Faculty Bibliography

PURPOSE: To report the short-term anatomic and visual acuity response after intravitreal injection of bevacizumab (Avastin, Genentech) in patients with proliferative diabetic retinopathy complicated by vitreous hemorrhage. METHODS: Two patients with vitreous hemorrhage due to proliferative diabetic retinopathy were treated with at least one intravitreal injection of bevacizumab 1.25 mg in 0.05 mL. The patients underwent Snellen visual acuity testing, ophthalmoscopic examination, and fluorescein angiography at baseline and follow-up visits. RESULTS: Both patients had proliferative diabetic retinopathy with vitreous hemorrhage extensive enough to preclude panretinal photocoagulation. Following intravitreal injection of bevacizumab both patients experienced improvement in visual acuity starting within the first week. At 1 month of follow-up one patient had 2 lines of improvement in visual acuity and the other 5 lines. Each patient had regression of retinal neovascularization at 1 month of follow-up. Repeat injection was given to one patient at the 1-month follow-up because of slight leakage from neovascularization on the nerve, and to the other patient at 3 months because the retinal neovascularization showed early signs of reperfusion. The vitreous hemorrhage in each patient showed partial resolution at 1 week and nearly complete regression at 1 month. No adverse events were observed in either patient. CONCLUSIONS: Initial treatment results of patients with vitreous hemorrhage and proliferative diabetic retinopathy did not reveal any short-term safety concerns. Intravitreal bevacizumab resulted in marked regression of neovascularization and rapid resolution of vitreous hemorrhage. The favorable short-term results suggest further study is needed in a larger group of patients

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 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