Improving differential diagnosis of angioid streaks retinal physician. The choroid may be thinner in eyes with angioid streaks and cnv compared to angioid streaks without cnv. 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. 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. Ivfa will show hyperfluorescence as the fluorescein leaks through bruchs membrane, highlighting these cracks. In addition, mild trauma to the eye may cause the choroid to rupture at these areas, leading to submacular hemorrhage.
Ophthalmologists should be aware that there are numerous systemic diseases associated with angioid streaks, the most common being pseudoxanthoma elasticum. Additionally, genetic testing obtained showed that the patient was both abcc6 and enpp1 positive diagnosis. Fluorescein angiography and oct scanning help diagnosis the presence of mnv. Pxe affects the retina through a dimpling of the bruch membrane. A 37yearold patient with bilateral high myopia who visited our clinic. Jan 11, 20 angioid streaks photographer alfredo gomezleal, md top and gerardo garciaaguirre, md bottom description top. This ocular change is generally bilateral and is often noted several years after the onset of cutaneous lesions. Angioid streaks definition of angioid streaks by the. 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. Unlike other similar presentations such as lacquer cracks, they dont radiate from the center of the posterior pole. Angioid streaks present as radial spokes emanating from the optic nerve head and seldom extend beyond the equator of the orbit.
Peripheral lacquer cracks as an early finding in pathological. Improving differential diagnosis of angioid streaks. Knapp, md, a germanborn american ophthalmologist, as the conversion of extravasated blood into streaks of dark brown or black pigment in the retina. Angioid streaks were first discovered in 1889 by robert walter doyne. Retinal physician easily misdiagnosed retinal entities. Angioid streaks are evident in fundoscopy a few millimetres from the optic disc and rarely occur in the periphery of the posterior pole. Choroidal ruptures, on the other hand, share with lacquer cracks. These breaks are usually linear and hence named angioid or like blood vessels. 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. Figure 6 highdefinition horizontal oct scan of left optic disc and. Patients with angioid streaks associated with pseudoxanthoma elasticum have a very high frequency of cnv. Angioid streaks are breaks in the degenerated and mineralized bruchs membrane which typically form around the optic disc and radiate from the optic disc.
Secondary choroidal neovascularization is also possible. Angioid streaks are irregular, jagged, radiating lines that surround the optic disc and extend outward radially. Wet amd lookalikes american academy of ophthalmology. Conversely, lacquer cracks are found in more than 80. Typical features of degenerative myopia demonstrating. They are caused by a breakdown in the collagen and elastic lamina of bruchs membrane. Lacquer cracks observed in peripheral fundus of eyes with. Angioid streaks are linear cracks in bruchs membrane. A ngioid streaksdefects in bruchs membranecan cause visual disturbances in multiple ways. Angioid streaks differential diagnoses medscape emedicine. Sep 20, 2014 optical coherence tomography oct detects the breaks in bruchs membrane and helps in detecting and monitoring associated abnormalities such as cnv. Angioid streaks synonyms, angioid streaks pronunciation, angioid streaks translation, english dictionary definition of angioid streaks. Angioid streak may be associated with choroidal neovascularization cnv.
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. What will be seen on oct of a pt w solar retinopathy. Angioid streaks, clinical course, complications, and. In many cases, however, these fine cracks in the membrane expand, leading to visual impairment. Patients with degenerative myopia develop floaters and posterior vitreous detachment earlier in life. A 37yearold patient with bilateral high myopia who visited our clinic was examined by. Jan 15, 2018 clinicians should always be on the lookout for conditions that can mimic angioid streaks. 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. Multiple areas of pigment epithelium and choriocapillaris atrophy involving the macular area. 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. 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. Angioid definition of angioid by medical dictionary. 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. 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.
Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid following. We report a case with lacquer cracks observed in the peripheral fundus. Lacquer cracks must be differentiated from 2 similar diseases of bruch membrane, namely angioid streaks and choroidal ruptures. Lacquer cracks are spontaneous ruptures of the elastic lamina of bruchs membrane that appear yellowishwhite and are usually located in the posterior pole. 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. We report an unusual case of bilateral vertical lacquer crack with no history of ocular trauma and with progressive marked enlargement and consequent.
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. Angioid streaks usually emanate from the disc, tend to be straighter, and are reddish in color. Progressive symmetric vertical macular wide angioid streaklike. Angioid streaks represent a rare ophthalmic condition that has very specific ophthalmoscopic. If you have angioid streaks in one or both eyes, it is possible to remain asymptomatic and maintain perfect vision for a long time. Pseudoxanthoma elasticum march 2018 illinois retina. Lacquer crack started in the right eye and became evident 1 year later in the left eye. These streaks can have a negative impact on vision due to choroidal neovascularization or choroidal rupture.
Generally, they taper and fade a few millimeters away from the optic disc. Full text lacquer cracks observed in peripheral fundus of. The enface oct may reveal the areas of angioid streaks. Multimodal imaging including spectraldomain optical. 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. Octangiogram may be used to demonstrate the cnv in the angioid streak. At least 50% of all individuals with pxe demonstrate angioid streaks. We report herein a patient with angioid streaks who developed choroidal.
