Angioid streaks

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Angioid streaks
Buchs membrane.svg
Bruch's membrane
Specialty Ophthalmology   OOjs UI icon edit-ltr-progressive.svg
Complications Loss of vision [1]
Diagnostic method FFA, 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]

Contents

Clinical features

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.

Signs

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]

Diagnosis

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]

Management

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]

History

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 stasis and small vessel occlusion.

Related Research Articles

This is a partial list of human eye diseases and disorders.

<span class="mw-page-title-main">Fluorescein angiography</span> Technique for examining the circulation of the retina and choroid of the eye

Fluorescein angiography (FA), fluorescent angiography (FAG), or fundus fluorescein angiography (FFA) is a technique for examining the circulation of the retina and choroid using a fluorescent dye and a specialized camera. Sodium fluorescein is added into the systemic circulation, the retina is illuminated with blue light at a wavelength of 490 nanometers, and an angiogram is obtained by photographing the fluorescent green light that is emitted by the dye. The fluorescein is administered intravenously in intravenous fluorescein angiography (IVFA) and orally in oral fluorescein angiography (OFA). The test is a dye tracing method.

<span class="mw-page-title-main">Bruch's membrane</span> Component of the eye

Bruch's membrane or lamina vitrea is the innermost layer of the choroid of the eye. It is also called the vitreous lamina or Membrane vitriae, because of its glassy microscopic appearance. It is 2–4 μm thick.

<span class="mw-page-title-main">Metamorphopsia</span> Type of vision distortion

Metamorphopsia is a type of distorted vision in which a grid of straight lines appears wavy or partially blank. In addition, metamorphopsia can result in misperceptions of an object's size, shape, or distance to the viewer. People can first notice they suffer from the condition when looking at mini blinds in their home.

<span class="mw-page-title-main">Pseudoxanthoma elasticum</span> Genetic disease in which elastic fibers in certain tissues harden

Pseudoxanthoma elasticum (PXE) is a genetic disease that causes mineralization of elastic fibers in some tissues. The most common problems arise in the skin and eyes, and later in blood vessels in the form of premature atherosclerosis. PXE is caused by autosomal recessive mutations in the ABCC6 gene on the short arm of chromosome 16 (16p13.1).

Eales disease is a type of obliterative vasculopathy, also known as angiopathia retinae juvenilis, periphlebitis retinae or primary perivasculitis of the retina. It was first described by the British ophthalmologist Henry Eales (1852–1913) in 1880 and is a rare ocular disease characterized by inflammation and possible blockage of retinal blood vessels, abnormal growth of new blood vessels (neovascularization), and recurrent retinal and vitreal hemorrhages.

<span class="mw-page-title-main">Presumed ocular histoplasmosis syndrome</span> Medical condition

Presumed ocular histoplasmosis syndrome (POHS) is a syndrome affecting the eye, which is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the optic disc and maculopathy.

<span class="mw-page-title-main">Optic disc drusen</span> Medical condition

Optic disc drusen (ODD) are globules of mucoproteins and mucopolysaccharides that progressively calcify in the optic disc. They are thought to be the remnants of the axonal transport system of degenerated retinal ganglion cells. ODD have also been referred to as congenitally elevated or anomalous discs, pseudopapilledema, pseudoneuritis, buried disc drusen, and disc hyaline bodies.

<span class="mw-page-title-main">Intermediate uveitis</span> Medical condition

Intermediate uveitis is a form of uveitis localized to the vitreous and peripheral retina. Primary sites of inflammation include the vitreous of which other such entities as pars planitis, posterior cyclitis, and hyalitis are encompassed. Intermediate uveitis may either be an isolated eye disease or associated with the development of a systemic disease such as multiple sclerosis or sarcoidosis. As such, intermediate uveitis may be the first expression of a systemic condition. Infectious causes of intermediate uveitis include Epstein–Barr virus infection, Lyme disease, HTLV-1 virus infection, cat scratch disease, and hepatitis C.

