Faculty Bibliography

OBJECTIVE: To characterize an unusual clinical entity resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and serpiginous choroiditis but with an atypical clinical course. PATIENTS: We describe 6 patients, aged 17 through 51 years, exhibiting this unusual entity who were seen at 6 different centers from 1984 to 1997. RESULTS: The acute retinal lesions in this series were similar to those of APMPPE or serpiginous choroiditis, both clinically and on fluorescein and indocyanine green angiography. However, the clinical course, number of lesions, and location of these lesions were atypical. These patients had evidence of numerous posterior and peripheral retinal lesions predating or occurring simultaneously with macular involvement. Older, healing pigmented lesions were often accompanied by the appearance of new active white placoid lesions. Additionally, these cases all demonstrated prolonged periods of activity resulting in the appearance of more than 50 and sometimes hundreds of lesions scattered throughout the fundus. Growth of subacute lesions and the appearance of new lesions continued for 5 to 24 months after initial examination, and relapses were common. CONCLUSIONS: This entity has clinical features similar to APMPPE and serpiginous choroiditis but has a prolonged progressive clinical course and widespread distribution of lesions. It may represent a variant of serpiginous choroiditis or may be a new entity. We call it relentless placoid chorioretinitis. Arch Ophthalmol. 2000;118:931-938

PURPOSE: To describe the clinical findings and course in four patients with acute annular outer retinopathy. METHODS: Four patients were evaluated during the course of acute annular outer retinopathy, and the historical and clinical findings were retrospectively collected. RESULTS: Four healthy patients developed the acute onset of visual field loss associated with a localized, white annular outer retinopathy. All patients were caucasian; two were women, aged 29 and 32 years, and two were men, aged 71 and 79 years. The mean follow-up was 3.9 years (range, 1 to 6 years). On presentation, four eyes had an irregular, incomplete, peripapillary, annular band of gray-white, deep retinal opacification with visual CONCLUSIONS: These four cases of acute annular outer retinopathy expand our knowledge of this disorder. Acute annular outer retinopathy may be a distinct entity or it may represent a variant of acute zonal occult outer retinopathy retinopathy. As more cases are recognized, the characteristic features of the disease spectrum, the etiology, and treatment options may be better elucidated

PURPOSE: To report a case of primary systemic nonfamilial amyloidosis studied by fluorescein angiography and indocyanine green angiography. METHODS: Case report. A 59-year-old woman with primary systemic nonfamilial amyloidosis presented bilateral diffuse deep hemorrhages and pigmentary mottling at the posterior pole. RESULTS: On fluorescein angiography bilateral diffuse areas of hypofluorescence were present. Indocyanine green angiography showed large hypofluorescent areas with hypofluorescent lines in the midperiphery and hyperfluorescent streaks in the peripapillary area. CONCLUSIONS: In this case of primary systemic nonfamilial amyloidosis, diffuse bilateral chorioretinal abnormalities included hemorrhages and pigmentary mottling at the posterior pole, with hypofluorescent areas on fluorescein angiography and indocyanine green angiography, as well as hypofluorescent lines in the midperiphery

PURPOSE: To characterize a newly discovered choroidal vascular abnormality in patients who have received radiation therapy for subfoveal choroidal neovascularization (CNV) secondary to age-related macular degeneration. DESIGN: Two-center cross-sectional study. PARTICIPANTS: In the United States, there were 95 patients who were treated with 10 or 12 Gy of external beam photons. In Belgium, 98 patients were treated with 20 Gy. These patients were examined retrospectively for the presence of a specific CNV abnormality. RESULTS: During the follow-up period, an unusual vascular growth pattern was identified in 12 patients (12.6%) of those treated in the United States and in 7 (7.1%) of those treated in Belgium. These patients developed round or oval vascular blebs along the outer border of their neovascular lesions. These blebs profusely leaked fluorescein dye and could be imaged best by indocyanine green angiography. Patients with these blebs appeared to have a marked propensity for loss of visual acuity. CONCLUSION: An unusual pattern of new vessel growth occurred in 19 of the 193 patients with CNV treated with radiation. This new entity, termed radiation-associated choroidal neovasculopathy, is a recognizable disorder that appears to have a particularly poor prognosis

