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Angioid streaks

AI overview

Angioid streaks are small breaks in Bruch's membrane, an elastic tissue containing membrane of the retina that may become calcified and crack.

Key points
  • Up to 50% of angioid streak cases are idiopathic.
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    Up to 50% of angioid streak cases are idiopathic.
  • May occur secondary to blunt trauma or be associated with many systemic diseases.
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    It may occur secondary to blunt trauma, or it may be associated with many systemic diseases.
  • Often associated with pseudoxanthoma elasticum, Paget's disease, sickle cell disease, and Ehlers–Danlos syndrome.
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    Angioid streaks are often associated with pseudoxanthoma elasticum , but have been found to occur in conjunction with other disorders, including Paget's disease , sickle cell disease and Ehlers–Danlos syndrome .
  • First described by Robert Walter Doyne in 1889 in a patient with retinal hemorrhages.
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    They were first described by Robert Walter Doyne in 1889 in a patient with retinal hemorrhages.
  • Discovered in the 1930s to be caused by changes at the level of Bruch's membrane.
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    From histopathological research in the 1930s, they were discovered to be caused by changes at the level of Bruch's membrane .
  • Pathology may involve elastic degeneration of Bruch's membrane, iron deposition, and impaired nutrition due to stasis and small vessel occlusion.
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    Presently, it is believed that its pathology may be a combination of elastic degeneration of Bruch's membrane, iron deposition in elastic fibers from hemolysis with secondary mineralization, and impaired nutrition due to stasis and small vessel occlusion .
Symptoms
  • The condition is usually asymptomatic.
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    The condition is usually asymptomatic
  • Decrease in vision may occur due to choroidal neovascularization.
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    decrease in vision may occur due to choroidal neovascularization.
  • Vision can be impaired if streaks progress to the fovea and damage the retinal pigment epithelium.
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    vision can be impaired if the streaks progress to the fovea and damage the retinal pigment epithelium .
  • Choroidal neovascularization can lead to vision loss and/or metamorphopsia.
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    Secondary ocular complications like choroidal neovascularization lead to vision loss, and/or metamorphopsia .
Diagnosis
  • Diagnosis is mainly clinical.
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    The diagnosis is mainly clinical
  • Fundus fluorescein angiography shows streaks appear hyperfluorescent (window defect) in the early phase.
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    fundus fluorescein angiography shows that the streaks appear hyperfluorescent (window defect) in the early phase.
  • Indocyanine green angiography can be used for diagnosis and associated ocular pathologies.
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    Indocyanine green angiography can also be used for diagnosing angioid streaks and their associated ocular pathologies.
  • Retinal fundus examination may reveal grey or dark red spoke-like lesions around the optic disk radiating outward.
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    Retinal fundus examination may reveal grey or dark red spoke like lesions around optic disk and radiating outward from peripapillary area.
  • Peau d'orange (leopard skin pattern) may be seen in association with pseudoxanthoma elasticum.
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    Peau d'orange (orange skin), also known as leopard skin pattern may be seen in association with pseudoxanthoma elasticum.
  • Optic disc drusen may also be seen.
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    Optic disc drusen may also seen.
Treatment
  • Management starts with complete medical checkup to rule out underlying systemic associations.
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    Management of angioid streaks starts with complete medical checkup to rule out underlying systemic associations.
  • Usually asymptomatic and initially does not need any treatment.
    source quote
    The condition is usually asymptomatic and at first do not need any treatment.
  • If choroidal neovascularization is present, treatment options include anti-VEGF medication, laser photocoagulation, photodynamic therapy, transpupillary thermotherapy, and macular translocation surgery.
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    If choroidal neovascularization is present, treatment options like anti-VEGF medication , laser photocoagulation , photodynamic therapy , transpupillary thermotherapy, macular translocation surgery etc. may be needed.
Red flags
  • Choroidal neovascularization leads to vision loss and/or metamorphopsia.
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    Secondary ocular complications like choroidal neovascularization lead to vision loss, and/or metamorphopsia .
  • Streaks progressing to the fovea can impair vision by damaging the retinal pigment epithelium.
    source quote
    vision can be impaired if the streaks progress to the fovea and damage the retinal pigment epithelium .
AI-synthesized from the Wikipedia article “Angioid streaks”. Not medical advice. Verify source →
Angioid streaks
Bruch's membrane
SpecialtyOphthalmology 
ComplicationsLoss of vision[1]
Diagnostic methodFFA, ICGA