<span class="mw-page-title-main">Acute posterior multifocal placoid pigment epitheliopathy</span> Eye disease causing lesions in retina

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an acquired inflammatory uveitis that belongs to the heterogenous group of white dot syndromes in which light-coloured (yellowish-white) lesions begin to form in the macular area of the retina. Early in the course of the disease, the lesions cause acute and marked vision loss that ranges from mild to severe but is usually transient in nature. APMPPE is classified as an inflammatory disorder that is usually bilateral and acute in onset but self-limiting. The lesions leave behind some pigmentation, but visual acuity eventually improves even without any treatment.

<span class="mw-page-title-main">Fundus photography</span> Medical imaging of the eyes

Fundus photography involves photographing the rear of an eye, also known as the fundus. Specialized fundus cameras consisting of an intricate microscope attached to a flash enabled camera are used in fundus photography. The main structures that can be visualized on a fundus photo are the central and peripheral retina, optic disc and macula. Fundus photography can be performed with colored filters, or with specialized dyes including fluorescein and indocyanine green.

<span class="mw-page-title-main">Choroidal neovascularization</span> Creation of new blood vessels in the choroid layer of the eye

Choroidal neovascularization (CNV) is the creation of new blood vessels in the choroid layer of the eye. Choroidal neovascularization is a common cause of neovascular degenerative maculopathy commonly exacerbated by extreme myopia, malignant myopic degeneration, or age-related developments.

<span class="mw-page-title-main">Maculopathy</span> Term for pathological conditions effecting the macula

A maculopathy is any pathological condition of the macula, an area at the centre of the retina that is associated with highly sensitive, accurate vision.

<span class="mw-page-title-main">Intraocular hemorrhage</span> Medical condition

Intraocular hemorrhage is bleeding inside the eye. Bleeding can occur from any structure of the eye where there is vasculature or blood flow, including the anterior chamber, vitreous cavity, retina, choroid, suprachoroidal space, or optic disc.

Jeffrey W. Berger was an American vitreoretinal surgeon and engineer.

Joan Whitten Miller is a Canadian-American ophthalmologist and scientist who has made notable contributions to the treatment and understanding of eye disorders. She is credited for developing photodynamic therapy (PDT) with verteporfin (Visudyne), the first pharmacologic therapy for retinal disease. She also co-discovered the role of vascular endothelial growth factor (VEGF) in eye disease and demonstrated the therapeutic potential of VEGF inhibitors, forming the scientific basis of anti-VEGF therapy for age-related macular degeneration (AMD), diabetic retinopathy, and related conditions.

<span class="mw-page-title-main">Indocyanine green angiography</span> Diagnostic procedure

Indocyanine green angiography (ICGA) is a diagnostic procedure used to examine choroidal blood flow and associated pathology. Indocyanine green (ICG) is a water soluble cyanine dye which shows fluorescence in near-infrared (790–805 nm) range, with peak spectral absorption of 800-810 nm in blood. The near infrared light used in ICGA penetrates ocular pigments such as melanin and xanthophyll, as well as exudates and thin layers of sub-retinal vessels. Age-related macular degeneration is the third main cause of blindness worldwide, and it is the leading cause of blindness in industrialized countries. Indocyanine green angiography is widely used to study choroidal neovascularization in patients with exudative age-related macular degeneration. In nonexudative AMD, ICGA is used in classification of drusen and associated subretinal deposits.

Sickle cell retinopathy can be defined as retinal changes due to blood vessel damage in the eye of a person with a background of sickle cell disease. It can likely progress to loss of vision in late stages due to vitreous hemorrhage or retinal detachment. Sickle cell disease is a structural red blood cell disorder leading to consequences in multiple systems. It is characterized by chronic red blood cell destruction, vascular injury, and tissue ischemia causing damage to the brain, eyes, heart, lungs, kidneys, spleen, and musculoskeletal system.

Polypoidal choroidal vasculopathy (PCV) is an eye disease primarily affecting the choroid. It may cause sudden blurring of vision or a scotoma in the central field of vision. Since Indocyanine green angiography gives better imaging of choroidal structures, it is more preferred in diagnosing PCV. Treatment options of PCV include careful observation, photodynamic therapy, thermal laser, intravitreal injection of anti-VEGF therapy, or combination therapy.

Richard Frederick Spaide is an American ophthalmologist and retinal specialist known for his work in retinal diseases and advancements in ocular imaging techniques.

References

  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.