Faculty Bibliography

PURPOSE: The purpose of this study was to evaluate anecortave acetate treatment of retinal angiomatous proliferation (RAP), a neovascular form of age-related macular degeneration, with specific regard to inhibition of neovascularization and maintenance of vision. METHODS: Thirty-four patients with RAP with any stage of neovascularization were randomized 1:1:1 for treatment with three different quantities (30 mg, 15 mg, 3 mg) of anecortave acetate sterile suspension for juxtascleral administration. Best-corrected visual acuity (Early Treatment Diabetic Retinopathy Study chart), intraocular pressure measurement, biomicroscopy, funduscopy, digital fluorescein, and indocyanine green angiography were recorded at baseline and at 3 months. A 6-month retreatment interval was established for this study with a follow-up of 12 months. In selected patients optical coherence tomography was performed. The outcomes were mean changes in visual acuity and lesion size at 1 year. RESULTS: The detachment of the neurosensory retina and retinal pigment epithelium improved in all eyes, but all neovascular lesions increased in size. Vision loss occurred in the majority of study eyes (22 out of 34 eyes, 64.7%) independent of the concentration administered. CONCLUSION: The results suggest that a posterior juxtascleral injection of anecortave acetate reduces capillary permeability in patients with RAP. However, in spite of improvement of the exudation there is a progression of neovascularization and a significant loss of vision in all these patients. Like other monotherapeutic methods used to treat this variant of neovascular age-related macular degeneration, anecortave acetate alone does not appear to benefit these patients. Future studies should investigate a combination form of therapy

A 42-year-old man with a history of human immunodeficiency virus and previous immune reconstitution after initiating a highly active antiretroviral therapy regimen was referred to rule out cytomegalo-virus retinitis. He had an unusual juxtapapillary collection of white superficial material and multiple preretinal collections resting on a peripheral ground glass opacification of the retina. These lesions quickly resolved after initiating treatment for syphilitic retinitis. The patient appeared to have developed an exaggerated response to the ocular syphilitic infection that may be related to the augmentation of his impaired immune system by highly active antiretroviral therapy

PURPOSE: To investigate and integrate the photographic, angiographic, and tomographic findings from a group of patients with various stages of vitelliform macular dystrophy type 2 (VMD2; also known as Best's disease) and use this information to propose mechanisms of disease pathogenesis. DESIGN: Retrospective observational case series. PARTICIPANTS: Nine consecutive patients seen in a private practice referral setting by the authors. METHODS: Patients with VMD2 were imaged with conventional fundus and autofluorescence photography, fluorescein angiography, fundus photography, and optical coherence tomography (OCT). MAIN OUTCOME MEASURES: The integrated ocular imaging findings. RESULTS: Early stage lesions were smaller and had accumulation of yellowish material in the central macula. This material was highly autofluorescent and appeared to be located on the outer retinal surface by OCT. Later stages were characterized by larger lesions with central clearing of the yellowish material and deposition of autofluorescent subretinal material at the outer borders of the lesion. Both early and late lesions had a subretinal fluid component with no reflectivity as detected by OCT. Fluorescein angiography showed transmission defects with a suggestion of late leakage, much like that seen in chronic central serous chorioretinopathy (CSC). CONCLUSIONS: Similar to that seen in CSC, patients with VMD2 have an accumulation of material on the outer retina, which may represent shed photoreceptor outer segments in association with subretinal fluid

PURPOSE: To evaluate the autofluorescence findings of patients with pseudoxanthoma elasticum, a disease resulting from a defect in a reputed transport protein encoded by the gene adenosine triphosphate-binding cassette subtype C number 6. DESIGN: Observational case series. METHODS: Color, red-free monochromatic, and autofluorescence photography and fluorescein angiography of patients with pseudoxanthoma elasticum seen in a referral practice were evaluated. MAIN OUTCOME MEASURES: Cataloging of the abnormalities as detected by autofluorescence photography. RESULTS: The 8 subjects ranged in age from 26 to 60 years (mean, 55+/-12), and their best-corrected visual acuity ranged from 20/20 to 5/400 (mean, 20/50). Of the 16 eyes of the 8 patients, all had abnormalities typical of pseudoxanthoma elasticum, including angioid streaks in 14, peau d'orange in 4, and choroidal neovascularization in 11. Angioid streaks appeared as hypoautofluorescent fissures, sometimes showing expansion of the hypoautofluorescence suggestive of retinal pigment epithelium (RPE) absence or atrophy. Peau d'orange had a stippled appearance of autofluorescence, and drusen of the optic nerve appeared as hyperautofluorescent bodies. In addition to the expansion of RPE atrophy around angioid streaks, 3 additional configurations of RPE atrophy were recognized as RPE rips in 6 eyes, multilobular areas of atrophy in 9 eyes, and broad areas of poorly demarcated atrophy in 5 eyes. Some eyes had more than one manifestation of RPE atrophy, but the latter 3 types of atrophy occurred in eyes with, but not necessary contiguous to, concurrent choroidal neovascularization. CONCLUSIONS: Autofluorescence photography demonstrated that patients with pseudoxanthoma elasticum have more widespread areas of RPE disturbance, particularly atrophy, than what is detectable by other means of ocular imaging, which suggests that the RPE disturbance may play a role in the pathogenesis of visual loss in patients with pseudoxanthoma elasticum