Angioid streaks, also called Knapp streaks or Knapp striae, are small breaks in Bruch's membrane, an elastic tissue containing membrane of the retina that may become calcified and crack.[2] Up to 50% of angioid streak cases are idiopathic.[3] It may occur secondary to blunt trauma, or it may be associated with many systemic diseases.[4] The condition is usually asymptomatic, but decrease in vision may occur due to choroidal neovascularization.[5]

Angioid streaks are often associated with pseudoxanthoma elasticum, but have been found to occur in conjunction with other disorders, including Paget's disease, sickle cell disease and Ehlers–Danlos syndrome. These streaks can have a negative impact on vision due to choroidal neovascularization or choroidal rupture. Also, vision can be impaired if the streaks progress to the fovea and damage the retinal pigment epithelium.[citation needed]

The diagnosis is mainly clinical, however fundus fluorescein angiography shows that the streaks appear hyperfluorescent (window defect) in the early phase.[1] Indocyanine green angiography can also be used for diagnosing angioid streaks and their associated ocular pathologies.[6]

Retinal fundus examination may reveal grey or dark red spoke like lesions around optic disk and radiating outward from peripapillary area. Peau d'orange (orange skin), also known as leopard skin pattern may be seen in association with pseudoxanthoma elasticum. Optic disc drusen may also seen.[1]

Management of angioid streaks starts with complete medical checkup to rule out underlying systemic associations. The condition is usually asymptomatic and at first do not need any treatment.[3] Secondary ocular complications like choroidal neovascularization lead to vision loss, and/or metamorphopsia.[3] If choroidal neovascularization is present, treatment options like anti-VEGF medication, laser photocoagulation, photodynamic therapy, transpupillary thermotherapy, macular translocation surgery etc. may be needed.[3]

They were first described by Robert Walter Doyne in 1889 in a patient with retinal hemorrhages. In 1892, ophthalmologist Hermann Jakob Knapp called them "angioid streaks"[4] because of their resemblance to blood vessels. From histopathological research in the 1930s, they were discovered to be caused by changes at the level of Bruch's membrane. Presently, it is believed that its pathology may be a combination of elastic degeneration of Bruch's membrane, iron deposition in elastic fibers from hemolysis with secondary mineralization, and impaired nutrition due to and small vessel occlusion.[citation needed]

  1. 1 2 3 John F., Salmon (2020). "Acquired macular disorders". Kanski's clinical ophthalmology : a systematic approach (9th ed.). Edinburgh: Elsevier. pp. 607–609. ISBN 978-0-7020-7713-5. OCLC 1131846767.
  2. DermAtlas Archived June 7, 2012, at the Wayback Machine - Johns Hopkins
  3. 1 2 3 4 Tripathy, Koushik; Quint, Jessilin M. (August 22, 2022). "Angioid Streaks". StatPearls Publishing. PMID 30844178. Retrieved October 27, 2023.
  4. 1 2 "Angioid Streaks — EyeWiki". eyewiki.aao.org.
  5. "Retina". The Wills eye manual : office and emergency room diagnosis and treatment of eye disease (7th ed.). Philadelphia, PA: Wolters Kluwer. 2017. ISBN 978-1-4963-5366-5. OCLC 951081880.
  6. Creig S, Hoyt; David, Taylor (January 2012). Pediatric ophthalmology and strabismus (4th ed.). Saunders/Elsevier. p. 524. ISBN 9780702046919.