Faculty Bibliography

PURPOSE: To determine the nature and risk of neovascularization in the fellow eyes of patients with unilateral retinal angiomatous proliferation (RAP), a neovascular form of age-related macular degeneration (AMD). METHODS: A consecutive series of 52 patients diagnosed with unilateral RAP were studied retrospectively. Clinical biomicroscopic examination, fluorescein angiography, and indocyanine green angiography were used to evaluate all patients for the development of neovascular manifestations in the fellow eye. RESULTS: Neovascularization developed in the fellow eye in 52 patients over the follow-up period (range, 2-36 months). All patients developed neovascular manifestations of RAP in the fellow eye. Twenty-one patients (40%) developed a RAP lesion within 1 year; 29 (56%), within 2 years; and 52 (100%), within 3 years. At the time of diagnosis of neovascularization in the fellow eye, 8 patients (15%) had a stage I RAP lesion, 36 (70%) had a stage II RAP lesion, and 8 (15%) had a stage III RAP lesion. Other characteristic findings in these patients included the presence of preretinal, intraretinal, and subretinal hemorrhages in 49 patients (94%) and pigment epithelial detachments in 41 patients (79%). CONCLUSIONS: In patients diagnosed with unilateral RAP lesions, the form of neovascularization that develops in the fellow eye is virtually always RAP. The annual and accumulative risk of neovascularization in the fellow eye is higher in patients with RAP than in those with other forms of neovascular AMD. These new findings enhance our understanding of the clinical spectrum of RAP in terms of its natural course and visual prognosis and may possibly offer useful information to establish future treatment options

PURPOSE: To report a peculiar curvilinear pigmentary lesion in the peripheral fundus in a rod-cone dystrophy. METHODS: Observational case report. Fundus examination of a 57-year-old woman who was known to have a generalized rod-cone dystrophy since she was 8 years old. RESULTS: The peripheral fundus examination revealed a curvilinear lesion which resembles a well-known finding associated with a presumed ocular histoplasmosis syndrome or multifocal choroiditis. CONCLUSIONS: The differential diagnosis of a peculiar curvilinear pigmentary lesion in the peripheral fundus may be expanded to include a generalized rod-cone dystrophy

PURPOSE: To investigate the manifestations and clinical course of Coats' disease diagnosed in adulthood and to describe the characteristics of patients who develop it. DESIGN: Retrospective observational case series. PARTICIPANTS: Thirteen eyes of 13 patients with Coats' disease diagnosed after age 35 years. METHODS: All patients examined in the authors' referral practices for Coats' disease diagnosed after the age of 35 years were identified. Patients with a history of radiation exposure, intraocular inflammation, retinal vascular occlusion, or Coats' response were excluded. A review of their historical, clinical, and fluorescein angiographic features and demographics was performed. RESULTS: There were 13 patients with a mean age at diagnosis of 50 years (range, 36-79) and a mean follow-up of 5.8 years (range, 0-17). These patients manifested many findings typical of Coats' disease, including the unilateral nature of the disease (13/13 patients), male predominance (12/13), vascular telangiectasis, lipid exudation, macular edema, and areas of capillary nonperfusion with adjacent webs of filigreelike capillaries. Disease was limited to < or =6 clock hours in 10 of 13 patients (76.9%), did not generally involve the retinal vasculature anterior to the equator, and showed limited potential to expand during the follow-up period. A localized hemorrhage was noted in 10 of 13 patients (76.9%), almost always near larger aneurysmal dilatations. Limited exudative detachment of the retina was seen in 4 eyes, 3 of which responded to localized laser photocoagulation. One patient had subretinal fluid seen on ocular coherence tomography that did not require treatment. On average, patients lost 2.1 lines of visual acuity during the follow-up period; 6 patients had a decline in vision, 2 patients had improved vision, and 3 patients had stable vision. No patient had end-stage findings of Coats' disease, such as iris neovascularization or total exudative detachment. CONCLUSIONS: Coats' disease can first be diagnosed in adulthood with retinal vascular abnormalities similar to those seen in younger patients. There are a number of important differences in disease manifestation in adults, including limited area of involvement, slower apparent progression of disease, and hemorrhage near larger vascular dilatations