Coats Disease can cause strabismus and blindness

Jamie's son - yellow eye photo typical of Coats' DiseaseSee About the Photo for copyright information.
Jamie’s Warning: “Request dilation at regular intervals and definitely before any strabismus surgery.”

The following story was originally posted by Jamie Schmidt in our Strabismus Hope email support group. Jamie and I have edited that original post together to provide additional info from Jamie here:

My 9 month old son was diagnosed with strabismus at 5 months, specifically esotropia, and after several appointments with a highly recommended ophthalmologist, surgery was being scheduled for January to relax the inner eye muscles. Up until this point, his eyes appeared healthy, and they had no known cause for his strabismus.

We decided to get a 2nd opinion (which actually felt like a 3rd because our very good optometrist agreed with the first ophthalmologist) from another top ophthalmologist at a different hospital. Same eye exam, dilate the eyes, same routine. However, on examination one of the dilated eyes showed an abnormality and on closer examination by a retina specialist and an ultrasound, he was diagnosed with Coats’ Disease. Our hope is that the vision loss from the Coats’ disease was the root cause of the strabisimus, but only time will tell.

I write to inform all of you about Coats’ Disease, so that if you have a little one and no cause for the strabismus, keep asking for the eyes to be dilated.

Coats’ is considered a rare disease. One internet site stated that Coats’ Disease occurs in approximately 1 in 30,000 births. It is a disease that most of the time affects males under age 18 in one eye. It can usually only be detected with a dilated eye. One other ‘symptom’ is that sometimes instead of having a red eye in pictures, the child may have a yellow eye.

With Coats’ Disease, it is my understanding that the blood vessels at the back of the retina are leaky. They leak water, proteins, and lipids or fats, like cholesterol. The body absorbs the water and proteins, but has a harder time absorbing the fats, which deposit on the retina. If left untreated, Coats’ Disease could cause complete retinal detachment.

Even partial retinal detachment causes blurry to no vision in different spots, and the brain may shut off the mixed signals from that eye. It is sometimes a rapidly progressing disease and needs to be confirmed by an exam under anestheia. If it is truly Coats’ Disease, they can seal off the leaky blood vessels with laser or freezing.

I encourage all of you to request dilation at regular intervals and definitely before any strabismus surgery. If someone has Coats’ Disease, strabismus surgery won’t be effective until the Coats’ Disease is treated. Even the most highly skilled optometrists and ophthamologists could miss this disease unless the eyes are being regularly checked with dilation. Within the last 6 months, our son’s eye was dilated twice and both times the Coats’ was undetected because the lipids had not accumulated.

By the grace of God we decided to get a 2nd opinion at the right time. Any earlier and they probably wouldn’t have been able to see the lipid build-up on the retina and any later and permanent blindness may have resulted, as we weren’t scheduled to have the eyes dilated until before surgery in January.

Jamie shared a separate email about her about her son’s laser surgery yesterday after the procedure:

If left untreated, Coats’ Disease could cause complete blindness in the affected eye and sometimes the eye has to be removed. However, there are methods to reattach the retina. Coats’ Disease can be a cause of strabismus and amblyopia.

Our son is doing great. He just had the exam under anestheia this morning and laser surgery to seal the blood vessels. We couldn’t be more pleased with how he did. He will have to have follow-up laser surgeries. They will have to wait and see how much damage has been done to the retina. He had a moderate amount of build-up of the lipids.

Thank you for forwarding this information on. I hope it may help someone else in the same position. And, just a reminder to everyone, get that 2nd opinion!!


About the Photo:
Photo copyright Jamie Schmidt, used by permission. Jamie writes, “We just took his picture after the exam/laser surgery and actually saw the yellow eye, which we hadn’t seen before. Amazing!” Inquiries about the photo may be sent to ‘jerome DOT schmidt AT mchsi DOT com.’

