Angioid streaks, clinical course, complications, and. There are many causes of mnv including angioid streaks, choroidal rupture, idiopathic, ocular histoplasmosis, and agerelated macular degeneration. Angioid streaks are linear cracks in bruchs membrane. These breaks in bruchs membrane typically radiate out from the optic nerve and can be hard to see, so if you dont look for them, you might mistakenly diagnose amd. However, lacquer cracks are a classical symptom of myopic degeneration and while your condition may remain stable for many years, the odds are more in favor of problems as you age. Improving differential diagnosis of angioid streaks. Typical features of degenerative myopia demonstrating. The reported frequencies of angioid streaks in pseudoxanthoma elasticum vary tremendously with the era of the observer, the specialty dermatology, genetics, ophthalmology, etc. Jul 22, 2019 in 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to trauma.
Cnv has also been associated with extreme myopia or malignant myopic degeneration, where in choroidal neovascularization occurs primarily in the presence of cracks within the retinal specifically. Angioid streaks definition of angioid streaks by the free. In 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to trauma. Implications of lacquer cracks for perforating vessels in pathologic myopia francesco bandello, md based on topographic correspondence visible only with octa, he speculates that perforating vessels may be consequential for choroidal neovascularization. Angioid streaks are breaks in the degenerated and mineralized bruchs membrane which typically form around the optic disc and radiate from the optic disc. Angioid streaks are irregular, jagged, radiating lines that surround the optic disc and extend outward radially. If you have angioid streaks in one or both eyes, it is possible to remain asymptomatic and maintain perfect vision for a long time. The choroid may be thinner in eyes with angioid streaks and cnv compared to angioid streaks without cnv. Optical coherence tomography oct reveals the retinalsubretinalsubrpe changes in cnv, and also hyperreflectivity at the bruchs membrane level due to calcification. Clinicians must determine the presence of such etiologies, as well as address the ocular complications. The beginning stages of posterior staphylomas are very difficult to discern on physical exam for runofthemill retinal specialists or general ophthalmologists. Unlike other similar presentations such as lacquer cracks, they dont radiate from the center of the posterior pole. We report herein a patient with angioid streaks who developed choroidal.
Conversely, lacquer cracks are found in more than 80. Angioid streaks with cnvm oct re retina image bank. Sep 20, 2014 optical coherence tomography oct detects the breaks in bruchs membrane and helps in detecting and monitoring associated abnormalities such as cnv. Color photographs showed areas of yellowish opacification that obscured. As the vitreous degenerates, there is liquefaction of the vitreous gel, known as synchisis, resulting in small pockets of liquefied vitreous within the firmer vitreous. What will be seen on oct of a pt w solar retinopathy. While myopic lacquer cracks are short and usually found in a reticular. Additionally, genetic testing obtained showed that the patient was both abcc6 and enpp1 positive diagnosis. Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid following blunt trauma. Hermann jacob knapp coined the term because he thought their presentation was suggestive of blood vessels. Angioid streaks are broad, irregular, red to brown to grey lines which radiate from the area around the optic nerve head under the retinas of individuals with pseudoxanthoma elasticum pxe.
Progressive symmetric vertical macular wide angioid streaklike. With the presence of angioid streaks emanating from the bilateral optic discs, along with the characteristic rpe changes in the posterior pole and pathognomonic skin findings on the patients neck, the. Angioid streaks, also known as knapp striae, are irregular jagged dehiscences in the mineralized, degenerated, brittle bruch membrane that typically form alongside force lines exerted by intrinsic and extrinsic ocular muscles that radiate in a centrifugal pattern emanating from the. Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid. Patients with angioid streaks associated with pseudoxanthoma elasticum have a very high frequency of cnv. Full text lacquer cracks observed in peripheral fundus of. In addition, mild trauma to the eye may cause the choroid to rupture at these areas, leading to submacular hemorrhage. Lacquer cracks are seen in almost 5% of highly myopic eyes, and the reported incidence of cnvm associated with these lesions is as high as 10%. Jun 01, 2007 the pathologies that we describe here are angioid streaks, optic nerve drusen, radiation retinopathy, idiopathic perifoveal telangiectasia, and lacquer cracks. Angioid streaks conditionkeywords angioid streaks, choroidal neovascularization cnv photographer neha goel imaging device optical coherence tomography system rtvue description horizontal oct scan through the right macula. Lacquers cracks a degenerative finding noted in pathological. Apr, 2020 angioid streaks are bilateral, narrow, irregular lines deep to the retina configured in a radiating fashion emanating from the optic disc, which result from breaks in a weakened bruchs membrane.
