쉐도잉 연습: Other Corneal Disorders - CRASH! Medical Review Series - 영상으로 영어 말하기 배우기

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we'll talk here about some other corneal problems
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so we'll talk about the wide range of corneal dystrophies as well as keratotorus
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and keratoconus so the cornea is seen here it's right here it's this transparent portion consistent with the sclera
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that covers the anterior chamber it's important that the sclera is transparent
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because it allows light to go through the lens
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and ultimately to the retina that being said even though the cornea
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and the sclera are consistent the cornea is much different tissue than the sclera the cornea has to be transparent
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and so it's made up of very organized collagen fibers as well as cells.
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The cornea gets its nutrients through the anterior aqueous,
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not through vessels, and that allows it to stay clear in its non -pathologic state.
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Okay, so corneal dystrophies, I don't want you to memorize all of these by any means
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because you don't have to memorize these, you don't have to memorize the treatment, you just need to know when to suspect corneal dystrophy because these are going to be treated by an ophthalmologist.
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So these are a group of progressive congenital eye disorders in which
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abnormal material accumulates in one or several levels of the cornea
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and this disrupts the transparent property of the cornea as well as the corneal structure itself
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so essentially what's going on is
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because you have a genetic mutation the cells are creating something
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that they shouldn't be creating and because of that you're disrupting
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that fine organized corneal layer the vast majority of corneal dystrophies are congenital
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and among those most of them are going to be autosomal dominant
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and that's useful because you have a patient who you're suspecting some kind of corneal problem in
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because they have corneal haze corneal opacities one of the best
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questions you can ask is do your parents have any problems
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do your parents have blindness does your parent has have your parents ever had anything like this
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that will be really helpful in establishing this diagnosis.
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They tend to affect both eyes simultaneously, but they don't have to, but that usually is how it goes.
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Common symptoms to most corneal dystrophies, including a long -standing foreign body sensation or mild pain in the affected eye or eyes,
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and that just has to do with the chronic nature of this.
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The foreign body sensation is due to the fact
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that the cornea is a very sensitive structure
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if you don't believe me try and touch the middle of your eye
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and see how long you can do it it's very very highly innervated
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and very sensitive
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so even the smallest changes these dystrophies will cause foreign body sensation the patient may experience halos
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when looking into light and that's just because of the refraction from the foreign body, from the compound that's in the cornea,
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refracting back to the retina.
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Also, there's reduced visual acuity, and you'll see this particularly in patients where there's significant corneal haze.
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And then otherwise, the patient is normal, so their pupils will react to light and to accommodation.
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Extraocular movements should be normal.
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The only thing that's abnormal is the fact that these patients generally have some level of corneal haze, difficulty seeing, and a foreign body sensation.
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And this is a chronic problem.
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So this isn't something that came up a week ago.
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This is something that the patient has been dealing with for several months.
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So among corneal dystrophies, and there's about 17 or 18 that are known.
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There's probably even more.
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The similarities are that they tend to be congenital.
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The symptoms are consistent.
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There's a long -standing foreign body sensation or mild pain in the eyes.
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Decreased acuity as these corneal opacities come in.
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They don't affect other parts of the body.
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These are syndromes, but they don't affect anywhere else in the body except for
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the eyes they also tend to affect both eyes similarly at the same time
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and they're not caused by external forces this is a genetic problem this has nothing to do with trauma or infection
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The differences lie in the particular location of the cornea that's affected.
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Some tend to be more superficial, some tend to affect the stroma more, and some tend to affect the endothelium more.
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Some have symptoms very, very early on, and some don't.
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The age of presentation can be different.
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Some of these corneal dystrophies present in childhood, others present in later adulthood could.
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The extraneous materials that you would see if you were to do a histology would be different.
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So some of them will make lipids, some will make cholesterol, and that's certainly not the end.
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There's many more extraneous materials that could be secreted into the cornea.
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The prognosis can be different based on whether
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or not we have a good treatment modality for that particular corneal dystrophy and that kind of works into the treatment.
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So here's our cornea here.
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This is just cut right down the middle.
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This is the outside here and then this is the inside here bordering on the anterior aqueous chamber.
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So you have your stratified squamous epithelium here.
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There's several layers.
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These cells regenerate and they move upward.
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So these outer cells are flat like a pancake they're squamous
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cells you see a lot of nucleus material the more inner cells are more of a columnar shape beneath
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that you have Bowman's membrane
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which serves as basement membrane for the epithelium the largest section of the cornea is made up of collagen
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and it is the stroma
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and then down here you see a little layer of cells this is endothelium
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and But in between the endothelium and the stroma, you have what's known as decimates membrane.
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So the anterior corneal dystrophies affect the epithelium and the Bauman's layer.
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There's four.
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There's the epithelial basement membrane dystrophy, which is autosomal dominant, but it can be de novo.
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So the patient wouldn't have any parents who had it.
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The patient just developed the mutation on their own.
