Coats Disease can cause strabismus and blindness
Written on November 11, 2006 – | by Lois (admin)
See 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
2 Responses to “Coats Disease can cause strabismus and blindness”
By Susan Hedeen on Feb 13, 2007 | Reply
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
By Sarah on May 4, 2008 | Reply
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??