Eye Issues and Treatment in Marfan Syndrome, Stickler Syndrome, and Other Related Conditions
16:18-19:11
Now here is the real patient. There's two eyes. If the first eye, you can see this, that the blue eye on your left screen and that you see the dark hole, that's the aperture, that's the pupil. You don't see anything and if you look at the top part, you don't see the zonules, you don't see the elastic bands because it's really blocked by the iris, right? So you only see that through the dark hole.
では、実際の水晶体亜脱臼の症例を見ていきます。まず左側の青い眼を見てください。黒い穴がみえますが、これが瞳孔です。上の図をみると、虹彩があることで、チン小帯は見えなくなっています。これが正常な眼です。
But if you look at look at the right, the second, the other eye and you can see the flimsy, the stringy stuff. Those are zonules. Why you're seeing this through the small holes, a small hole, the aperture? Because the lens is dislocated. The zonules are broken so that happens in Marfan patients. That's ectopia lentis.
次に右側の眼ですが、何かひものようなものが見えます。これがチン小帯です。なぜこのようなものが見えるかというと、レンズがずれているからです。マルファン症候群の方では、チン小帯が切れてこのような症状がみられます。これが水晶体亜脱臼です。
The cataract, so now here is another patient of mine. I wanna show you, said again that you have to be your own advocate and this is a patient and it's one and this is the other eye.
その他の症例もお見せします。私が担当する患者さんの両目です。
The first eye, you can see this. I'm sure you can appreciate this. This again is the dark hole, aperture, the pupil and is clear. But on the bottom part you can appreciate. I have the blue arrow indicating there's a crescent shape. Okay? That's because the lenses are dislocated superiorly, moved the upward so that's why you have this empty space, and this point those patient can be have it totally asymptomatic. They don't complain. And this, if you just do like a casual eye exam, you're not aware of this and you can easily miss it so you have to make sure that your physicians are aware of this.
左上の眼では、瞳孔はきれいに見えますが、青い矢印で示した部分が三日月型になっているのがおわかりになると思います。レンズが上に動いたためにこのような隙間ができます。このくらいのズレでは、自覚症状がないこともあります。通常の眼の検査では、この所見は見逃されることがありますので、担当医に確実に見つけてもらう必要があります。
And we encounter, at least I encounter those patients. The cardiologist, for instance, the internist, send patients to me asked me to check the eyes to provide this finding, "do I see the ectopia lentis?" That's, that is actually is that one of the major criteria for diagnosing Marfan syndrome.
例えば、循環器内科医からの紹介で、患者さんが水晶体亜脱臼かどうかの確認をお願いされることがあります。水晶体亜脱臼は、マルファン症候群の診断にあたっての重要な基準の一つです。
And this eye is just way too obvious and this is the other eye. You can see this the lens is actually bisecting the aperture, the pupil, and you can imagine at this point you get confused when the light go through not only just going this portion, through this portion as well. So look like light gets scattered on the back so that becomes this, at this point the patient can be very, very symptomatic. They do complain. There ways to fix it, but you do have to be aware of the potential complications. So look like light gets scattered on the back so that becomes this, at this point the patient can be very, very symptomatic. They do complain.
右下の症例では、水晶体亜脱臼がはっきりとわかります。レンズにより瞳孔が2つの部分に分かれています。このような状態になると、光がレンズのある部分とない部分を通過することになるため、眼の後ろに届いた時点で光が分散し、はっきりとした自覚症状が出ます。
There ways to fix it, but you do have to be aware of the potential complications.
治療法はありますが、合併症の可能性もあることも覚えておいてください。
The Marfan Foundation did not participate in the translation of these materials and does not in any way endorse them. If you are interested in this topic, please refer to our website, Marfan.org, for materials approved by our Professional Advisory Board.
The Marfan Foundation は、当翻訳には関与しておらず、翻訳内容に関してはいかなる承認も行っておりません。このトピックに興味をお持ちの方は、Marfan.org にアクセスし、当協会の専門家から成る諮問委員会が承認した内容をご参照ください。