In many cases, however, these fine cracks in the membrane expand, leading to visual impairment. With the presence of angioid streaks emanating from the bilateral optic discs, along with the characteristic rpe changes in the posterior pole and pathognomonic skin findings on the patients neck, the patients presentation is highly suggestive. Angioid streaks are visible irregular cracklike dehiscences in bruchs membrane associated with atrophic degeneration of the overlying retinal pigmented epithelium. Angioid streaks usually do not extend past the equator. Retinal physician easily misdiagnosed retinal entities. Angioid streaks usually emanate from the disc, tend to be straighter, and are reddish in color.
Generally, they taper and fade a few millimeters away from the optic disc. An update on the ocular phenotype in patients with. Learn vocabulary, terms, and more with flashcards, games, and other study tools. These streaks can have a negative impact on vision due to choroidal neovascularization or choroidal rupture. Lacquer crack means that there is break in bruchs membrane it is a significant finding as you are at risk of getting cnvm choroidal neovascular membranes, which means new blood vessels will start forming. We report a case with lacquer cracks observed in the peripheral fundus. Angioid streaks are evident in fundoscopy a few millimetres from the optic disc and rarely occur in the periphery of the posterior pole. Other associated findings in patients with angioid streaks may include peau dorange or leopardskin spotting, consisting of speckled yellowish mottling of the posterior pole mostly apparent in the temporal macula.
Lacquer cracks are spontaneous ruptures of the elastic lamina of bruchs membrane that appear yellowishwhite and are usually located in the posterior pole. Color photographs were evaluated directly as were the component red and green channels. Although angioid streaks can be idiopathic, there is often an underlying cause, which the driving force when managing these patients. Choroidal ruptures, similar distribution, color, and fluoresceinangiographic appearance to lc, but are caused by a traumaticevent.
Also break or absence of choriocapillaris beneath the angioid streaks as associations. These include the classic signs of angioid streaks, peau dorange, comet lesions. Jan 11, 20 angioid streaks photographer alfredo gomezleal, md top and gerardo garciaaguirre, md bottom description top. Pseudoxanthoma elasticum pxe is a rare genetic disorder characterized by elastorrhexia, or progressive calcification and fragmentation, of elastic fibers primarily affecting the skin, the retina, and the cardiovascular system. Patients with degenerative myopia develop floaters and posterior vitreous detachment earlier in life. Clinicians should always be on the lookout for conditions that can mimic angioid streaks.
Angioid streaks represent a rare ophthalmic condition that has very specific ophthalmoscopic. Angioid streaks are often associated with pseudoxanthoma elasticum, but have been found to occur in conjunction with other disorders, including pagets disease, sickle cell disease and ehlersdanlos syndrome. Improving differential diagnosis of angioid streaks retinal physician. Optical coherence tomography images were evaluated for reflectivity pattern of the band corresponding to the retinal pigment epitheliumbruch membrane complex. Eventually the mineralization of the elastic fibers, loss of elasticity and enhanced calcification lead to cracks in bruchs membrane called angioid streaks that radiate out from the optic nerve and surround it concentrically as brownish. Lacquer cracks observed in peripheral fundus of eyes with high myopia mitsuki suga, kosei shinohara, kyoko ohnomatsui department of ophthalmology and visual science, tokyo medical and dental university, tokyo, japan abstract. Angioid streaks cracklike dehiscences of bruch membrane o with deposition of calcium, magnesium, or iron salts due to disturbed metabolism case presentation s differential diagnoses o lacquer cracks o choroidal rupture choroidal neovascular membrane cnvm diagnostic tests.