More about Coats’ Disease
Coats’ Disease is also referred to as “Coates Disease,” but Coats’ is the correct spelling. Coats’ Disease is named after Ophthalmologist George Coats, who first identified the disease in 1908.

Synonyms for Coats’ Disease include

  • Congenital Retinal Telangiectasia
  • Exudative Retinitis
  • Leber miliary aneurysm

However, according to “The Handbook of Ocular Disease Management” (see Resources below), there is a distinct difference between Coats’ Disease and Leber miliary aneurysm.

Resources
Here are two sites recommended by Jamie:
Coats Disease Information for Parents

Coats Disease Resources – Home

Additional sites that may be helpful:
Handbook of Ocular Disease Management – Coats’ Disease and Leber’s Miliary Aneurysm

Royal National Institute for the Blind (RNIB) – Coats’ Disease

Orphanet: About rare diseases – Coats’ Disease

11 thoughts on “Coats Disease can cause strabismus and blindness”

  1. I believe there is hope for Coates disease in terms of management; however, as Jamie eluded to above, one needs to be proactive.
    In my case in 1982 I was a grown woman of 32 years of age, married with two toddlers at the time I was diagnosed. What prompted the visit to the retinal surgeon was a direct referral from an optometrist I had gone to see for glasses. Although he did not dilate my eyes, when he looked through the pupil he said they “looked all wet” and believed I’d best see a surgeon immediately. Fortunately for me, by the grace of God, I picked that particular optometrist who had just been to an inservice by a retinal surgeon concerning signs of retinal distress.
    Once to the retinal surgeon and a mountain of tests, scans, and photographs the diagnosis came that day. I was afflicted in both eyes with Coats’ with all of the symptoms inclusive of the cysts and bilateral retinal detachments (which were close to detaching thru the macular area, the place of the retina where we have central vision).
    I had Coates Disease, probably had been afflicted since childhood or before, and luckily for me it progressed so slowly that serious vision problems didn’t show until adulthood. Unfortunately for me, it was almost too late. Pitiful as it may seem, I had seen an ophthamologist just the year before because I was noticing floaters and vision problems. He did dilate my eyes, but obviously did not know what he was seeing, and thus sent me away saying everything was fine.
    As a child and young adult I always prided myself with having exceptionally good vision–at least I thought so. Three things I always wondered about, however, is, when I closed my eyes I always saw weird lights that I thought was odd because my eyes were closed; my depth perception was not great, but I had fantastic eye-hand coordination; and I had difficulty in reading, although my vision tested seemed just fine.
    The disease was treated very aggressively by this young retinal surgeon who had decided to save my vision. Laser surgery was a bust–no help at all, yet it was the first course. The second course was surgery. The first involved relieving pressure from my left eye (the worst of the two), performing cryosurgery on all the vessels in both eyes, and banding the left eye. With in a week of being released from the 7 day stay in the hospital the retina in my right eye snapped off completely, and I was back for another 5.5–6 hour stint in the operating room (that day) banding the right eye and receiving some additional cryosurgery. The surgeon was quite relieved the next morning that I could see his light as it really was a crap shoot at that point. I spent another 7 days in the hospital sedated–I was on the mend and he wanted to make certain that it continued for the short term. The second major eye surgery left me with considerable conjunctival scarring and muscle problems, and my left eye was then what we call “wall-eyed”. It took several months for things to settle down and many different prescriptions for glasses prior to being able to really see other than fuzzy images, light and dark. The following year another retinal surgeon, a pediatric muscle surgeon, and an ophthamology plastic surgeon took me back into an operating room for another 5+ hour stint, removed the band from my right eye as it had by then reattached, straightened the muscles as best they could, realigned my eyes so that they looked normal, and removed as much conjunctival scar tissue as reasonable.
    Well, I looked normal again, and a week later on examination it was discovered that I was fusing my vision, and the pediatric guy was just too thrilled about that as there was uncertainty–they could realign my eyes, but they were not really aligned enough to predict that I would be able to use them together.
    That was nearly 24 years ago, and 25 since diagnosis. Day before yesterday I was cleared by the retinal surgeons to have my second cataract removed and a new lens inserted in my right eye (my left eye had cataract removed 5 years ago at which time my iris was repaired as well–it had a laser burn hole caused by breaking an adhesion which left my pupil dilated all the time–the hole itself really was nearly as bad as the dilated pupil).
    Cataracts aside, no, things have not been perfect. My left eye is my bad eye, and I have partial central vision and some, although very little peripheral vision in it; I have better central vision and more peripheral vision in my right eye. Corrected with glasses, however, except for occlusion by cataracts, my central vision has remained at 20/40 left and 20/30 right for the better part of 23 years.
    Yes, I have blind spots, light flashes, I don’t see color well, and my depth perception is next to nonexistant. Additionally the scar tissue from the cryosurgery has grown into a cellophane like membrane over the back of my eyes so that at times my vision is worse, and is always somewhat distorted. These membranes, I am told can be removed if absolutely necessary, but often resolve themselves, and thus unless I start showing significant visual difficulties because of them, they prefer to leave them alone.
    BUT, I CAN SEE, AND SEE RELATIVELY WELL DESPITE the assault on my eyes from both the disease and the following eye surgeries. I have managed my husband’s business for the last 17 years (I had little children at the time of diagnosis), I read, write, DRIVE, and do all the things normal well seeing people do.
    I would like to hope that all Coats’ patients were as blessed as I have been; I know that not to be the case, and truly the disease seems to behave differently among afflicted individuals. It seems clear, however, that the aggressive manner in which my condition was treated (along with considerable faith on my part and refusal to give in to it) is the key to my success. I hope that anyone viewing this blog gains some hope from that. Susan

