Irregular dark red or brown lines radiating from a ring of peripapillary atrophy surrounding the optic nerve head are referred to as angioid streaks because their appearance mimics the appearance of blood vessels. On FA, characteristic window defects with late staining are noted resulting from dehiscences or cracks in the thickened and calcified Bruch membrane (Fig 4-16).
The systemic disease most commonly associated with angioid streaks is pseudoxanthoma elasticum (PXE), or Grönblad-Strandberg syndrome, a predominantly autosomal recessive disorder inherited through mutation in the ABCC6 gene located on band 16p13.1. Additional fundus findings associated with PXE include optic nerve head drusen, peripheral round atrophic scars with a “comet” sign, and a mottled RPE appearance referred to as peau d’orange (“skin of an orange”). Paget disease of bone, beta thalassemia, sickle cell anemia (SS), and Ehlers-Danlos syndrome may also be associated with angioid streaks. If the ophthalmologist establishes a new diagnosis of angioid streaks, and the patient is not known to have any of the aforementioned conditions, the patient should be referred for work-up and management of the possible underlying systemic disease.
Figure 4-15 Ocular histoplasmosis syndrome with CNV. A, Fundus photograph shows peripapillary atrophy and numerous atrophic scars. B, Transit frame of the angiographic study reveals blocked fluorescence from blood and pigment as well as hyperfluorescence resulting from the CNV (arrows) and choroidal transmission in areas of atrophy. C, Leakage from the choroidal neovascular membrane (arrows) late in the study, as well as staining of the sclera beneath atrophic scars.
Angioid streaks usually are asymptomatic unless they are subfoveal. Visual disturbances may develop due to submacular hemorrhage resulting from trauma, but these disturbances may resolve spontaneously if there is no CNV. The most significant visual complication is the development of CNV.
Safety glasses are an advisable precaution for patients with angioid streaks because they can be highly susceptible to choroidal rupture following even minor blunt injury. Medical consultation is indicated to evaluate for systemic manifestations of PXE including “plucked chicken” skin appearance, calcific arteriosclerosis of coronary arteries, and gastrointestinal and cerebrovascular bleeding.
When CNV develops, treatment with anti-VEGF agents has supplanted the use of laser treatment or PDT, as noted previously.
Figure 4-16 Color montages of fundus photographs from a patient with pseudoxanthoma elasticum showing, in both eyes, angioid streaks radiating from the optic nerve head, a “peau d’orange” appearance of the fundus temporal to the macula, optic nerve head drusen, midperipheral comet lesions, and, in the left eye, an old, inactive CNV.
(Courtesy of Stephen J. Kim, MD.)
Chang LK, Spaide RF, Brue C, Freund KB, Klancnik JM Jr, Slakter JS. Bevacizumab treatment for subfoveal choroidal neovascularization from causes other than age-related macular degeneration. Arch Ophthalmol. 2008;126(7):941–945.
Heier JS, Brown D, Ciulla T, et al. Ranibizumab for choroidal neovascularization secondary to causes other than age-related macular degeneration: a phase I clinical trial. Ophthalmology. 2011;118(1):111–118.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.