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.

Intravitreal aflibercept for refractory choroidal neovascularization secondary to angioid streaks

“…CNV is the most comman and serious sight threatening complication of AS. 2,3 Aflibercept is a new anti-VEGF agent which binds all isomers of VEGF-A, VEGF-B, placental growth factor and prevent their activity. 4 There are restricted information about using intravitreal aflibercept in CNV secondary to AS.…”

Angioid Streaks in Psuedoxanthoma Elasticum

“…The association of the optic nerve head drusen and angioid streaks has been reported in the literature with a frequency of 5% in PXE patients [11]. It is important to pay attention to this point that the Patients described with angioid streaks here had been diagnosed with PXE before ophthalmologic examination and it should be kept in mind that when a patient is diagnosed with angioid streaks there are numerous other diseases such as Paget's disease, sickle-cell anemia, acromegaly, Ehlers-Danlos syndrome, and diabetes mellitus that these lesions may be associated with, and not only PXE, a thorough systemic evaluation and differentiation between these etiologies is therefore necessary for preventing mismanagement [7].…”

“…PXE, also known as Groenblad Strandberg syndrome [7] is an autosomal recessive disease, characterized by systemic mineralization and fragmentation of elastic fibers affecting the skin, eye, cardiovascular system and arterial blood vessels…”

“…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) which will then lead to profound loss of vision [7].…”

Drastic effect of ranibizumab on choroidal neovascularization in idiopathic angioid streaks

“…Angioid streaks may be observed in isolation, but are often associated with systemic diseases such as Paget's disease, pseu doxanthoma elasticum, Ehlers-Danlos syndrome, or sickle cell anemia. The main cause of severe visual loss in cases of angioid streaks is the development of choroidal neovascularization (CNV) (1) . CNV develops in 42% to 86% of eyes with angioid streaks and typically has a poor outcome (2) .…”