  2. Hi, my name is Sarah and I have strabismus, dipolopia and sixth nerve palsy. After seeing my uncle who is an eye surgeon and who examined me briefly (without dialiting the eyes) suggested that I not get the surgery because my case was too complicated and that I should just live with it. I then went to see an eye surgeon I was referred to by my ophtalmologist and who is practicing at the biggest hospital. She examined me and dialated my eyes and then had another doctor come and examine me as well. After much notes, she suggested surgery but said it would only correct the palsy a bit. Should I go through with the surgery? I also have a droopy eyelid on one eye side and it bothers me. The double vision is annoying and people judge me because my eyes look crossed at times. Should I proceed with getting the surgery??

  3. When I was 5, (in 1961) I first noticed something different about the sight in my left eye. I could see a black dot in the middle of my site of vision. You know how kids alternately blink they’re eyes to see the overlapping vision? That’s how it began for me. I told my parents about it, and they thought I was just trying to get attention. (I have a brother who is 3 years younger than me) It wasn’t until 5 years later, during a school vision test, that I had someone to tell about my vision, who would listen to me. They did and sent me to see an eye Dr. He did tests and x-rayed my head. He gave me two different diagnoses, the first one being that I was looking at an eclipse of the sun without proper equipment. Well, my father was an Astronomer at the time and we did use proper equipment. Then he said that is was do to me being stabbed in the arm with a pencil (by the class bully) and I got blood poisoning, which I did, and it affected the weakest part of my body at the time, which was my left eye. He then said that nothing could be done for me and that was the end of that.

