The continuum of primary retinal telangiectasia.

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.

"Idiopathic" intracranial hypertension caused by venous sinus thrombosis associated with contraceptive usage.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a life-threatening condition that may present with symptoms and signs identical to idiopathic intracranial hypertension (IIH). Both conditions consist of increased intracranial pressure (ICP); however, IIH shows no evidence of contributory intracranial pathology. Oral hormonally based contraceptive usage has been associated with both conditions as well. Because disc edema often occurs in CVST, and IIH and is evident in other sight- and life-threatening conditions such as intracranial mass lesions, neuroimaging and other ancillary testing must be conducted for definitive diagnosis. CASE: An 18-year-old white woman with IIH previously diagnosed presented with headaches and severe visual loss in the left eye. Bilateral disc edema with macular edema in the left eye (O.S.) was present. Threshold perimetry found bilaterally enlarged blind spots as well as a central scotoma O.S. Her medical history was significant for a right transverse, sigmoid and jugular siphon thrombosis secondary to a hypercoagulable state and associated with her use of oral birth control medication. Subsequent magnetic resonance venography (MRV) found the presence of CVST. Despite anticoagulation therapy and acetazolamide, she had severe, nonresolving papilledema. Treatment with oral prednisone was initiated. She recovered full visual fields and excellent visual acuity. CONCLUSION: Because of the similarity in clinical presentation of CVST and IIH, it is important to differentiate distinguishing characteristics of these diseases for correct diagnosis and prompt treatment.