Bilateral Purtscher-Like Retinopathy in a Child With Connective Tissue Disease

Abstract

A previously healthy 12-year-old girl presented with a 2-month history of intermittent severe myalgia, irregular fever, anorexia, alopecia, hyperpigmentation, and 1 week of bilateral acute vision loss. The Rheumatology and Immunology Department diagnosed her with connective tissue disease by signs and corresponding ancillary tests. Her best-corrected visual acuity was 20/500 in both eyes, and fundoscopy showed optic swelling, multiple cotton-wool spots around the macula, a few intraretinal hemorrhages, mild venous tortuosity, retinal artery narrowing, clear polygonal areas of retinal whitening (Purtscher flecken), and pseudo cherry-red spots (Fig. 1A, B). Fundus fluorescein angiography of the right eye showed widespread arteriolar occlusion and capillary nonperfusion of the superior and temporal retina involving the macula. The left eye showed nonperfusion of the temporal macular area (Fig. 1C, D). Purtscher-like retinopathy (PLR) was diagnosed. PLR is a rare devastating ocular manifestation of connective tissue disease. The most accepted theory about patho-physiology of PLR is embolic occlusion of the precapillary arterioles. 1 Timely glucocorticoid treatment may partially relieve the embolism to retain as much useful vision as possible. 2 The widespread nonperfusion area requires prompt laser treatment to prevent the development of neovascularization.