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Lisch corneal dystrophy, which stands alone as the one X -linked dominant corneal dystrophy, Rice -Buckler corneal dystrophy, and Theobanky corneal dystrophy.
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Those are both autosomal dominant.
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When it comes down to the stroma, there are several different types.
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Most, all but two, are autosomal dominant.
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Gelatinous droplet corneal dystrophy, there's the two granular corneal dystrophies, type one and two the two lattice corneal dystrophies type one
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and two macular corneal dystrophy which is autosomal recessive and schneider crystalline corneal dystrophy which is autosomal dominant
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like i said you don't need to memorize these
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and then the posterior corneal dystrophies these affect the endothelium
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and the decimates membrane there's congenital hereditary endothelial dystrophy
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or ched type one that's an autosomal dominant variant of the same disease as chad type 2 which is autosomal recessive.
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And then Fuchs endothelial dystrophy is an autosomal dominant disorder.
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This has a distinguishing factor in that this tends to affect females more than men.
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It also tends to affect older women more than younger women.
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And then finally posterior polymorphous dystrophy which is autosomal dominant.
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So here's all your corneal dystrophies, the major types of corneal dystrophies, do not memorize these.
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Here's some distinguishing factors though.
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So lattice corneal dystrophy type 1, macular corneal dystrophy, and Schneider crystalline corneal dystrophy,
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as well as both of the CHED dystrophies, these all have a tendency to affect young people.
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So CHED 1 and 2 will be apparent at birth and
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so by apparent I mean corneal opacities
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and in case you're wondering how do you tell a corneal
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opacity from a lenticular opacity the corneal opacity will appear over
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even the iris whereas the lenticular opacity you won't see any opacities over the iris just over the pupil.
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So CHED 1 and 2 will be apparent at birth.
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Signs of lattice corneal dystrophy may be present at birth, but symptoms do not typically present until the child is older,
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post -pubescent, or even early adulthood.
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Schneider crystalline corneal dystrophy can be a present in the first decade of life, so young children again here,
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and then signs of macular corneal dystrophy can be present around puberty.
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So these are some of the corneal dystrophies that tend to show up in younger people.
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On the other hand, we have Fuchs endothelial dystrophy.
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These tend to present in older people, in people who are in their 50s, 60s, 70s and beyond.
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This also has a tendency to present in women more than men, about 4 to 1 ratio.
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So a Fuchs endothelial dystrophy would be notorious for striking the
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otherwise healthy 60 to 70 year old lady with no other health problems other than a progressive decreasing visual acuity,
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which she ascribes to aging.
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Very important to look in the patient's eyes.
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Don't always say, oh, you're an old person.
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It must be presbyacusis.
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Maybe your lens is getting stiff.
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Look into the eyes.
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You know corneal precipitates.
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Obviously, those shouldn't be there.
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And then you look back on her history questionnaire.
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And indeed, her father was totally blind by age 75.
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And this is an autosomal dominant disorder, meaning that if a parent has it, you have a 50 -50 chance of getting it.
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So all suspected corneal dystrophies are going to require consult to an ophthalmologist for specialized diagnostics and treatment.
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If they're a younger person, especially if it's an autosomal dominant disease, you should provide them with genetic counseling at some point in the future
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and occupational therapy can be useful for helping these people maximize their potential.
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Your job as the clinician, as the internist, as the pediatrician,
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as the ED doctor, is not to make the official diagnosis of which type of corneal dystrophy the patient has.
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That's done by the ophthalmologist.
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Your job is to suggest
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that this patient may have a corneal dystrophy to rule out
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any other possible causes of corneal haze such as keratitis
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and then to identify these patients and send them off to the ophthalmologist.
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There's very little you can do for corneal dystrophies in a clinical setting and the condition itself is not an emergency.
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So this might be a patient that
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if they say oh I've only noticed it for one or two days you might get a fluorescein stain on.
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But if they say they've had it for several weeks, maybe not.
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It's probably going to be a corneal dystrophy.
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Okay, keratotorus, this is also known as pellucid marginal degeneration,
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is an idiopathic degenerative corneal condition characterized by abnormal thinning of the stroma.
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And the stroma is part of the cornea.
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So when the stroma thins, the corneus thins.
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You also have a thinning of Baumann's layer and possibly other structures, but it's primarily the stroma.
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And the bulk of this thinning happens below the horizontal midline of the cornea and then out along the periphery.
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So if you think of a clock, you would think of
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if you were to take out the inner circle of the
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clock from about four o 'clock to eight o 'clock
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so the the periphery on the bottom of the eye
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and this shape creates an irregular astigmatism remember astigmatism is just due to an abnormally shaped lens
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or an abnormally shaped cornea and the result of that is
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that you're projecting different dimensions in different areas either in front on
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or behind the retina and so this creates an irregular astigmatism so a patient presenting with new astigmatism, this is a possible diagnosis.
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Typically, carototaurus affects both eyes, not just one.
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The symptoms include gradual decrease in visual acuity, distorted images, symptoms that are consistent with astigmatism.