Bruchs membrane below the rpe, which is thickened, elevated and fractured. Progressive symmetric vertical macular wide angioid streaklike lacquer crack article pdf available in international medical case reports journal 9issue 1. Fluorescein angiography and oct scanning help diagnosis the presence of mnv. Angioid definition of angioid by medical dictionary. Angioid streaks usually present as linear gray or dark red lines with irregular serrated edges lying beneath normal retinal blood vessels. These streaks or striations are typically bilateral and vary in width from 50 to 100 microns, barely detectable by ophthalmoscopy, to 34 times the average. Secondary choroidal neovascularization is also possible. Angioid streaks differential diagnoses medscape emedicine. The pathologies that we describe here are angioid streaks, optic nerve drusen, radiation retinopathy, idiopathic perifoveal telangiectasia, and lacquer cracks. Lacquer crack started in the right eye and became evident 1 year later in the left eye.
Recently near infrared reflectance imaging studies using diode of 820 nm have shown very high sensitivity in detection of angioid streaks. Pdf progressive symmetric vertical macular wide angioid. Peripheral lacquer cracks as an early finding in pathological. Angioid streaks synonyms, angioid streaks pronunciation, angioid streaks translation, english dictionary definition of angioid streaks. Angioid streaks, also known as knapp striae, are irregular jagged dehiscences in the mineralized, degenerated, brittle bruch membrane that typically form alongside force lines exerted by intrinsic and extrinsic ocular muscles that radiate in a centrifugal pattern.
In 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to. Oct 20, 2017 the streaks represent cracks and fissures in the calcified bruch membrane. The aim of this study was to characterize peau dorange and angioid streaks, characteristic findings in eyes of patients with pseudoxanthoma elasticum, by examining fundus photography and optical coherence tomography imaging. Angioid streaks light microscopy and oct retina image bank. Ophthalmologists should be aware that there are numerous systemic diseases associated with angioid streaks, the most common being pseudoxanthoma elasticum. Figure 6 highdefinition horizontal oct scan of left optic disc and. A 37yearold patient with bilateral high myopia who visited our clinic was examined by. Angioid streaks typically radiate out in a cruciate pattern from an area of peripapillary pigment alterations, although they may circumferentially ring the peripapillary area as well.
Among the most common are exudative and advanced atrophic amd, choroidal rupture, toxoplasmosis, choroidal sclerosis, myopic lacquer cracks, histoplasmosis, retinal vasculitis and papillitis and traumatic retinal hemorrhage with or without cnv figures 24. There are a number of potential causes of angioid streaks, including pseudoxanthoma elasticum, ehlerdanlos syndrome, pagets disease, sickle cell disease, and other hemoglobinopathies paton, 1972. Wet amd lookalikes american academy of ophthalmology. Angioid streak may be associated with choroidal neovascularization cnv. Knapp, md, a germanborn american ophthalmologist, as the conversion of extravasated blood into streaks of dark brown or black pigment in the retina.
