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