PURPOSE: To report the peripheral abnormalities seen only with indocyanine green angiography in patients with vitelliform macular dystrophy (Best disease, caused by a mutation in the bestrophin gene). DESIGN: Observational case report series. METHODS: Eight eyes of four patients, two with only a central macular lesion and two with multifocal lesions, were studied. Results of indocyanine green angiography were compared with findings from ophthalmoscopy and fluorescein angiography. RESULTS: Throughout the fundus periphery, indocyanine green angiography demonstrated a number of hyperfluorescent spots in all eight eyes. The spots were observed in the midperiphery and the periphery in areas with no abnormality visible by ophthalmoscopy or fluorescein angiography. CONCLUSIONS: Although Best disease generally causes lesions visible in the posterior pole, the extensive distribution of the hyperfluorescent spots is consistent with the wide-ranging abnormalities of the retinal pigment epithelium, Bruch membrane, and the choroid as seen histopathologically

PURPOSE: To describe the short-term anatomical and visual acuity responses after intravitreal injection of bevacizumab (Avastin, Genentech) in patients with choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD). METHODS: We conducted a retrospective study of patients with CNV secondary to AMD who were treated with intravitreal injection of bevacizumab (1.25 mg) during a 3-month period. Patients underwent best-corrected Snellen visual acuity testing, optical coherence tomography, and ophthalmoscopic examination at baseline and follow-up visits. RESULTS: There were 266 consecutive eyes of 266 patients who received injections, and follow-up information was available for 251 (94.4%). The mean age of the patients was 80.3 years, the mean baseline visual acuity was 20/184, and 175 (69.7%) had inadequate response to alternate methods of treatment. At the 1-month follow-up (data available for 244 patients), the mean visual acuity was 20/137 (P < 0.001 as compared with baseline), and 74 (30.3%) of patients had improvement in visual acuity as defined by a halving of the visual angle. At the 2-month follow-up (data available for 222 patients), the mean visual acuity was 20/122 (P < 0.001), and 78 (31.1%) of patients had visual improvement. At the 3-month follow-up (data available for 141 patients), the mean visual acuity was 20/109 (P < 0.001), and 54 (38.3%) of patients had visual acuity improvement. The mean central macular thickness at baseline was 340 mum and decreased to a mean of 247 microm at month 1 (P < 0.001) and 213 microm at month 3 (P < 0.001). At 1 month, two patients had mild vitritis, as did one patient at 2 months, who had a history of recurrent uveitis. No endophthalmitis, increased intraocular pressure, retinal tear, or retinal detachment occurred. The risk for thromboembolic disorders did not seem to be different than reported previously in studies concerning macular degeneration. CONCLUSION: There were no apparent short-term safety concerns for intravitreal bevacizumab injection for CNV. Treated eyes had a significant decrease in macular thickness and improvement in visual acuity. The follow-up was too short to make any specific treatment recommendations, but the favorable short-term results suggest further study is needed

OBJECTIVES: To study the clinical and angiographic features of lesions in a case series of multiple evanescent white dot syndrome (MEWDS), to describe a newly recognized clinical variation of the disorder, and to gain insight into its pathophysiological nature. METHODS: Five patients with MEWDS (selected based on angiographic manifestations of the disorder) were examined using slitlamp biomicroscopy and studied using fluorescein angiography and indocyanine green angiography. RESULTS: All 5 patients exhibited the newly recognized angiographic features termed dots and spots, which varied in size and location in the fundus. Small dots were in the inner retina or at the level of the retinal pigment epithelium, and larger spots were more external in the subpigment epithelial area. All patients exhibited other characteristics typical of MEWDS, including field loss and foveal granularity. CONCLUSIONS: In this case series of MEWDS, a clinical variant consisting of dual-layered lesions with specific features on clinical examination, fluorescein angiography, and indocyanine green angiography was identified. On late indocyanine green angiography, these lesions produced highly specific findings of small hypofluorescent lesions overlying larger hypofluorescent lesions. Based on the angiographic findings, it seems as if MEWDS is a chorioretinopathy with varying degrees of retinal and choroidal involvement