Progressive symmetric vertical macular wide angioid streak. Nov 01, 2011 the name angioid streaks was coined in 1892 by herman j. Pxe affects the retina through a dimpling of the bruch membrane. Angioid streaks knapp streaks statpearls ncbi bookshelf. Ivfa will show hyperfluorescence as the fluorescein leaks through bruchs membrane, highlighting these cracks. Dec 15, 2019 angioid streaks are bilateral blood vessellike cracks emanating from the optic nerve. Lacquer cracks must be differentiated from 2 similar diseases of bruch membrane, namely angioid streaks and choroidal ruptures. Multiple areas of pigment epithelium and choriocapillaris atrophy involving the macular area. These breaks are usually linear and hence named angioid or like blood vessels. Choroidal ruptures, on the other hand, share with lacquer cracks. Calcification of bruchs membrane may be seen on oct images. Aug 08, 2018 cnv has also been associated with extreme myopia or malignant myopic degeneration, where in choroidal neovascularization occurs primarily in the presence of cracks within the retinal specifically. They are caused by a breakdown in the collagen and elastic lamina of bruchs membrane.
Angioid streaks are bilateral, narrow, irregular lines deep to the retina configured in a radiating fashion emanating from the optic disc, which result from breaks in a weakened bruchs membrane. At least 50% of all individuals with pxe demonstrate angioid streaks. We report an unusual case of bilateral vertical lacquer crack with no history of ocular trauma and with progressive marked enlargement and consequent. Angioid streaks present as radial spokes emanating from the optic nerve head and seldom extend beyond the equator of the orbit.
Kyoko ohnomatsui department of ophthalmology and visual science, tokyo medical and dental university, tokyo, japan abstract. Lacquer cracks are often mistaken for angioid streaks. Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid following. Angioid streaks definition of angioid streaks by the. Pseudoxanthoma elasticum march 2018 illinois retina. Progressive symmetric vertical macular wide angioid streaklike lacquer crack ahmad m mansour1,2 1department of ophthalmology, american university of beirut, 2department of ophthalmology, rafic hariri university hospital, beirut, lebanon purpose. Angioid streaks are bilateral, narrow, irregular lines deep to the retina configured in a. Lacquer cracks vs angioid streak vs choroidal rupture all three diseased statesof bruchs membrane angioid streaks emanate radially from disc, are straighter,and are reddish in color. A 37yearold patient with bilateral high myopia who visited our clinic.
Angioid streaks may be idiopathic or associated with pseudoxanthoma elasticum, ehlers danlos syndrome, sickle cell disease, acromegaly, pagets. This ocular change is generally bilateral and is often noted several years after the onset of cutaneous lesions. The enface oct may reveal the areas of angioid streaks. Octangiogram may be used to demonstrate the cnv in the angioid streak. However, there have been no reports of any angioid streak patients in whom choroidal neovascularization occurred after vitrectomy surgery. A ngioid streaksdefects in bruchs membranecan cause visual disturbances in multiple ways. Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid following blunt. The streaks run a convoluted course and tend to terminate abruptly. Angioid streaks were first discovered in 1889 by robert walter doyne. Lacquer cracks observed in peripheral fundus of eyes with. Patients with angioid streaks are prone to developing subretinal hemorrhage after ocular or head injury due to the brittleness of bruchs membrane.
The effects of angioid streaks are much more severe if the cracks extend into the center of the retina. Vitreous vitreous synchisis and syneresis vitreous degeneration is a physiologic process that occurs with age and may be accelerated in eyes with longer axial length. Oct scan of an angioid streak not of the same patient showing elevation and fracture of the rpe. Look for lacquer cracks in high myopes for early warning of. We report herein a patient with angioid streaks who developed choroidal neovascularization after vitrectomy. Choroidal ruptures, on the other hand, share with lacquer cracks similar distribution, color, and fluorescein angiographic. Look for lacquer cracks in high myopes for early warning. The streaks intercommunicate in a ringlike fashion around the optic disc in 27% of cases and radiate outward in a tapering fashion from the peripapillary area in 73% of patients. Multimodal imaging including spectraldomain optical.
Jan 15, 2018 clinicians should always be on the lookout for conditions that can mimic angioid streaks. Three diseases that involve bruchs membrane give somewhat similar fundus appearance in different clinical settings. The condition was first described by the french dermatologist rigal in 1881 and later named by darier in 1896, who. Although uncommon, angioid streaks often have a systemic cause and.
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