Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review

Abstract

Idiopathic peripheral retinal telangiectasia (IPT), often termed as Coats disease, can present in a milder form with the onset in adulthood. The goal of this case series study and literature review was to describe and classify different presenting forms and treatment of this entity and to review contemporary methods of its management. Six cases of adult onset IPT were described with the following phenotypes based on fundus ophthalmoscopy, fluorescein angiography, and optical coherence tomography findings: IPT without exudates or foveal involvement, IPT with peripheral exudates without foveal involvement, IPT with peripheral exudates and cystoid macular edema, and IPT with peripheral and macular hard exudates. Treatments applied in this series included observation, laser photocoagulation, and anti-vascular endothelial growth factor (VEGF) treatment with variable outcomes depending upon the extent of IPT, the aggressiveness of laser treatment, and the stringency of follow-up. The accompanying literature review suggests that ablative therapies, especially laser photocoagulation, remain the most effective treatment option in adult-onset IPT, with anti-VEGF therapy serving as an adjuvant procedure. Close follow-up is necessary to achieve and maintain reasonable good visual and morphological results.

Not your usual macula edema!

“…[ 1 ] It is essential to differentiate the idiopathic variant of coats disease from “coats-like response” which may be secondary to infective, inflammatory, degenerative, and neoplastic ocular pathologies. [ 2 ] The disease is slowly progressive in adults with less than two quadrant involvement in contrast to the classical childhood variant. Management depends on features at presentation and includes laser ablation of the telangiectatic vessels and cryotherapy.…”

High-Resolution Imaging in Macular Telangiectasia Type 2: Case Series and Literature Review

“…Capillary, venular, and arteriolar aneurysms represent the primary features of Coats' disease. Yannuzzi et al proposed that IMA Type 1 is essentially a variant of Coats' disease, uniquely localized to the macular region [2,6]. Peripheral aneurysmal telangiectasias typically exhibit a larger extent compared to Coats' disease in the macular region and are more frequently associated with areas of hypoperfusion [2].…”

“…These conditions vary in appearance, are presumed to be pathogenesis, and necessitate different management approaches [1]. These entities are distinguished from the generalized retinal telangiectasis of the "idiopathic peripheral retinal telangiectasia" (IPT), also known as Coats disease [2]. They are additionally set apart from the more common retinal telangiectasis, which arises as a secondary effect of inflammatory or vascular conditions such as retinal vein occlusion, diabetes, irradiation, or the obstruction of the carotid artery [1,3].…”

Unilateral Retinal Telangiectasia with Peripheral Vascular Sheathing: A Case Report

“…Idiopathic retinal telangiectasia (IRT) refers to aneurysmal or ectatic changes in retinal blood vessels without identi able secondary causes. [1][2][3] It is usually associated with exudation and can cause exudative retinal detachment in severe form. [1][2][3] IRT comprises a wide spectrum of disease including Coats' disease, which represents the most severe entity, to less severe entities such as Leber's miliary aneuryms, macular telangiectasia (MACTEL), and simple peripheral retinal telangiectasia.…”

“…[1][2][3] It is usually associated with exudation and can cause exudative retinal detachment in severe form. [1][2][3] IRT comprises a wide spectrum of disease including Coats' disease, which represents the most severe entity, to less severe entities such as Leber's miliary aneuryms, macular telangiectasia (MACTEL), and simple peripheral retinal telangiectasia. [1][2][3] Typically, IRT affects males, with more severe disease presenting early during the rst decade, and vice versa.…”