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

Maciej Gawęcki

doi:10.3390/jcm10081767

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.

Highlights

Idiopathic peripheral retinal telangiectasia (IPT), usually referred to as Coats disease, has been well-described in the medical literature since its discovery in 1908 [1]
A literature search was performed in the PubMed database for the phrase “peripheral retinal telangiectasias” and the combination of words “Coats disease” and “adult onset.”
IPT falls under the umbrella of the aneurysmal form of telangiectasia potentially associated with severe intraretinal leakage, subretinal exudation, and risk of retinal detachment

Summary

Introduction

Idiopathic peripheral retinal telangiectasia (IPT), usually referred to as Coats disease, has been well-described in the medical literature since its discovery in 1908 [1]. Coats disease is an idiopathic condition characterized by telangiectatic and aneurysmal retinal vessels with intraretinal and subretinal exudation and fluid without appreciable retinal or vitreal traction, frequently associated with retinal detachment [2]. Alterations of endothelium of retinal vessels lead to breaking of blood-retinal barrier and presence of abnormal pericytes, causing formation of telangiectasias and closure of some vessels [3,4]. Exudation of lipids from the damaged vessels follows and is responsible for retinal detachment and cyst formation as well as retinal ischemia due to vessel closure [5]. The process is described as similar to pathogenesis of diabetic retinopathy, but definitely more severe and rapid [6]

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Discussion

Conclusion