Angioid Streaks And

Factsheet

our public information line on 0845 129 5001 or log on to our public information line on 0845 129 5001 or log on www.eyecaretrust.org.uk The Eyecare Trust is a registered charity that exists to promote charity that exists to promote is a registered Trust The Eyecare information call of all aspects of eye health. For more awareness Angioid Streaks and PXE Streaks Angioid fundus photography documents their appearance and serves as baseline for fundus photography documents their appearance and serves recognition future observations. Retinal fluorescein angiography will enhance Angioid streaks are not present in newborns or infants but, in some Angioid streaks are not present in newborns or infants but, 10 years. Retinal individuals and families can, be identified before the age of observations of individuals with PXE followed sequentially over years and observations of individuals with PXE followed sequentially decades. all family members are scrutinized. At least 50% of all individuals with PXE all family members are scrutinized. At least 50% of all individuals typically demonstrate angioid streaks. However, that "prevalence rate" of diligent reflects a "single encounter" and does not reflect the analysis vary tremendously with the era of the observer, the specialty (dermatology, vary tremendously with the era of the observer, the specialty individuals and genetics, ophthalmology, etc.), and the diligence with which year as Strandberg described them independently. elasticum The reported frequencies of angioid streaks in pseudoxanthoma Subsequently, in 1929, Groenblad, also an ophthalmologist, related the Subsequently, in 1929, Groenblad, with Pseudoxanthoma Elasticum, in the same occurrence of angioid streaks "the conversion of extravasated blood into streaks of dark brown or black "the conversion of extravasated pigment in the retina . . ." . Dr. Herman J. Knapp, a German-born American ophthalmologist, who coined Dr. Herman J. Knapp, a German-born streaks" in 1892, felt that they represented and introduced the term "angioid correspond in most places and are probably due to rupture of the pigment correspond in most places and layer of the retina . . .". especially around the discs, are irregular, jagged lines, nearly all deeply especially around the discs, are and jags in their outline exactly pigmented . . .". "The irregularities The first description of angioid streaks was provided in 1889 by Dr. Robert The first description of angioid He reported a male who had received W. Doyne, an English ophthalmologist. throughout the choroid of both eyes, injuries to both eyes: "In all directions and angular than normal retinal blood vessels. and angular than irregular, serrated, and occasionally ill-defined, particularly when they are and occasionally ill-defined, irregular, serrated, Their number and extent are blood vessels in that region. smaller than the tends to be more sinuous their age of onset. Their course variable, as are by ophthalmoscopy, to 3-4 times the average retinal vascular width, to 3-4 times the average by ophthalmoscopy, observer. Their borders are to the casual ophthalmic dramatically visible from the area around the optic nerve head under the retinas of individuals the optic nerve head under from the area around streaks or striations are elasticum (PXE). These with pseudoxanthoma 100 microns, barely detectable and vary in width from 50 to typically bilateral Angioid streaks are broad, irregular, red to brown to grey lines which radiate grey lines which red to brown to irregular, streaks are broad, Angioid

Factsheet

our public information line on 0845 129 5001 or log on to our public information line on 0845 129 5001 or log on www.eyecaretrust.org.uk The Eyecare Trust is a registered charity that exists to promote charity that exists to promote is a registered Trust The Eyecare information call of all aspects of eye health. For more awareness Angioid Streaks and PXE Streaks Angioid Other retinal changes in association with peau d'orange and angioid streaks Other retinal changes in association with peau d'orange and patches (which include focal hemorrhages with or without scarring, salmon the presence of peau d'orange even in the absence of angioid streaks. the presence of peau d'orange even in the absence of angioid throughout the posterior pole and out to the equator. Peau d'orange does not throughout the posterior pole and out to the equator. Peau when readily affect visual acuity, even when the fovea is involved. However, investigated for identifiable in one individual, siblings and parents should be description of the fine, relatively symmetrical, drusen-like, flat, yellow, and description of the fine, relatively symmetrical, drusen-like, flat, extending occasionally confluent lesions in the retinal pigment epithelium, The most common retinal feature associated with angioid streaks is the The most common retinal feature associated with angioid streaks which classically presence of "peau d'orange", a mottled fundus appearance persists as a precedes the appearance of the streaks. The French term surveillance. indicative of an underlying systemic illness, even if the specific disorder indicative of an underlying systemic accurately at the time that the angioid cannot be identified or defined changes are first noted. The patient must remain under continuing disease", but they have also been seen in younger individuals with PXE who disease", but they have also been of the disease but develop them have no (current) cutaneous manifestations of angioid streaks should be considered later. Therefore, the presence disease of bone, acromegaly and other pituitary disorders, and familial disease of bone, acromegaly angioid streaks are noted with "no other hyperphosphatemia. Occasionally, Angioid streaks are seen in a number of clinical disorders in addition to Angioid streaks are seen in a sickle cell anaemia, thalassemia, Paget's Pseudoxanthoma Elasticum: are noted under the retinal pigment epithelium. The streaks present as are noted under the retinal pigment of Bruch's membrane. ruptures in this elastic lamina Histopathology shows premature basophilia and staining with increased Histopathology shows premature much as seen in older eyes in general. Both amounts of calcium and iron, basement membrane and Bruch's membrane thinning and thickening of the retinal pigment epithelium and the choroid. retinal pigment cardiovascular systems is unknown, so the pathogenesis of angioid streaks systems is unknown, so the pathogenesis cardiovascular in information suggests a defect debate. The best current is similarly in much the the elastic layer between in Bruch's membrane, elastic fibres, particularly Just as the pathogenesis of PXE in the skin, gastrointestinal system, and of PXE in the skin, Just as the pathogenesis the retinal epithelium is thinned or atrophic, thereby permitting their ready is thinned or atrophic, the retinal epithelium of monochromatic contrast-enhanced presentation recognition in the photography. of the angioid streaks, because they are typically hyperfluorescent wherever are typically hyperfluorescent because they streaks, of the angioid

