Faculty Bibliography

PURPOSE: Coats disease is a rare condition characterized by retinal vascular telangiectasia, aneurysms, and leakage from these abnormal blood vessels. We report the phenomenon and treatment of Coats disease with diffuse hyperpermeability from angiographically normal retinal capillaries. METHODS: This case series describes two patients with Coats disease, diagnosed based on fundus photography and fluorescein angiography. The first patient was treated with intravitreal bevacizumab and limited laser photocoagulation. The second patient was treated only with limited photocoagulation. RESULTS: The diffuse exudation from normal retinal capillaries resolved with treatment of the focal primary vascular Coats lesions. CONCLUSION: Vasculopathies such as Coats disease may produce diffuse hyperpermeability of otherwise normal capillaries in the fundus. Limiting treatment only to the primary Coats lesions is a minimally invasive strategy that preserves normal capillaries.

BACKGROUND: Best disease is an autosomal dominant retinal dystrophy with a variable phenotypic expression. Clinically, it is characterized by a vitelliform lesion in the macula because of the deposition of yellow material in a dome-shaped configuration, believed to be lipofuscin that accumulates within and beneath the retinal pigment epithelium. Best disease is occasionally complicated by the development of choroidal neovascularization (CNV), which typically occurs in the macula. We report a case of peripapillary CNV in Best disease. METHODS: Interventional case report. RESULTS: A 12-year-old boy who was previously diagnosed with Best disease was treated with reduced fluence photodynamic therapy for subfoveal CNV in the right eye. After 2 months, he presented with peripapillary CNV in the left eye, which was treated with repeated sessions of reduced fluence photodynamic therapy. CONCLUSION: Ophthalmologists must be aware that peripapillary CNV may occasionally complicate Best disease and can be successfully treated with photodynamic therapy.

PURPOSE: To report a case of atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits. METHODS: Observational case report of one patient. RESULTS: A 52-year-old man presented with reduced vision in the left eye. Findings resembling acute exudative polymorphous vitelliform maculopathy were noted with ophthalmoscopy, fluorescein angiography, and optical coherence tomography. Funduscopic examination revealed an exudative macular detachment with yellowish subretinal deposits inferior to the fovea. On fluorescein angiography, the perifoveal lesions were minimally hyperfluorescent, with no abnormal fluorescence in the central macula. The subretinal deposits were found to be hyperautofluorescent on fundus autofluorescence imaging. Optical coherence tomography confirmed a serous detachment of the retina with intraretinal cystic spaces. The right eye did not show any abnormalities except for an epiretinal membrane. CONCLUSION: We describe a case of atypical unilateral maculopathy associated with acute exudative polymorphous vitelliform maculopathy-like yellowish deposits.

A series of young adult patients developed solar retinopathy during sun exposure over a two-day period in a particular region of the United States during March of 1986. Evaluation of the photobiological and geophysical parameters involved in solar retinitis are presented. A multifactorial pathogenesis is proposed. Of interest, a possible increase in terrestrial ultra-violet B radiation secondary to a localized relatively low ozone column during the days involved may have contributed to the retinal damage. Recommendations for protection from solar retinitis are noted.

PURPOSE: To describe the clinical and angiographic features of a series of patients with acute syphilitic posterior placoid chorioretinitis (ASPPC) in the context of previously published cases. METHODS: A retrospective, noncomparative, multicenter chart review was performed on 16 patients with active ASPPC. Positive serologic tests supported the diagnosis in all patients. Color and red-free photographs as well as fluorescein angiography were obtained in each case. Indocyanine green angiography, optical coherence tomography, and fundus autofluorescence were performed on selected patients. A total of 44 previously published cases of ASPPC were identified using both a Medline Search and references listed in articles identified. RESULTS: Ocular involvement was bilateral in 9 of our 16 patients (56.3%). The mean and median ages at presentation were 40 and 38 years, respectively (range 28-57 years). Nine patients (56.3%) were human immunodeficiency virus positive, with most recent CD4 cell counts ranging from 160 cells/muL to 450 cells/muL and a median CD4 cell count of 250 cells/muL. Seven of 16 patients (43.8%) had a history of mucocutaneous manifestations of secondary syphilis, whereas 4 (25.0%) had evidence of neurosyphilis. Anterior chamber and/or vitreous inflammation was evident in 13 patients (81.3%). Fifteen of 16 patients had positive venereal disease research laboratory or rapid plasma regain titers, and 13 of 13 tested patients had a positive serum fluorescent treponemal antibody absorption. The initial vision in the 25 affected eyes ranged from 20/20 to counting fingers, with a median of 20/80. In all patients, posterior segment examination in the involved eyes revealed a large, yellowish, placoid, outer retinal lesion. Fluorescein angiography showed progressive hyperfluorescence in the area of the lesion, often with scattered focal hypofluorescence, or leopard spotting. Inflammation subsided, the yellowish lesions resolved, and vision improved shortly after antibiotic therapy in 20 of 25 affected eyes. Visual acuity at last visit ranged from 20/20 to 20/150, with a median final vision of 20/25. A review of the literature revealed 44 previously reported cases of ASPPC. Shared demographic, clinical, and angiographic features were summarized. CONCLUSION: Acute syphilitic posterior placoid chorioretinitis is an uncommon but clinically and angiographically distinct manifestation of ocular syphilis. All patients with characteristic clinical and angiographic findings of ASPPC should be tested for both neurosyphilis and human immunodeficiency virus coinfection. Vision recovery typically followed completion of appropriate antibiotic therapy.

Twenty-five patients with central serous pigment epitheliopathy (CSP), also known as central serous chorioretinopathy, have been observed to have inferior hemispheric retinal pigment epithelial atrophic tracts, presumptive of antecedent retinal detachments. Five of these patients were noted to have clinically discernible, dependent peripheral retinal detachments. The clinical and fluorescein angiographic features of these patients are reviewed. Alterations in the retina, the retinal pigment epithelium (RPE) and the choroid are also described. They include the commonly associated manifestations of CSP such as RPE leaks and macular detachment as well as some newly recognized disturbances such as retinal capillary dilatation (telangiectasia), retinal capillary leakage, retinal lipid deposition, cystoid macular edema, choriocapillaris atrophy, choroidal neovascularization and disciform scarring.

The theoretical rationale, the histopathologic evidence, and the preliminary clinical studies related to krypton red laser (KRL) photocoagulation of the ocular fundus are reviewed. The authors report on their experience with currently available laser systems using this wavelength (647.1 nm) for photocoagulation of retinal vascular proliferative diseases and chorioretinal diseases associated with exudative manifestations. A histopathologic and clinical comparison of argon blue-green laser (ABGL), the pure argon green laser (AGL), and the krypton yellow laser (KYL), with reference to photocoagulation treatment of the ocular fundus is also discussed.