35 mm). In the parapapillary region, we differentiated between the beta zone (Bruch's membrane without RPE), gamma zone (parapapillary region without Bruch's membrane), and delta zone (elongated and thinned gamma zone). RESULTS: In 12 (8.7%) eyes, a macular Bruch's membrane defect (MBMD) was detected. The MBMD showed a complete lack of RPE and choriocapillaris, and an almost complete lack of photoreceptors. Presence of MBMD was associated with longer axial length (P < 0.001), longer gamma zone (P = 0.04) and delta zone (P < 0.001), thinner peripapillary scleral flange, and thinner sclera just outside of the optic nerve meninges (P < 0.001) and at the posterior pole (P < 0.001). An MBMD was found only in eyes with an axial length of 27 mm or longer. MBMD prevalence in highly myopic eyes was 12/39 or 30.8%. MBMD presence was not significantly related to length of beta zone (P = 0.09). In multivariate binary regression analysis, MBMD presence was significantly (P < 0.001) associated only with axial length. CONCLUSIONS: Highly myopic eyes (axial length >/=27mm) can show an MBMD associated with complete loss of RPE and choriocapillaris, and marked reduction of photoreceptors and large choroidal vessels. MBMD presence was strongly associated with axial length and indirectly with parapapillary gamma zone and delta zone. The myopia-associated secondary MBMDs may occur parallel to the myopia-associated widening of Bruch's membrane opening around the optic nerve head.