Angioid streaks usually emanate from the disc, tend to be straighter, and are reddish in color. An update on the ocular phenotype in patients with. Color photographs were evaluated directly as were the component red and green channels. 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.
Macular choroidal volume within the outer circle of the etdrs grid was 6. Generally, they taper and fade a few millimeters away from the optic disc. Most had repeated fundus photography and fluorescein angiography during a followup period of 6 months to 7 years. Ultimately, large areas of chorioretinal atrophy may be found in the posterior pole of the eye. Angioid streaks are linear cracks in bruchs membrane. Jun 11, 2016 angioid streaks ehlersdanlos syndromes ehlersdanlos syndromes eds comprise a heterogeneous group of diseases of the connective tissue. Pseudoxanthoma elasticum pxe pseudoxanthoma elasticum pxe is a systemic disease that can affect the eye. Octangiogram may be used to demonstrate the cnv in the angioid streak. In the latter case, the slightly whitish or opaque fundus reflex may have become.
Patchy atrophy and lacquer cracks predispose to the development of. Fiftysix patients with angioid streaks were evaluated ophthalmologically. Lacquer cracks are typical clinical findings in a highly myopic fundus and should alert the clinician to the possibility of retinal hemorrhage with or without cnvm. Description high myopia is defined as myopia greater than 6 diopters and an axial length of 26. Furthermore, whether or not noninvasive multimodal cslo imaging. Pseudoholes of the macula may result from vitreoretinal traction. 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 have an increased risk of developing hemorrhages associated with trauma because of the rupture of the choroid. Fundus autofluorescence, fluorescein angiography ffa, indocyanine green angiography icga, and optical coherence tomography oct may help identify areas of angioid streaks or their complications fig. However, early in the course, they can mask choroidal fluorescence. In 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to. Angioid streaks arise from an alteration in bruchs membrane due to visible crack like dehiscences in the tissue coupled with an atrophy of the overlying retinal pigment epithelium rpe. Then, owing to cracks in bruchs membrane, pseudoangioid streaks lacquer cracks fig.
Figure 6 highdefinition horizontal oct scan of left optic disc and. Because cnv secondary to angioid streaks is often refractory to treatment, it leads in many cases to poor vision if the cnv involves the macular area. We studied the pattern of break formation in 60 eyes with myopic lacquer cracks, angioid streaks, or traumatic tears in bruchs membrane, using a graphics composition technique and computer analysis of digitized images. Angioid streaks may be idiopathic or associated with.
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. 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 following blunt trauma. We report an unusual case of bilateral vertical lacquer crack with no history of ocular trauma and with progressive marked enlargement and consequent. This is because angioid streaks begin as thickened, calcified areas of bruchs membrane prior to disruption of the choriocapillaris, and loss of rpe and photoreceptors. In groups 3 through 5, choroidal volume of the macula was significantly reduced compared with that of group 1 p a ngioid streaks defects in bruchs membranecan cause visual disturbances in multiple ways.
Fluorescein how do these two principal players measure up in the diagnosis and monitoring of retinal diseases. To stratify the literature on angioid streaks, from pathophysiology to treatment. Ophthalmologists should be aware that there are numerous systemic diseases associated with angioid streaks, the most common being pseudoxanthoma elasticum. The differential diagnosis includes agerelated macular degeneration, choroidal sclerosis, myopic lacquer cracks, traumatic hemorrhage, and choroidal rupture. Progressive symmetric vertical macular wide angioid streak like lacquer crack article pdf available in international medical case reports journal 9issue 1. Patients with angioid streaks associated with pseudoxanthoma elasticum have a very high frequency of cnv. Regarding autofluorescence, angioid streaks appear as fissures with central hypoautofluorescence and with a variable amount of hyperautofluorescence at the borders of the cracks figure 3.
We report a case with lacquer cracks observed in the peripheral fundus. Pseudoxanthoma elasticum march 2018 illinois retina. The authors felt that patients with high myopia really rely on their optometrist at yearly eye exams and these patients want to feel confident in their od. Cnv can complicate angioid streaks in approximately 86 percent of the affected eyes see figure 3 and the streaks are usually bilateral. Peripheral lacquer cracks as an early finding in pathological. Calcification of bruchs membrane may be seen on oct images. Angioid streaks appear as cracks deep to the retinal vascular architecture and originate in a ringlike fashion in the peripapillary area and radiate from the optic nerve head coursing in all directions.
