Southwest, FL | Case of the Day- Fuchs' Dystrophy | Cataract & Refractive Institute of Florida
Dr. Croley: Hello and welcome to Case of the Day. I'm Dr. Crowley. And today we're going to discuss an interesting disease and a lady who came in with this particular problem. And this disease is called Fuchs' as F-U-C-H-S dystrophy, which was first described from an Austrian back many years ago in the 1800s, late 1800s and the beginning of the 1900s.
Dr. Croley: This disease is characterized by a dystrophy or problem with the inside lining of the cells of your cornea. So the cells of your cornea, which is called the endothelium, line the inside lining of your cornea and these cells are responsible for popping the fluid out of your cornea and therefore making the cornea stay clear so your vision's clear. As time goes on, these cells quit working and gradually the cornea swells, which then leads to blurred vision. It probably occurs maybe in 1% of the population, much higher in women. And it is an inherited process and it is a autosomal dominant inherited process with a variety of penetration. That is, not everyone gets it, but it is a dominant-type inheritance.
Dr. Croley: It usually can start seeing what are called guttata or little bumps in this endothelial layer, sometimes even as early as the thirties or forties, but then gradually progresses and can start causing swelling of the cornea in some, in the sixties and later.
Dr. Croley: As far as their treatment of this goes, in the beginning it's just a medical treatment and a lot of people are tried on a hypertonic solution or eyedrop, this place in the eye and the salt that's put on the surface of the eye tries to draw out the fluid from the cornea and keep the vision better. And that has some success, not that greatest a success.
Dr. Croley: The other choices is many people can try using a hairdryer. You blow a hairdryer from arm's length distance on your eyes and dry the cornea out using a hairdryer and that helps to thin the cornea down in help the vision in some people. But if it gets to the point that someone's not seeing well from this, then the actual treatment is to replace the cornea or in today's time, actually only replace that inside lining of cells. So prior to the last few years, these people had to have a full thickness corneal transplant where an incision was made all the way through the cornea, like a cookie cutter, and then a donor cornea was sutured into place. And many times there was a long healing period before people had good vision with this treatment.
Dr. Croley: Recently, there's been a new surgical procedure called DSAEK, where now just that inner lining of endothelium and a little bit of Descemet's membrane underneath that are removed. And then just that layer is replaced and this even can be done without sutures today. And you get a much more rapid recovery of vision and still maintain the outer surface of your eye and cornea, which gives you a better visual result. And so now the treatment for this is now actually called DSAEK and it works very well.
Dr. Croley: People typically, when they have this, they start complaining of blurred vision and a lot of problem with glare because if the cornea swells, light hits the cornea and it tends to cause glare, so people complain about blurred vision and glare and just not seeing right and eventually, like I said, it progresses.
Dr. Croley: Typically speaking, a lot of people end up developing cataracts at the same time and so the cataract surgery is done first and then this is followed by the corneal transplant maybe three or four weeks later. And even if someone doesn't have a cataract but has developed severe enough Fuchs' dystrophy, then we usually end up taking their lens out anyway and doing a cataract surgery type procedure before there's much of a cataract because you don't want to go back inside the eye after this has been done and do any kind of surgery.
Dr. Croley: In some cases you have to go back or you have to do the cataract surgery or what's called a clear lensectomy in a few cases where you're taking the lens out first and then have a cornea replaced. The success rate of this has gotten very good and people can do very well with Fuchs' dystrophy now. And so it's a disease that we have a great way of treating as far as surgically repairing that.
Dr. Croley: If you have any questions about Fuchs' dystrophy or anything else, you can try contacting us through the website. If not, may God grant you healthy vision and healthy eyes.