Coats' disease: a study of 62 histologically confirmed cases.

We studied 62 eyes submitted to the Armed Forces Institute of Pathology (AFIP) from 1958 through 1980 that satisfied our criteria for the histologic diagnosis of Coats' disease. Our histopathologic definition of Coats' disease was the presence of a primary vascular lesion consisting of retinal telangiectasia with leakage of plasma to form intraretinal and subretinal exudates. In the cases we reviewed, Coats' disease occurred more frequently in boys, it usually affected only one eye, and was generally detected in the first decade of life. In 52 cases (79%) the clinical manifestations, strabismus and leukokoria, were thought to be caused by retinoblastoma. Angle closure glaucoma was present in 36 cases (58%). In all but one of the cases studied, the lesion was located peripheral to the equator. We further identified diffuse involvement of capillaries in the peripheral retina using trypsin-digest preparations. Associated histologic findings included: rubeosis iridis, cataract, vitreous neovascularization, and nodules resulting from fibrous metaplasia of the retinal pigment epithelium. These fibrous nodules typically occurred in the macular area and occasionally contained calcium or bone.