    It went on for years with the odd change in my vision. The black dot in the middle got bigger, and the rest of the surrounding vision became blurred. It wasn’t till I was 24, married and living in a new city. I went to see an Eye Surgeon, who became my eye Dr. She hadn’t seen this before but she took good care of me. I went to see her every 6 months. Then when I was 27, something happened to scare me. At the time I was studding to be a nurse. I was on my practical training in the hospital when an aneurysm haemorrhaged. I heard the pop. It sounded like the noise made when you click you nails. My eye immediately filled up with blood. I started to panic. My eye Dr.’s office was across for the hospital, so I called her and ran over there. She took a look and was amazed. She also got her colleague in to take a look. It was then that she found the rupture and sent me to see specialists at the Eye Center at Toronto’s St, Michael’s Hospital. I was injected with a solution called florazine which is a dye that they use to high light the blood vessels in the eye to see the damage. In order to do this, they inject the dye and take pictures of the retina at different angles and directions. They do this to both eyes. It was a long and teary process. I was then told the name of the disease and it’s affects on me. They told me that they could do nothing for me, and that IF they did any treatment, it would just speed up the deterioration process to were I would have an ugly looking eye and was eventually going to loose the eye. I was devastated. As a result I dropped out of nursing, and my marriage broke down as well.

    Since then, I moved back to my home town with my second husband and two kids. During the past 18 years, I am nearly completely blind in that eye. I can see some light but not much else. I have near total retinal detachment, cholesterol deposits, haemorrhaged aneurysms that have caused scar tissue on the retina and a deformed cataract. Therefore, I have no useful vision. I’m in the final stages of the disease. It was miss diagnosed in the beginning, and didn’t get any treatment, and I will eventually loose my eye. I have made a return visit to the Eye Center at Toronto’s St, Michael’s Hospital to let them see how far advanced it is. I even had to test a couple of interns, by letting them examine my eye and make a diagnosis. Then I was to tell them what it was. They had no idea what it was and were amazed. They thanked me for letting them take a look. I kind of enjoyed the experience, and would let anyone else have the chance to take a look, if it would help others with Coates disease or Dr’s.

    I have had this disease in my left eye since I was 5. I’m now 50. My present eye Dr. takes great care of me and my kids to make sure they don’t have it either. Neither of them does. My daughter is 25 (married) and my son is 19 and un-affected by this disease. I wear glasses now, bi-focals to read with and to protect my right eye.

    No one can tell when or how I will loose my eye, but as long as I’m not in any pain or the eye doesn’t look deformed, I keep it. I have been searching of information on this disease for some time and not much is known about it. I have compiled what I have found to help others with Coates disease. Please click the ” Coates Disease”link to read more on this disease.

    Mon, 17 Jul 2006
    So far, I’m doing ok, with near total vision loss. I can’t see much of anything, and it feels like the eye shuts down when I’m not thinking about it. The girls at work all know about it and let me know when they are coming up on me from the left. Although, they do sneak up on me at times on purpose! It’s all in good fun. Some times they stand behind me and wait till I turn around to see me jump! All kidding aside, they do know how serious it is. They ask questions etc. I’ve had Dr.’s ask me about it too. That is way I set up my web page. If I can help one person, I’m happy.

    Friday, July 21, 2006
    I guess I’m used to the surprises. Besides, I get them back. 🙂 I try to walk on the right side of the sidewalk, so I won’t bump into things or people. If I don’t hear someone coming up on my left, it does scare me. I’m used to my kids doing it to me when they were little. I find my left arm gets banged a lot. I try to give myself room but sometimes if I’m in a hurry, I bang it. I’m tempted to get some padding to wear on it, or one of those elbow things Skateboarders use. What do you think? I have a hard time giving or taking things too. I have no depth perception or judge of distance. I do drive though. I sometimes have difficulty in parking, unless I practice. I’m constantly looking in my mirrors. All my life, I’ve always tried to compensate and re-adjust to situations.

    July 3, 2008. I went to my eye Dr. this month. He told me that I was his only patient with Coates Disease, and possibly in Peterborough (where I live). So far it hasn’t affected my right eye, but he couldn’t see much of anything in my left eye. We still don’t know how much longer I will have the eye.

