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Angioid Streaks and Associated Conditions

Update: Nicole Morrow, BS; Lorraine M. Provencher, MD

Original Contributor: Eric Chin, MD

Photographer: Cindy Montague, CRA

Update: May 15, 2018; Original Post: August 15, 2013

Angioid streaks are visible crack-like breaks in Bruch's membrane due to an atrophic overlaying retinal pigment epithelium. They radiate from the optic nerve and can have a peau d'orange appearance originating from the macula and extending temporally (Figures 1 and 2). They are termed angioid streaks for their resemblance to retinal vasculature. Associated optic disc drusen are common (Figure 1 and 3). Patients with angioid streaks are typically asymptomatic, even if the streaks cross into the foveal region. Associated complications include choroidal neovascularization and choroidal rupture with minimal trauma.

Fig 1. Right eye (A): Disc drusen (arrow heads), normal vasculature, and angioid streaks (arrows) emanating from the optic nerve (click images for higher resolution). Left eye (B): Disc drusen (arrow heads), normal vasculature, and angioid streaks (arrows) emanating from the optic nerve (click image for higher resolution).
Right eye (A): Disc drusen (arrow heads), normal vasculature, and angioid streaks (arrows) emanating from the optic nerve (click images for higher resolution). Left eye (B): Disc drusen (arrow heads), normal vasculature, and angioid streaks (arrows) emanating from the optic nerve
Fig 2. Right eye (A): Another example of angioid streaks emanating from the optic nerve (click image for higher resolution). Left eye (B): Another example of angioid streaks emanating from the optic nerve (click image for higher resolution).
Right eye (A): Another example of angioid streaks emanating from the optic nerve Left eye (B): Another example of angioid streaks emanating from the optic nerve
Fig 3. Autofluorescence, both eyes. Areas of increased autofluorescence at the nerve corresponding to the disc drusen; radial areas of hypo-autofluorescence corresponding to angioid streaks (click image for higher resolution).
Autofluorescence, both eyes. Areas of increased autofluorescence at the nerve corresponding to the disc drusen; radial areas of hypo-autofluorescence corresponding to angioid streaks

Angioid streaks are most commonly associated with pseudoxanthoma elasticum (PXE). PXE is also known as Gronblad-Strandberg syndrome and is caused by a mutation in the ABCC6 gene. Cutaneous findings typically precede ocular findings by a few years. The cutaneous findings will be small, yellowish papular lesions with skin laxity affecting the neck, axilla, groin, and flexural creases (Figure 4). PXE patients may also have gastrointestinal and cardiovascular findings.

Fig 4. External photograph of the neck: Small papular lesions and cutaneous laxity of the neck ("plucked chicken skin") consistent with pseudoxanthoma elasticum (click image for higher resolution).
External Photo. Neck, right side view. Small papular lesions and cutaneous laxity of the neck ("plucked chicken skin") consistent with pseudoxanthoma elasticum.

In addition to PXE, angioid streaks are associated with Ehler's Danlos syndrome, Paget's disease of bone, sickle cell anemia, and idiopathic (PEPSI is a helpful memory mnemonic). Other associations include: abetalipoproteinemia, acromegaly, diabetes mellitus, facial angiomatosis, hemochromatosis, hemolytic anemia, hereditary spherocytosis, hypercalcinosis, hyperphosphatemia, lead poisoning, myopia, neurofibromatosis, senile elastosis, Sturge-Weber syndrome, and tuberous sclerosis.

Reference(s)

  1. Freund, BK, Sarraf, D, Mieler, WF, & Yannuzzi, L. (2017). Degeneration. The Retinal Atlas (2nd ed, pp 651-761).  Elsevier Inc.
  2. Georgalas, I, Papaconstantinou, D, Koutsandrea, C, Kalantzis, G, Karagiannis, D, Georgopoulus, G, & Ladas, I. Angioid streaks, clinical course, complications, and current therapeutic management. Ther Clin Risk Manag. 2009; 5: 81-89.

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last updated: 08/15/2013; updated 05/17/2018
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