Knapp coined the term angioid streaks because their appearance resembles retinal vasculature. Vander definition irregular, jagged breaks in an abnormal, calcified bruchs membrane. Intravitreal bevacizumab for choroidal neovascularisation. They typically are configured as irregular radiations from a ringlike area of peripapillary pigment mottling. Progressive symmetric vertical macular wide angioid streak like lacquer crack ahmad m mansour1,2 1department of ophthalmology, american university of beirut, 2department of ophthalmology, rafic hariri university hospital, beirut, lebanon purpose. Apr 07, 2004 the streaks generally appear hyperfluorescent. While myopic lacquer cracks are short and usually found in a reticular. 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. Angioid streaks knapp streaks statpearls ncbi bookshelf. They are caused by a breakdown in the collagen and elastic lamina of bruchs membrane. Progressive symmetric vertical macular wide angioid streak.
Introduction angioid streaks were first described by doyne in 1889. The reported frequencies of angioid streaks in pseudoxanthoma elasticum vary tremendously with the era of the observer, the specialty dermatology, genetics, ophthalmology, etc. Look for lacquer cracks in high myopes for early warning. Radiating out from the optic disc in a fashion similar to the retinal vessels. Angioid streaks present as radial spokes emanating from the optic nerve head and seldom extend beyond the equator of the orbit.
Also break or absence of choriocapillaris beneath the angioid streaks as associations. Jun 01, 2007 easily misdiagnosed retinal entities inna zhitomirsky, ba. Rating 2 ratings appears in miscellaneous conditionkeywords myopic macular degeneration, lacquer cracks imaging device fundus camera description lacquer cracks related files. Three diseases that involve bruchs membrane give somewhat similar fundus appearance in different clinical settings. Additionally, genetic testing obtained showed that the patient was both abcc6 and enpp1 positive diagnosis. Pxe can cause a weakening, or cracking, of one of the important layers in the retina bruchs membrane. What is the most likely diagnosis, and what are other considerations. The streaks run a convoluted course and tend to terminate abruptly. These streaks can have a negative impact on vision due to choroidal neovascularization or choroidal rupture.
In many cases, however, these fine cracks in the membrane expand, leading to visual impairment. In 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to trauma. Angioid streaks are associated with several systemic diseases, the most common being pseudoxanthoma elasticum. Angioid definition of angioid by medical dictionary. 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. Fluorescein and indocyanine green angiography reveal the angioid streaks as well as an associated neovascular membrane. Lacquer cracks must be differentiated from 2 similar diseases of bruch membrane, namely angioid streaks and choroidal ruptures. 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. Ophthalmology grand rounds suny downstate medical center. Angioid streaks were first reported in 1889 by doyne and the term angioid streaks was first used by knapp in 1892. Exam 2 disease pics optometry v 745 with mal at indiana.
Myopic cracks, angioid streaks, and traumatic tears in bruch. Angioid streaks are a retinal condition characterized by breaks and cracks in bruchs membrane, the lower layer of the retina that helps feed and nourish the overlying photoreceptor layer. Is optic nerve drusen an optic nerve disease or is it a manifestation of a systemic disease. 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. 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.
It has been reported that 87% of patients with pseudoxanthoma elasticum have associated angioid streaks as. 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. Lacquer cracks are uncommon findings in the posterior pole of highly myopic. Retinal physician easily misdiagnosed retinal entities. The most likely diagnosis is angioid streaks associated with pseudoxanthoma elasticum. Study 117 exam 2 disease pics flashcards from joe s. Angioid streaks appear as bilateral, narrow, jagged lines, deep to the retina. Angioid streaks with cnvm oct re retina image bank. Clinicians should always be on the lookout for conditions that can mimic angioid streaks. Comparison of multimodal retinal imaging in eyes with lacquer cracks and angioid streaks.
Macular disorders that may be confused with an impending or fullthickness macular hole include cystoid macular edema, solitary drusen, epiretinal membranes, lamellar macular hole, and exudative maculopathy, such as central serous retinopathy or agerelated macular degeneration. Secondary cnv in pseudoxanthoma elasticum has been associated with a poor visual. Dec 12, 2019 lacquer cracks in an eye with high myopia are in the differential diagnosis of choroidal rupture. 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. 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. Angioid streaks are crack like dehiscences of bruch membrane, which may coexist with systemic diseases, such as pseudoxanthoma elasticum, paget disease, ehlersdanlos syndrome, hemoglobinopathies, or. These include the classic signs of angioid streaks, peau dorange, comet lesions. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Improving differential diagnosis of angioid streaks. No drusen or other signs of amd were evident figure 1. Ehlersdanlos syndrome, angioid streaks in springerlink. Patients with degenerative myopia develop floaters and posterior vitreous detachment earlier in life. We report the occurrence of lacquer crack in a 43yearold woman with no history. Angioid streaks usually do not extend past the equator.
Lacquer crack started in the right eye and became evident 1 year later in the left eye. Angioid streaks represent a rare ophthalmic condition that has very specific ophthalmoscopic features but is caused by different etiologies. There are many causes of mnv including angioid streaks, choroidal rupture, idiopathic, ocular histoplasmosis, and agerelated macular degeneration. Key features narrow, jagged lines deep to the retina. In addition, mild trauma to the eye may cause the choroid to rupture at these areas, leading to submacular hemorrhage. 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 represent a rare ophthalmic condition that has very specific ophthalmoscopic. The cracks appear as thick, brown or grayish lines that radiate from the area around the optic nerve.