  4. Hi,
    MY son is 18 month old. In November 2008 I noticed an abnormal reflection of the light in one of his eye. Since I am in the medical field I immediately know that it could be sign of an eye cancer called retinoblastoma.My husband and eye took him to a Pediatric ophthalmologist who wanted to rule out several diagnosis including retinoblastoma and Coat’s disease. He referred us to a retinal specialist. At our first visit to the specialist he was able to confirm that my son has Coats’ disease. At that point we schedule an appointment to have him evaluate under anesthesia and proceed with the laser therapy.
    We were relieved that it was Coats’ disease since the treatment and prognostic are not as aggressive and stressful as retinoblastoma.
    Now, my concern is about his sight from the Coats’ eye. It is clear that his sight is disturbed but we do not know how much he can see if he can see at all. We hoping to find resources that would enable us to effectively address his condition.
    My advise for parents is to request that your infants and toddlers have an eye exam once a year.

  5. thats a very intresting thing about peripheral vision.his refection toward the eye is unique i think so because who do you know has an eye like him god created this world and everyone in it diffrent.theres a lot of touch towards this humean eye….

  6. When I was 5, (in 1961) I first noticed something different about the sight in my left eye. I could see a black dot in the middle of my site of vision. You know how kids alternately blink they’re eyes to see the overlapping vision? That’s how it began for me. I told my parents about it, and they thought I was just trying to get attention. (I have a brother who is 3 years younger than me) It wasn’t until 5 years later, during a school vision test, that I had someone to tell about my vision, who would listen to me. They did and sent me to see an eye Dr. He did tests and x-rayed my head. He gave me two different diagnoses, the first one being that I was looking at an eclipse of the sun without proper equipment. Well, my father was an Astronomer at the time and we did use proper equipment. Then he said that is was due to me being stabbed in the arm with a pencil (by the class bully) and I got blood poisoning, which I did, and it affected the weakest part of my body at the time, which was my left eye. He then said that nothing could be done for me and that was the end of that.

    It went on for years with the odd change in my vision. The black dot in the middle got bigger, and the rest of the surrounding vision became blurred. It wasn’t till I was 24, married and living in a new city. I went to see an Eye Surgeon, who became my eye Dr. She hadn’t seen this before but she took good care of me. I went to see her every 6 months. Then when I was 27, something happened to scare me. At the time I was studding to be a nurse. I was on my practical training in the hospital when an aneurysm hemorrhaged. I heard the pop. It sounded like the noise made when you click you nails. My eye immediately filled up with blood. I started to panic. My eye Dr.’s office was across for the hospital, so I called her and ran over there. She took a look and was amazed. She also got her colleague in to take a look. It was then that she found the rupture and sent me to see specialists at the Eye Center at Toronto’s St, Michael’s Hospital. I was injected with a solution called florazine which is a dye that they use to high light the blood vessels in the eye to see the damage. In order to do this, they inject the dye and take pictures of the retina at different angles and directions. They do this to both eyes. It was a long and teary process. I was then told the name of the disease and it’s affects on me. They told me that they could do nothing for me, and that IF they did any treatment, it would just speed up the deterioration process to where I would have an ugly looking eye and was eventually going to lose the eye. I was devastated. As a result I dropped out of nursing, and my marriage broke down as well.

    Since then, I moved back to my home town with my second husband and two kids. During the past 18 years, I am nearly completely blind in that eye. I can see some light but not much else. I have near total retinal detachment, cholesterol deposits, hemorrhaged aneurysms that have caused scar tissue on the retina and a deformed cataract. Therefore, I have no useful vision. I’m in the final stages of the disease. It was miss diagnosed in the beginning, and didn’t get any treatment, and I will eventually lose my eye. I have made a return visit to the Eye Center at Toronto’s St, Michael’s Hospital to let them see how far advanced it is. I even had to test a couple of interns, by letting them examine my eye and make a diagnosis. Then I was to tell them what it was. They had no idea what it was and were amazed. They thanked me for letting them take a look. I kind of enjoyed the experience, and would let anyone else have the chance to take a look, if it would help others with Coates disease or Dr’s.

