Angioid streaks (AS) refer to linear, irregular, crack-like ruptures of a calcified Bruch membrane (BM) radially spreading from the optic nerve toward the retinal periphery. Pathogenic processes include mechanical forces exerted by the extraocular muscles on a fragile and less flexible posterior pole.1 On the optical coherence tomography (OCT) B-scan, AS appear as a focal hyporeflective area of the retinal pigment epithelium (RPE)-BM complex.2 Recently, en face OCT performed at the depth of the BM has demonstrated enhancement of the visualization and characterization of AS.3
We describe a 58-year-old man diagnosed with pseudoxanthoma elasticum. Fundus photography showed peripapillary AS and macular geographic atrophy (Figure 1A). Fundus autofluorescence revealed a butterfly-shaped pattern dystrophy (Figure 1B). Angioid streaks appeared as speckled hyperautofluorescent lines (Figure 1B). En face OCT at the level of the RPE-BM complex enhanced the visualization of AS and demonstrated clustered hyperreflective dots (Figure 1C) that colocalized with the mottled RPE-BM complex on the OCT B-scan (Figure 1D). Intraretinal hyperreflective foci were also documented in the fovea on the OCT B-scan (Figure 1D). The 6-month follow-up en face OCT demonstrated loss of these hyperreflective dots and subsequent enlargement of the AS. On the OCT B-scan, marked RPE-BM complex atrophy developed within AS (Figure 2, A and B). The OCT B-scan in the superior macula depicted varying maturity of AS, including flat elevation of the RPE and RPE-BM atrophy (Figure 2C).
Intraretinal hyperreflective foci in AS have been described using the OCT B-scan and are supposed to be derived from the RPE cells undergoing transdifferentiation.4 In this article, we reported a novel en face OCT feature at the level of the RPE-BM complex within AS termed “hyperreflective dots.”
1. Chatziralli I, Saitakis G, Dimitriou E, et al. Angioid streaks
: a comprehensive review from pathophysiology to treatment. Retina 2019;39:1–11.
2. Corbelli E, Carnevali A, Marchese A, et al. Optical coherence tomography
angiography features OF angioid streaks
. Retina 2018;38:2128–2136.
3. Hanhart J, Greifner H, Rozenman Y. Locating and characterizing angioid streaks
with en face optical coherence tomography
: Retin Cases Brief Rep 2017;11:203–206.
4. Romano F, Mercuri S, Arrigo A, et al. Identification of hyperreflective foci in angioid streaks
. Eye 2019;33:1916–1923.