Also break or absence of choriocapillaris beneath the angioid streaks as associations. The reported frequencies of angioid streaks in pseudoxanthoma elasticum vary tremendously with the era of the observer, the specialty dermatology, genetics, ophthalmology, etc. However, there have been no reports of any angioid streak patients in whom choroidal neovascularization occurred after vitrectomy surgery. In 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to. Choroidal ruptures, similar distribution, color, and fluoresceinangiographic appearance to lc, but are caused by a traumaticevent. 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. 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. Optical coherence tomography images were evaluated for reflectivity pattern of the band corresponding to the retinal pigment epitheliumbruch membrane complex. 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. 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. In 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to trauma.
Recently near infrared reflectance imaging studies using diode of 820 nm have shown very high sensitivity in detection of angioid streaks. 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. Although uncommon, angioid streaks often have a systemic cause and. Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid following blunt. Angioid streaks are visible irregular cracklike dehiscences in bruchs membrane associated with atrophic degeneration of the overlying retinal pigmented epithelium. Bruchs membrane below the rpe, which is thickened, elevated and fractured. The effects of angioid streaks are much more severe if the cracks extend into the center of the retina. Choroidal rupture is a break in the retinal pigment epithelium, bruchs membrane, and throughout the choroid.
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. 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. Angioid streaks are bilateral, narrow, irregular lines deep to the retina configured in a. Kyoko ohnomatsui department of ophthalmology and visual science, tokyo medical and dental university, tokyo, japan abstract. 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. Progressive symmetric vertical macular wide angioid streak. Oct 20, 2017 the streaks represent cracks and fissures in the calcified bruch membrane. Jun 01, 2007 the pathologies that we describe here are angioid streaks, optic nerve drusen, radiation retinopathy, idiopathic perifoveal telangiectasia, and lacquer cracks. Choroidal ruptures, on the other hand, share with lacquer cracks similar distribution, color, and fluorescein angiographic. Clinicians should always be on the lookout for conditions that can mimic angioid streaks. Angioid streaks usually do not extend past the equator. Color photographs showed areas of yellowish opacification that obscured. The pathologies that we describe here are angioid streaks, optic nerve drusen, radiation retinopathy, idiopathic perifoveal telangiectasia, and lacquer cracks.
We report herein a patient with angioid streaks who developed choroidal neovascularization after vitrectomy. The streaks run a convoluted course and tend to terminate abruptly. Angioid streaks may be idiopathic or associated with pseudoxanthoma elasticum, ehlers danlos syndrome, sickle cell disease, acromegaly, pagets. Look for lacquer cracks in high myopes for early warning of. Angioid streaks usually present as linear gray or dark red lines with irregular serrated edges lying beneath normal retinal blood vessels.
Oct scan of an angioid streak not of the same patient showing elevation and fracture of the rpe. The beginning stages of posterior staphylomas are very difficult to discern on physical exam for runofthemill retinal specialists or general ophthalmologists. 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. The condition was first described by the french dermatologist rigal in 1881 and later named by darier in 1896, who. Learn vocabulary, terms, and more with flashcards, games, and other study tools. 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.
Look for lacquer cracks in high myopes for early warning. 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%. Three diseases that involve bruchs membrane give somewhat similar fundus appearance in different clinical settings. 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 blunt trauma. Choroidal neovascularization in angioid streaks following. Hermann jacob knapp coined the term because he thought their presentation was suggestive of blood vessels. 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. Angioid streaks are present in 85% of patients with pxe.
Nov 01, 2011 the name angioid streaks was coined in 1892 by herman j. Calcification of bruchs membrane may be seen on oct images. Pdf progressive symmetric vertical macular wide angioid. These include the classic signs of angioid streaks, peau dorange, comet lesions. Angioid streaks light microscopy and oct retina image bank. Although angioid streaks can be idiopathic, there is often an underlying cause, which the driving force when managing these patients. Clinicians must determine the presence of such etiologies, as well as address the ocular complications. Angioid streaks knapp streaks statpearls ncbi bookshelf. Dec 15, 2019 angioid streaks are bilateral blood vessellike cracks emanating from the optic nerve. 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.
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. Lacquers cracks a degenerative finding noted in pathological. There are many causes of mnv including angioid streaks, choroidal rupture, idiopathic, ocular histoplasmosis, and agerelated macular degeneration. Angioid streaks definition of angioid streaks by the free. 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. 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. Progressive symmetric vertical macular wide angioid streaklike lacquer crack article pdf available in international medical case reports journal 9issue 1. Optical coherence tomography oct reveals the retinalsubretinalsubrpe changes in cnv, and also hyperreflectivity at the bruchs membrane level due to calcification. 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. Patients with angioid streaks are prone to developing subretinal hemorrhage after ocular or head injury due to the brittleness of bruchs membrane.
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