The continuum of primary retinal telangiectasia

Abstract

Background Primary retinal telangiectasia is characterized by abnormalities in the retinal vasculature. Any alteration of the normal retinal vasculature may result in variable degrees of retinal leakage, hemorrhages, and exudates. The retinal telangiectatic conditions of Coats’ disease, Leber’s miliary aneurysms, and idiopathic macular telangiectasia (IMT), although historically considered separate entities, may in fact be variants of the same pathophysiologic vascular process. This is based on observations noting that they share similarities in pathogenesis, histology, and clinical presentation. These observations are controversial and are contested in the literature. Conversely, others have documented Coats’ disease, Leber’s miliary aneurysms, and IMT as unique and separate retinal conditions, each with specific features. Methods Three cases are presented spanning the spectrum of primary retinal telangiectasia. Clinical evaluation, dilated fundus examination, and auxiliary testing document both the similar characteristics that the entities share as well as the distinct features, which define each disease’s nomenclature and categorization. Conclusion Coats’ disease, Leber’s miliary aneurysms, and IMT may be part of a singular clinical spectrum sharing pathophysiologic and histopathologic features and similarities in clinical presentation.