    I have had this disease in my left eye since I was 5. I’m now 50. My present eye Dr. takes great care of me and my kids to make sure they don’t have it either. Neither of them does. My daughter is 25 (married) and my son is 19 and un-affected by this disease. I wear glasses now, bi-focals to read with and to protect my right eye.

    No one can tell when or how I will lose my eye, but as long as I’m not in any pain or the eye doesn’t look deformed, I keep it. I have been searching of information on this disease for some time and not much is known about it. I have compiled what I have found to help others with Coates disease.
    Mon, 17 Jul 2006
    So far, I’m doing ok, with near total vision loss. I can’t see much of anything, and it feels like the eye shuts down when I’m not thinking about it. The girls at work all know about it and let me know when they are coming up on me from the left. Although, they do sneak up on me at times on purpose! It’s all in good fun. Sometimes they stand behind me and wait till I turn around to see me jump! All kidding aside, they do know how serious it is. They ask questions etc. I’ve had Dr.’s ask me about it too. That is way I set up my web page. If I can help one person, I’m happy.

    Friday, July 21, 2006
    I guess I’m used to the surprises. Besides, I get them back. 🙂 I try to walk on the right side of the sidewalk, so I won’t bump into things or people. If I don’t hear someone coming up on my left, it does scare me. I’m used to my kids doing it to me when they were little. I find my left arm gets banged a lot. I try to give myself room but sometimes if I’m in a hurry, I bang it. I’m tempted to get some padding to wear on it, or one of those elbow things Skateboarders use. What do you think? I have a hard time giving or taking things too. I have no depth perception or judge of distance. I do drive though. I sometimes have difficulty in parking, unless I practice. I’m constantly looking in my mirrors. All my life, I’ve always tried to compensate and re-adjust to situations.

    July 3, 2008.
    I went to my eye Dr. this month. He told me that I was his only patient with Coates Disease, and possibly in Peterborough (where I live). So far it hasn’t affected my right eye, but he couldn’t see much of anything in my left eye. We still don’t know how much longer I will have the eye.
    August 30, 2010
    I saw my Eye Dr. today. So far nothing has changed much since last year. Now I have a grade 4 Cataract in my left eye. That’s the worse form. Nothing can help that. I also was told I have a small Cataract forming in my right eye, but not bad enough for surgery

  7. my 7yr old son is suffering from coats disease it came to our notice when he was 4yr old the doctors said there is no further treatment for this disease.ipray to god if their is some new treatment for this disease.my son is doing excellent in his academics i hope he will continue the same in future.

  8. Hi rumana,
    they say that there is no treatment for this disease but there are procedures to stop the disease from progressing and to save the remaining vision . laser and cryotherapy are the very common ones depending on what stage your son is. i hope and pray that something has been done already to save your son’s eye and his vision. my son was diagnosed when he was 11. he was treated with cryotherapy. after 5 treatments, the doctor hasn’t found any activity in the eye and we’re hoping that there won’t be any anymore. he still has his peripheral vision and i’m thankful to God for that.

  9. Hi my name is Bhaskar, my son has been diagnosed with Coats disease with 3B stage with total retina detached in his right eye who is 1 year old. So doctors have advised to wait for 6 weeks and then have one round of tests to do to see any progress to disease, if yes then we have to remove his eye totally. My concern is, does Coats disease affects the good eye in future (left eye) and what tests to be done regularly to keep it healthy. Will coats disease affects both eyes at same time or not? are we safe as it affected only one eye now, as most of them say it affects to only one eye?

  10. Jay, you might want to have a look at this post from November 2006:
    http://www.eyesapart.com/2006/11/11/coats-disease/

    There have probably been advances in understanding and treating Coats disease since this item was posted about 9 years, but this might be a starting place. You can check out the resources at the end of the item and also google Coats disease to find the latest info.

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