Angioid streaks

Angioid streaks are bilateral, reddish-brown to gray, branched, vessel-like (angioid) pigment streaks in the retina that radiate outward from the optic disc in all directions. These streaks are clearly distinct from the light- or dark-red arteries or veins of the fundus.

The incidence is estimated at 1:100,000.

The diagnosis is usually made in the third to fourth decade of life. In this distinctly chronic disease one can distinguish 2 phases, a short, acute one, in which suddenly gaping tears in the lamina vitrea with subretinal bleeding and exudation appear at the posterior pole of the eye, and a long, subacute one, in which a concentric and meridional system of tears develops, which gradually become wider and longer. The colour and colour change of the cracks depend on the pigment tissue that appears in the crack area under different optical conditions and whose quantity and spatial distribution changes. Pseudodrusen are often detectable by OCT examinations (Kumar V 2018).

Pathologically and anatomically, these are tears in the thickened and calcified fracture membrane that separates the vascular skin from the retina. As a consequence, secondary choroidal neovascularisations often develop, which, if centrally located, result in a significant loss of visual acuity for the often young patients.

In about half of the patients "angioid streaks" are associated with systemic diseases (Clarkson JG et al. 1982):

• Pseudoxanthoma elasticum
• Beta-Thalassemia
• Sickle cell anaemia
• Marfan syndrome
• Ehlers-Danlos Syndrome
• Paget's disease

Clinical findings; detection by infrared retinography; optical coherence tomography; fluorescein angiography, indocyanine green angiography (Chatziralli I et al. 2019)

There is no causal treatment for patients with "angioid streaks." All treatment regimens are aimed at treating secondary CNV (choroidal neovascularization). According to current recommendations, three intravitreal injections of VEGF inhibitors should be administered at monthly intervals upon the initial diagnosis of secondary CNV, followed by further follow-up injections as needed.

Spontaneous neovascularisation, haemorrhages and scars lead to changes in visual acuity (60-70% of patients have visual acuity changes in the 3rd - 4th decade of life).

1. Chatziralli I et al (2019) Angioid streaks: A Comprehensive Review From Pathophysiology to Treatment. Retina 39:1-11.
2. Clarkson JG et al (1982) Angioid streaks. Surv Ophthalmol 26:235-246.
3. Kumar V (2018) Reticular Pseudodrusen and Thin Choroid Are Associated With Angioid Streaks. Ophthalmic Surg Lasers Imaging Retina 49: 402-408.