31.1 Features
Angioid streaks are an ocular pathology of striking clinical appearance. The streaks present as reddish breaks in Bruch’s membrane that radiate and branch from the nerve, often emulating the course of the major retinal vessels. Angioid streaks are most commonly associated with pseudoxanthoma elasticum (PXE), but have also been described in association with Paget’s disease, Ehlers–Danlos syndrome, hemoglobinopathies, and approximately half of cases are idiopathic. The fundamental defect appears to be calcification of Bruch’s membrane, which leads to a brittle character that is predisposed to spontaneous breaks and the development of choroidal neovascularization (CNV). Less frequently, traumatic choroidal rupture may occur with minimal force. Although CNV may be treated with anti-vascular endothelial growth factor (VEGF) intravitreal injections, some patients progress to disciform scar formation or macular atrophy.
31.1.1 Common Symptoms
It is typically asymptomatic unless CNV or hemorrhage develops which presents as vision loss and/or metamorphopsia.
31.1.2 Exam Findings
Pale gray, pink, red, or brown radiating cracks in Bruch’s membrane emanate from the disc, a zone of peripapillary atrophy, or a peripapillary ring of similar appearance can be seen; often associated with disc drusen, pattern dystrophy of the macula, and a mottled appearance of the temporal macula known as “peau d’orange” (▶ Fig. 31.1). A subtle, diffuse opacification of Bruch’s membrane with obscuration of the choroidal vessels, presumably from pathologic calcification, extends from the disc to the area of peau d’orange where the calcification of Bruch’s is less confluent; the streaks frequently terminate at this border. In late stages, macular retinal pigment epithelial (RPE) atrophy and/or disciform scarring may be present, obscuring the streaks.
Fig. 31.1 (a,b) Color fundus photo of the left eye demonstrating characteristic appearance of angioid streaks.
31.2 Key Diagnostic Tests and Findings
31.2.1 Optical Coherence Tomography
Breaks in Bruch’s membrane correspond to the streaks. Breaks may be associated with focal detachment or absence of the overlying RPE; underlying choriocapillaris may also be absent (▶ Fig. 31.2 and ▶ Fig. 31.3). There may be fibrous tissue ingrowth at the breaks. CNV is typically seen in association with these breaks, and may have associated intra- and/or subretinal fluid. Enhanced reflectance is also noted at the most external aspect of the RPE–Bruch’s membrane reflectivity band, perhaps secondary to calcification of Bruch’s membrane.
Fig. 31.2 (a) Near-infrared reflectance (NIR) image and (b) optical coherence tomography (OCT) in the right eye of the same patient as ▶ Fig. 31.1 demonstrating angioid streaks and corresponding breaks in Bruch’s membrane (arrows). The temporal area of peau d’orange apparent as a granular pattern of reflectance on NIR is seen as greater speckling at the basal aspect of the retinal pigment epithelial band on OCT (arrowheads). There is an active choroidal neovascularization membrane with subretinal fluid and disruption of retinal architecture (asterisk).
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