It’s safe to say that the subject of angioid streaks seldom crosses our minds after National Boards. At best, they remain an obscure footnote from our optometric education with little to no impact in our daily practices.
Rarely seen let alone recognized, their presentation can confuse even the most experienced optometrist. Let’s briefly reacquaint ourselves with this seemingly exotic retinal finding to take some of the mystery out of its diagnosis and clinical significance.
Angioid streaks are typically seen in patients with a history of inherited disease. The most common systemic disease associated with its presentation is pseudoxanthoma elasticum (PXE) that follows an autosomal recessive pattern.
Ehlers-Danlos Syndrome (EDS) and Paget’s disease, the next two most commonly associated diseases, have an autosomal dominant inheritance association. The various hemoglobinopathies associated with angioid streaks, of which sickle cell trait is the most common, also have a well-documented genetic link.1-5
Angioid streaks arise from an alteration in Bruch’s membrane due to visible crack-like dehiscences in the tissue coupled with an atrophy of the overlying retinal pigment epithelium (RPE).1 They are not simply a benign retinal entity but can present a serious risk to vision—if untreated, they can lead to macular choroidal neovascularization (CNV) and subsequent central vision loss.
Although past treatments such as laser photocoagulation, transpupillary thermoplasty, and macular translocation surgery have been the accepted treatments in the past, their use has proven frustratingly ineffective. The advent of photodynamic therapy (PDT) and anti-vascular endothelial growth factor treatments (anti-VEGF) have recently shown much promise as less destructive and more effective ways to treat the problem.1