OBJECTIVE: To determine the nature and frequency of polypoidal choroidal vasculopathy (PCV) in a series of patients suspected of having neovascularized age-related macular degeneration (AMD). METHODS: A prospective analysis of 167 consecutive, newly diagnosed patients aged 55 years or older with presumed neovascularized AMD was performed. All patients were examined with fundus biomicroscopy as well as fluorescein and indocyanine green angiography. RESULTS: Choroidal neovascularization secondary to AMD was diagnosed in 154 (92.2%) of 167 patients; 13 (7.8%) patients had PCV. The patients affected by PCV were younger than those with AMD (P = .01). Peripapillary choroidal neovascularization was seen in 3 (1.9%) of 154 patients with AMD and 3 (23.1%) of 13 patients with PCV (P = .006). Significant drusen were present in 63 (70%) of 90 fellow eyes with unilateral AMD compared with only 1 (16.7%) of 6 eyes with PCV (P = .02). Only 5 patients with AMD (3.2%) were nonwhite compared with 3 patients with PCV (23.1%) (P = .02). CONCLUSIONS: A measurable number of elderly patients with findings suggestive of neovascularized AMD and serosanguineous macular manifestations will instead have PCV. Polypoidal choroidal vasculopathy can occur in any sex or race, but is more commonly seen in the peripapillary area, without associated drusen, and in nonwhite patients. It is important to differentiate AMD from PCV because there are significant differences in the demographic risk profile, natural course, visual prognosis, and management of these patients

PURPOSE: To determine systemic factors associated with central serous chorioretinopathy. METHODS: In a retrospective study, 230 consecutive patients with central serous chorioretinopathy examined in a referral setting were compared with a historical gender-matched and age-matched control group of 230 patients with ocular findings who were examined in the same referral setting. RESULTS: The median age of the patients was 49.8 years, and of the control subjects, 50.0 years. The male-female ratio for both groups was 2.7:1. Patients with central serous chorioretinopathy were more likely to use psychopharmacologic medications (odds ratio = 2.6; 95% confidence interval = 1.30 to 5.19; P = .0049) and corticosteroids (odds ratio = 3.17; 95% confidence interval = 1.30 to 7.70; P = .0067) and were more likely to have hypertension (odds ratio = 2.25; 95% confidence interval = 1.39 to 3.63; P = .0008) than were the control subjects. CONCLUSIONS: This study identified psychopharmacologic medication use, corticosteroid use, and hypertension as factors associated with central serous chorioretinopathy. These findings reinforce the concept that stress and adaptations to stress play a role in this disorder. The findings of possible associations between central serous chorioretinopathy and both hypertension and corticosteroid usage suggest that these modifiable factors may influence morbidity of central serous chorioretinopathy

OBJECTIVE: To examine the association between previous radiation exposure and idiopathic perifoveal telangiectasis (IPT). DESIGN: A multicentered, individually matched, case-control study design was used. PARTICIPANTS/CONTROLS: Sixty-five case subjects were matched with 175 control subjects. Individuals with unequivocal evidence of angiographically confirmed IPT were included as cases. Control subjects were matched for center, age, and gender. MAIN OUTCOME MEASURE: The main exposures of interest were a history of therapeutic head or neck irradiation and environmental radiation exposure. METHODS: A standardized questionnaire was administered to case and control subjects. Data were collected for the main exposures of interest as well as pertinent covariates. Conditional logistic regression was used to evaluate therapeutic and environmental radiation as risks for IPT. RESULTS: On univariate analysis, head or neck irradiation was associated with IPT (odds ratios [OR] = 4.15, 95% confidence interval [CI] = 1.30-13.24). While controlling for diabetes and family history of diabetes, IPT was found to be associated with both head or neck irradiation (OR = 4.06, 95% CI = 1.20-13.76) and with environmental irradiation (OR = 6.73, 95% CI = 1.06-42.74). CONCLUSIONS: This study presents a previously unreported association between prior radiation exposure and IPT