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Oftentimes, the patient has seen an optometrist for glasses, but the prescription keeps changing because carototaurus,
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pellucid marginal degeneration, is a progressive disease.
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In some cases the patient will have extreme pain and those cases it will reflect a corneal perforation but that's rare.
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So for diagnosis what you're going to do is a slit lamp examination
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and you're going to do corneal topography
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and the corneal topography will help you identify how thick the cornea is
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and in which areas and so if you notice that the cornea is thinner, down at the bottom, along that four o 'clock to eight o 'clock line,
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then your suspicion for cratitoris goes up.
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The treatment, 88 % of these patients will be managed by eyeglasses or contact lenses.
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They'll need frequent follow -up with an optometrist
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because they're going to need changes in their prescription relatively frequently
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or more frequently than most of us 12 % of these patients will be candidates for phototherapeutic keratectomy,
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but the primary treatment for keratotaurus is eyeglasses or contact lenses.
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Keratoconus is an idiopathic degenerative disorder of the cornea characterized by degeneration of the peripheral corneal structures.
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So if you think of the cornea as a globe,
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as a dome if you're degenerating all the area around it with the exception of the middle then
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that ball that globe is going to turn into a pyramid
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and this is where we get the word keratoconus so so
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that change in shape will certainly cause refractory issues remember
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that the cornea has a refractory index of about 40 45 to 50 diopters
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so as the shape of the cornea changes it's going to
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cause refractory issues can cause astigmatism as well as monocular diplopia most commonly keratoconus is diagnosed in the adolescent
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years.
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So the symptoms are distortion of images, decreased visual acuity at all distances,
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monocular diplopia, difficulty with night vision, and halos.
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And again here we're going to do a slit lamp examination for corneal topography.
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We want to get a good idea of what
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that cornea looks like because a coratotaurus versus a coratoconus versus a normal cornea look completely different.
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And the treatment here, there's abundant options.
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Contact lenses can be used, special contact lenses, to scleral lenses, to several different types of surgery, including also corneal transplant.
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So here's an example of keratoconus.
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If you look up really closely, you can see that this cornea is relatively normal on the front,
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but it's thinned out along the sides.
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and it makes that cone shape.
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It's not a perfect cone, but you can see where they get the word keratoconus.

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  • lens /lɛnz/ (명사) — 렌즈. An object, usually made of glass, that focuses or defocuses the light that passes through it.
  • symptom /ˈsɪm(p)təm/ (명사) — 증상, 증후. A perceived change in some function, sensation or appearance of a person that indicates a disease or disorder, such as fever, headache or rash; strictly, a…
  • thin /ˈθɪn/ (형용사) — 가늘다, 얇다. Having little thickness or extent from one surface to its opposite.
  • layer /ˈleɪ̯ɚ/ (명사) — 층. A single thickness of some material covering a surface.
  • sensation /sɛnˈseɪʃən/ (명사) — 감각. A physical feeling or perception from something that comes into contact with the body; something sensed.
  • membrane /ˈmɛm.bɹeɪn/ (명사) — 막. A mechanical, thin, flat flexible part that can deform or vibrate when excited by an external force.
  • disorder /dɪsˈɔɹdɚ/ (명사) — 혼란. Absence of order; state of not being arranged in an orderly manner.
  • diagnosis /daɪəɡˈnəʊsɪs/ (명사) — 진단. The process of, or an instance of, identification of the nature and cause of a medical condition or illness.
  • transparent /tɹænsˈpæɹənt/ (형용사) — 투명하다. See-through, clear; having the property that light passes through it almost undisturbed, such that one can see through it clearly.
  • chamber /ˈt͡ʃeɪ̯mbɚ/ (명사) — 침실. The private room of an individual, especially of someone wealthy or noble.
  • sensitive /ˈsɛn.sɪt.ɪv/ (형용사) — 민감하다. Having the faculty of sensation; pertaining to the senses.
  • difficulty /ˈdɪfɪkəlti/ (명사) — 어려움. The state of being difficult, or hard to do.
  • versus /ˈvɝsəs/ — 대. Against; in opposition to.
  • globe /ɡloʊb/ (명사) — 지구의, 지구본. The eyeball.
  • examination /ɪɡˌzæm.ɪˈneɪ.ʃən/ (명사) — 진찰, 검사. The act of examining.

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  • come up (동사) — 올라오다. To emerge or become known, especially unexpectedly.
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  • “th” 소리: thin /ˈθɪn/, thick /θɪk/, beneath /bɪˈniːθ/, ophthalmologist /ˌɔf.θə(l)ˈmɑ.lə.d͡ʒɪst/, epithelium /ˌɛpɪˈθiːlɪəm/
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  • 긴 단어 — 강세 위치에 주의: anterior /ænˈtɪɹ.i.ɚ/, difficulty /ˈdɪfɪkəlti/, examination /ɪɡˌzæm.ɪˈneɪ.ʃən/, irregular /ɪˈɹɛɡ.jə.lɚ/, primarily /ˈpɹaɪ.mə.ɹə.li/

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