We report an unusual case of bilateral vertical lacquer crack with no history of ocular trauma and with progressive. Macular choroidal thickness and volume in eyes with. 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. 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. Pxe affects the retina through a dimpling of the bruch membrane.
Dec 11, 20 we report an atypical case of suspected choroidal neovascularization associated with pseudoxanthoma elasticum. They are also called knapps striae as he first coined the term in 1892 because of their marked resemblance to blood vessels. Angioid streaks definition of angioid streaks by the free. Angioid streaks differential diagnoses medscape reference. Oct 20, 2017 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. 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. Although uncommon, angioid streaks often have a systemic cause and warrant. Angioid streaks are visible, irregular crack like dehiscences in bruch membrane that are associated with atrophic degeneration of the overlying retinal pigment epithelium. That this is the case is distinctly seen in thefundus here illustrated, which pigment epithelium below the disk is relatively poor in. Look for lacquer cracks in high myopes for early warning of. The choroid may be thinner in eyes with angioid streaks and cnv compared to angioid streaks without cnv. Bruchs membrane can thicken, calcify and begin to crack.
Fluorescein angiography and oct scanning help diagnosis the presence of mnv. A 37yearold patient with bilateral high myopia who visited our clinic. Pdf dominant inherited tilted disc syndrome and lacquer cracks. Choroidal ruptures, on the other hand, share with lacquer cracks similar distribution, color, and fluorescein angiographic. The retinal specialists that ive seen have said much the same to me. Choroidal ruptures, similar distribution, color, and fluoresceinangiographic appearance to lc, but are caused by a traumaticevent.
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. Full text lacquer cracks observed in peripheral fundus. Multimodal imaging including spectraldomain optical coherence. The ophthalmoscopic variations and diagnostic difficulties which. Lacquers cracks a degenerative finding noted in pathological myopia, also characterized. Pxe is not an eye disease, so to speak however, its effects can be seen in the eye. 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. Unlike other similar presentations such as lacquer cracks, they dont radiate from the center of the posterior pole. Apr 15, 1987 myopic cracks, angioid streaks, and traumatic tears in bruchs membrane. Progressive symmetric vertical macular wide angioid streaklike. A 47yearold caucasian woman with pseudoxanthoma elasticum and angioid streaks was referred because of decreased and distorted vision in her right eye of one weeks duration. Optical coherence tomography oct reveals the retinalsubretinalsubrpe changes in cnv, and also hyperreflectivity at the bruchs membrane level due to calcification.
Angioid streaks synonyms, angioid streaks pronunciation, angioid streaks translation, english dictionary definition of angioid streaks. The features of eds were first described by hippocrates in. Secondary choroidal neovascularization is also possible. At least 50% of all individuals with pxe demonstrate angioid streaks. Exam 2 disease pics optometry v 745 with mal at indiana universitybloomington studyblue flashcards. Angioid streaks are orange, red, grey or brown in color and can be confused for. At what dose of radiation is a patient at risk of developing radiation retinopathy. The effects of angioid streaks are much more severe if the cracks extend into the center of the retina. To compare multimodal imaging in detecting lacquer cracks in highly. Lacquer cracks are often mistaken for angioid streaks. Lacquer cracks are often bilateral and the eyes have other signs of high myopia including a scleral crescent adjacent to the tilted disc and a posterior staphyloma. If you have angioid streaks in one or both eyes, it is possible to remain asymptomatic and maintain perfect vision for a long time.
Lacquer cracks have only been observed in the posterior pole of highly myopic eyes. Dominant inherited tilted disc syndrome and lacquer cracks. Angioid streaks are visible irregular crack like dehiscences in bruchs membrane associated with atrophic degeneration of the overlying retinal pigmented epithelium. Wet amd lookalikes american academy of ophthalmology. Nov 01, 2011 improving differential diagnosis of angioid streaks diagnostic considerations and a case study. 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 optic disc. The enface oct may reveal the areas of angioid streaks. Improving differential diagnosis of angioid streaks retinal physician. Angioid streaks are bilateral, narrow, irregular lines deep to the retina configured in a. Ophthalmologic manifestations of pseudoxanthoma elasticum. Jul 22, 2019 in 1889, doyne first described angioid streaks in a patient with retinal hemorrhages secondary to trauma. 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. Jun 16, 2012 this article was created to educate students on the basics of degenerative myopia. Angioid streaks are visible irregular cracklike dehiscences in bruchs membrane associated with atrophic degeneration of the overlying retinal pigmented epithelium.
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