Factsheet

our public information line on 0845 129 5001 or log on to our public information line on 0845 129 5001 or log on www.eyecaretrust.org.uk The Eyecare Trust is a registered charity that exists to promote charity that exists to promote is a registered Trust The Eyecare information call of all aspects of eye health. For more awareness Angioid Streaks and PXE Streaks Angioid Other historical ocular complications of PXE include "blue sclerae" and Other historical ocular complications of PXE include "blue sclerae" have a slightly higher risk than the general population for retinal vascular have a slightly higher risk than the general population for retinal occlusive disease and non-arteritic ischemic optic neuropathy. Individuals with PXE also have systemic vascular disease involving large Individuals with PXE also have systemic vascular disease involving they may arteries, such as the carotid artery in the neck. For this reason, "central areolar choroidal sclerosis" and sometimes (incorrectly) called "central areolar choroidal sclerosis" and sometimes (incorrectly) "age-related macular degeneration". "chorioretinitis" are probably misinterpretations of the disciform process. "chorioretinitis" are probably misinterpretations of the disciform streaks will Occasional older individuals with peau d'orange and angioid simulating develop geographic atrophy of the retinal pigment epithelium, Other historical references to visual impairment in PXE from "choroiditis" or Other historical references to visual impairment in PXE from recurrences, have yielded a better outcome in many individuals. However, recurrences, have yielded a better because of the widespread defects the risk of recurrence is real, presumably membrane interface. in the pigment epithelium Bruch's outlook. More recent technologies, to identify fully the extent of neovascular outlook. More recent technologies, angiography and rapid retreatment of membranes with sequential fluorescein causes of disciform disease. Historical initial attempts to apply laser causes of disciform disease. Historical or red wavelengths met with limited photocoagulation with either green for this complication a variable and poor success, giving laser treatment d'orange is disciform degeneration of the central visual area. The mechanism d'orange is disciform degeneration membrane barrier and the of disruption of the pigment epithelium-Bruch's under the retina is not different from other invasion of new blood vessel The most visually devastating complication of angioid streaks and peau The most visually devastating dominantly inherited PXE. The usual complications of intrapapillary drusen dominantly inherited PXE. The juxtapapillary disciform disease, visual field including juxtapapillary bleeding, impairment, can occur in this association. deficits, and even advanced visual Intrapapillary drusen, or drusen of the optic nerve head, have been seen in head, have been seen or drusen of the optic nerve Intrapapillary drusen, families, typically but not universally with some individuals and in some epithelium simulating disciform degeneration. epithelium simulating subpigment epithelial hemorrhage, or a focal disciform scar with spontaneous hemorrhage, or a focal subpigment epithelial under the retina, near the peripheral hemorrhages resolution). Occasionally, of the retinal pigment evolve to discoid metaplasia ora serrata, may metaplasia of the pigment epithelium following focal or blot hemorrhages), pigment epithelium following metaplasia of the typically 150-300 microns in white or yellow-white foci and "pearls" (dead hemorrhage, either the result of subretinal size, again representing typically represent a healing subretinal hemorrhage), black dots, (the result of black dots, (the hemorrhage), subretinal represent a healing typically

Factsheet