Angioid streaks, also called Knapp streaks or Knapp striae, are small breaks in

Bruch's membrane Bruch's membrane 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. Layers Bruch's membrane consists of five l ...

, an elastic tissue containing membrane of the

retina The retina (from la, rete "net") is the innermost, light-sensitive layer of tissue of the eye of most vertebrates and some molluscs. The optics of the eye create a focused two-dimensional image of the visual world on the retina, which then ...

that may become calcified and crack. Up to 50% of angioid streak cases are idiopathic. It may occur secondary to blunt trauma, or it may be associated with many systemic diseases. The condition is usually asymptomatic, but decrease in vision may occur due to choroidal neovascularization.

Clinical features

Angioid streaks are often associated with

pseudoxanthoma elasticum 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 cau ...

, but have been found to occur in conjunction with other disorders, including Paget's disease,

sickle cell disease Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blo ...

and Ehlers–Danlos syndrome. These streaks can have a negative impact on vision due to

choroid The choroid, also known as the choroidea or choroid coat, is a part of the uvea, the vascular layer of the eye, and contains connective tissues, and lies between the retina and the sclera. The human choroid is thickest at the far extreme rear ...

neovascularization Neovascularization is the natural formation of new blood vessels ('' neo-'' + ''vascular'' + '' -ization''), usually in the form of functional microvascular networks, capable of perfusion by red blood cells, that form to serve as collateral circu ...

or choroidal rupture. Also,

vision Vision, Visions, or The Vision may refer to: Perception Optical perception * Visual perception, the sense of sight * Visual system, the physical mechanism of eyesight * Computer vision, a field dealing with how computers can be made to gain un ...

can be impaired if the streaks progress to the

fovea Fovea () (Latin for "pit"; plural foveae ) is a term in anatomy. It refers to a pit or depression in a structure. Human anatomy *Fovea centralis of the retina * Fovea buccalis or Dimple * Fovea of the femoral head * Trochlear fovea of the fr ...

and damage the retinal pigment

epithelium Epithelium or epithelial tissue is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. It is a thin, continuous, protective layer of compactly packed cells with a little intercellul ...

Signs

Retinal fundus examination may reveal grey or dark red spoke like lesions around

optic disk Optics is the branch of physics that studies the behaviour and properties of light, including its interactions with matter and the construction of instruments that use or detect it. Optics usually describes the behaviour of visible, ultravio ...

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.

Diagnosis

The diagnosis is mainly clinical, however fundus fluorescein angiography shows that the streaks appear hyperfluorescent (window defect) in the early phase.

Indocyanine green angiography 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, wi ...

can also be used for diagnosing angioid streaks and their associated ocular pathologies.

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. Secondary ocular complications like

choroidal neovascularization 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 (i.e. 'wet' macular degeneration) commonly exacerbated ...

lead to vision loss, and/or

metamorphopsia Metamorphopsia (from , ) is a type of distorted vision in which a grid of straight lines appears wavy and parts of the grid may appear blank. People can first notice they suffer with the condition when looking at mini-blinds in their home. For examp ...

. If choroidal neovascularization is present, treatment options like

anti-VEGF medication Anti–vascular endothelial growth factor therapy, also known as anti-VEGF () therapy or medication, is the use of medications that block vascular endothelial growth factor. This is done in the treatment of certain cancers and in age-related macu ...

laser photocoagulation Laser coagulation or laser photocoagulation surgery is used to treat a number of eye diseases and has become widely used in recent decades. During the procedure, a laser is used to finely cauterize ocular blood vessels to attempt to bring about var ...

photodynamic therapy Photodynamic therapy (PDT) is a form of phototherapy involving light and a photosensitizing chemical substance, used in conjunction with molecular oxygen to elicit cell death (phototoxicity). PDT is popularly used in treating acne. It is used cl ...

, transpupillary thermotherapy, macular translocation surgery etc. may be needed.

History

They were first described by

Robert Walter Doyne Robert Walter Doyne (1857–1916) was an Anglo-Irish ophthalmologist. He was born in Monart, County Wexford, Ireland, second son of the Reverend Philip Walter Doyne (died 1861), vicar of Monart, and Emily Sophia Richards, daughter of John Godda ...

in 1889 in a patient with

l hemorrhages. In 1892,

ophthalmologist Ophthalmology ( ) is a surgery, surgical subspecialty within medicine that deals with the diagnosis and treatment of eye disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Followin ...

Hermann Jakob Knapp called them "angioid streaks" because of their resemblance to

blood vessels The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away f ...

. From

histopathological Histopathology (compound of three Greek words: ''histos'' "tissue", πάθος ''pathos'' "suffering", and -λογία ''-logia'' "study of") refers to the microscopic examination of tissue in order to study the manifestations of disease. Spec ...

research in the 1930s, they were discovered to be caused by changes at the level of Bruch's

membrane A membrane is a selective barrier; it allows some things to pass through but stops others. Such things may be molecules, ions, or other small particles. Membranes can be generally classified into synthetic membranes and biological membranes. B ...

. Presently, it is believed that its

pathology Pathology is the study of the causes and effects of disease or injury. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in ...

may be a combination of elastic degeneration of Bruch's membrane,

iron Iron () is a chemical element with symbol Fe (from la, ferrum) and atomic number 26. It is a metal that belongs to the first transition series and group 8 of the periodic table. It is, by mass, the most common element on Earth, right in f ...

deposition in elastic fibers from

hemolysis Hemolysis or haemolysis (), also known by several other names, is the rupturing (lysis) of red blood cells (erythrocytes) and the release of their contents (cytoplasm) into surrounding fluid (e.g. blood plasma). Hemolysis may occur in vivo o ...

with secondary mineralization, and impaired nutrition due to stasis and small vessel

occlusion Occlusion may refer to: Health and fitness * Occlusion (dentistry), the manner in which the upper and lower teeth come together when the mouth is closed * Occlusion miliaria, a skin condition * Occlusive dressing, an air- and water-tight trauma ...

External links

eMedicine article on Angioid streaks

References

Angioid streaks 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. Up to 50% of angioid streak cases are idiopathic. It may oc ...

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