Angioid Streaks

SALIENT FEATURES

Angioid streaks (AS) are linear breaks in Bruch membrane that typically radiate from the optic disc and take on a deep red, brown, or gray appearance. They are usually bilateral (Figures 25.1 and 25.2).
AS are classically associated with pseudoxanthoma elasticum (59%-87% incidence), Ehlers-Danlos syndrome, Paget disease of bone (8%-15% incidence), and sickle cell anemia and other hemoglobinopathies (0.9%-6% incidence). Half of individuals with AS have an idiopathic etiology.
Mineralization of Bruch membrane can result in the rupture of the brittle layer with or without inciting trauma. Adjacent retinal pigment epithelium (RPE), choriocapillaris, and photoreceptors may secondarily be disrupted (Figures 25.1, 25.2, 25.3, 25.4).
Vision loss typically results from late complications of AS including hemorrhage, edema, and choroidal neovascularization (CNV) at the site of the breaks (42%-86% incidence, commonly subfoveal) with subsequent disciform scar (Figures 25.2, 25.4 and 25.5).

OCT IMAGING

Optical coherence tomography (OCT) identifies focal breaks in Bruch membrane corresponding to the location of AS (Figures 25.3 and 25.4).
OCT may also reveal hyperreflective areas of mineralized Bruch membrane.

Intraretinal and choroidal hyperreflective foci, similar to those seen in age-related macular degeneration, may correlate to CNV activity.

FIGURE 25.1 Color fundus photograph of the right eye of a patient with angioid streaks (AS). Numerous brown-gray streaks (white arrowheads) radiate from the optic nerve head and run deep to the retina vessels. Several demonstrate a halo of hypopigmentation which highlights the angioid streaks.

FIGURE 25.2 Color fundus photograph of the left eye of the same patient with angioid streaks (AS). Numerous prominent AS are seen extending from the optic nerve with two denoted by white arrowheads. Additionally, a pigmented, disciform scar with hemorrhage secondary to chronic choroidal neovascularization is centered in the macula at the intersection of several AS (white asterisk).

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