Angioid streaks, clinical course, complications, and current therapeutic management

Abstract

Angioid streaks are visible irregular crack-like dehiscences in Bruch's membrane that are associated with atrophic degeneration of the overlying retinal pigmented epithelium. Angioid streaks may be associated with pseudoxanthoma elasticum, Paget's disease, sickle-cell anemia, acromegaly, Ehlers-Danlos syndrome, and diabetes mellitus, but also appear in patients without any systemic disease. Patients with angioid streaks are generally asymptomatic, unless the lesions extend towards the foveola or develop complications such as traumatic Bruch's membrane rupture or macular choroidal neovascularization (CNV). The visual prognosis in patients with CNV secondary to angioid streaks if untreated, is poor and most treatment modalities, until recently, have failed to limit the devastating impact of CNV in central vision. However, it is likely that treatment with antivascular endothelial growth factor, especially in treatment-naive eyes to yield favorable results in the future and this has to be investigated in future studies.

Figure 1

Red free fundus photo showing typical angioid streaks.

Figure 2

Red free fundus photo showing an angioid streak crossing the macula.

Figure 3

“Window defect” in fluorescein angiography due to atrophy of RPE adjacent to angioid streaks.

Figure 4

Indocyanine Green angiography of angioid streaks please note the hyperfluorescent lines with ‘pinpoints’.

Figure 5

Macular hemorrhage complicating an eye with angioid streaks; note the “peu d’orange” appearance of the fundus temporal to the macula.

Figure 6

Color fundus photograph in an eye with angioid streaks and choroidal neovascularization.

Figure 7

Fluorescein angiography of left eye with angioid streaks complicated by choroidal neovascularization.

Figure 8

Fluorescein angiography of right eye with angioid streaks complicated by choroidal neovascularization.