Marfan Syndrome Management: Back to Basic
10:23-11:39
So what about the eyes in Marfan syndrome? So this is the second most affected besides skeleton and so the eye is the most common finding or the most, the most pathognomonic finding is something called ectopia lentis or we also call it lens subluxation or lens dislocation and this is where you can see here, the lens should be down here in the eye. It has an anterior sort of loosening slides up. This makes it look like you can see it on a regular exam but you can't, only an ophthalmologist, ophthalmologist and eye doctor can see it but it slides up and it can cause loss of vision over time so this is really important that it be, that it's followed and there are lens surgeries that can be done to prevent this acceleration.
Marfan症候群における眼の所見は骨格に次いでよく見られます。眼の所見で最も特徴的といえるのが、水晶体亜脱臼です。この右の図を見ればわかりますが、水晶体は本来真ん中にあるべきなのですが、上の方にずれています。このような状態になれば、普段受けている検査でも見つかりそうなものですが、実際は眼科医でなければわかりません。水晶体が上にずれてしまうことで、やがて視力が低下しますので、定期的な検査を受けることが重要です。水晶体のずれが進行しないようにするための手術もあります。
Also even more common than lens dislocation is myopia, which is near-sightedness. This is really common, so in a normal eye your focus is all the way at the back of the retina but in myopia it's a little close forward, so it's really common in people with Marfan syndrome to have significant myopia, you know, they need a, they need diopters and their glasses of positive five or six. That's really common.
水晶体亜脱臼以外に多くみられる所見が近視です。正常な眼の場合、焦点は網膜の上にありますが、近視の場合には、網膜に到達する前に焦点が結ばれてしまいます。Marfan症候群の患者さんの場合、重度の近視となることが多く、+5～+6D（ディオプター）といった度数のメガネが必要となります。非常に多い所見です。
And if you have severe myopia you're prone to get something called retinal detachment and this is a tear of this back this area called the retina of the eye. People who have severe myopia are higher risk of this so people with Marfan are at risk of retinal detachment, so this is why seeing an eye doctor is really important.
重度の近視により網膜剥離となる可能性が高まります。網膜剥離とは眼の後ろにある網膜が破れてしまう疾患です。重度の近視の方がなりやすい病気ですので、Marfan症候群の患者さんは網膜剥離となるリスクが高いといえます。やはり眼科の受診が大切です。
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。
