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Ask an Ophthalmologist—Retinal Diseases

Online Health Chat with Alex Yuan, MD, PhD

February 7, 2013

Introduction

Your retina—the light sensitive lining in the back of your eye—contains millions of special nerve cells that react to light and send electrical impulses to your optic nerve, which your brain converts into the images you see. The highly sensitive center of the retina—the macula—is responsible for central vision as well as picking up the detail in images.

Retinal diseases are the leading cause of blindness in adults in the United States. Trauma and postoperative conditions of the eye can also affect the retina. Diseases that involve the retina can be inherited, caused by infection or can result from unknown causes. Other diseases and medical conditions can also affect the retina, such as diabetic retinopathy. Some retinal diseases include: Diabetic Retinopathy, Macular Degeneration, Stargardt Syndrome (Juvenile Inherited Macular Degeneration), Retinal Detachment, Degenerative Myopia, Uveitis, Macular Edema, Macular Telangiectasia, Retinoschisis, Choroideremia, and Retinal Dystrophies, such as Usher Syndrome, Rod-Cone Dystrophy, Bardet-Biedl Syndrome, Refsum Disease, Best Disease and Oguchi Disease.

A healthy diet, exercise, blood pressure control, blood sugar and cholesterol control, and weight management, along with protective eye gear and sunglasses, are important to maintaining good eye health. Yearly eye examinations are crucial to ensuring good vision, but any sudden change in vision (such as floaters or areas of vision loss) should be reported immediately to your eye doctor.

 

For More Information

On Cleveland Clinic
At Cleveland Clinic’s Cole Eye Institute, our retina staff has the expertise to accurately diagnose and offer world-class treatment for retinal diseases.

Cole Eye Institute is among the world’s most advanced eye institutes, ranked by U.S.News & World Report as one of ‘America’s Best’ ophthalmology programs, and is the top-ranked program in Ohio. Our fully integrated model helps us provide patients with quick and easy access to specialty and subspecialty care for a wide spectrum of eye conditions—from the routine to the complex. Cole Eye Institute has extended its quality ophthalmic care to several locations throughout Northeast Ohio, allowing patient access to ophthalmologists closer to their homes.

For more information about the Cole Eye Institute, eye conditions and treatment, please visit: http://my.clevelandclinic.org/eye/default.aspx

If you would like additional information on retinal diseases, feel free to download our free treatment guide at: http://www.clevelandclinic.org/lp/retinal-diseases/index.html

On Your Health
MyChart®: Your Personal Health Connection, is a secure, online health management tool that connects Cleveland Clinic patients with their personalized health information. All you need is access to a computer. For more information about MyChart®, call toll-free at 866.915.3383 or send an email to: mychartsupport@ccf.org

A remote second opinion may also be requested from Cleveland Clinic through the secure Cleveland Clinic MyConsult® website. To request a remote second opinion, visit eclevelandclinic.org/myConsult


For Appointments

To make an appointment with Dr. Yuan or any of the other specialists at the Cole Eye Institute, please call 216.444.2020 or call toll-free at 800.223.2273, ext. 42020. You can also visit us online at www.clevelandclinic.org/eye


About the Speakers

Alex Yuan, MD, PhD is an associate staff physician at Cleveland Clinic’s Cole Eye Institute. Dr. Yuan completed his fellowship in vitreoretinal surgery at Cleveland Clinic after his residency in ophthalmology at Jules Stein Eye Institute, University of California School of Medicine in Los Angeles. He completed an internship at Forest Park Hospital in St. Louis after earning his medical degree and doctorate in neurosciences from Washington University School of Medicine in St. Louis, Missouri.

Dr. Yuan’s specialty interests include the medical and surgical diagnosis and treatment of retinal disorders. He has published several articles in peer-reviewed journals, and maintains several research awards and honors.


Let’s Chat About Retinal Diseases

 

Detached Retina

Detached Retina Diagnosis

laura628: I've started seeing yellow flashes in the outer corner of one eye. This happens in a room that is not well lit. From what I've read, this could possibly be a torn or detached retina. Is that correct? I have not seen my eye doctor yet about this, but will be making an appointment soon.
Dr_Yuan: You should make an appointment with your ophthalmologist for a dilated eye exam. You are correct; flashes of light can be due to a torn or detached retina.

blpayton: I am 49 years old, and began seeing floaters when I was 46 years old. They have become increasingly annoying, and I am very nearsighted. Recently, I have felt pressure behind my eyes, dryness and have difficulty reading up close with my contacts in. My 51-year-old brother just had a retinal detachment, which is the first to occur in our family to the best of my knowledge. I have just had my eyes checked and was told there is no issue to be concerned with. Should I get a second opinion? Is there anything I can do to relieve the pressure behind my eyes? Could it be due to sinus issues? How can I take good care of my eyes to prevent further complications?
Dr_Yuan: A sudden onset of floaters may be due to the separation of the vitreous (gel) from the retina. This is a common condition and is benign by itself. However, vitreous separation can lead to retina tears or retina detachments that must be treated. If you have already had a comprehensive dilated exam and your doctor did not find any retinal tears, you should be fine. However, if you have new or worsening floaters, flashing lights or a loss of vision—including your peripheral vision, then you should see your ophthalmologist right away. You can develop new tears after your initial visit, so a follow-up examination is recommended.

Pressure behind the eyes can be due to many things and sinus problems can certainly be one of them.

kay_kay: One year ago I went blind when my retina detached.  I had surgery and 14 days later it detached again, so I had another surgery via the scleral buckle technique to keep my retina attached. My retina had torn in two places, leaving me partially blind. Six months later the oil ball was removed, but with leaving some oil still in my eye. Approximately three months later I had cataract surgery. Three days later, my retina detached again, but the doctor was able to do a laser procedure to reattach it in his office.

I still have pain, headaches and my eye throbs. My doctor said I should not have pain, and he doesn't see anything wrong with my surgical eye other than the damage that is not repairable. He also prescribed Restasis® for both of my eyes because of chronic dry eye. Now I have a floater (that is very annoying) in my good eye. It has been there longer than thirty days. Both of my eyes are super sensitive to light. Do you have any suggestions? Is the pain in my surgical eye common a year after surgery?
Dr_Yuan: Make sure you get a full dilated examination in your good eye to rule out any tears of the retina, which can be treated to prevent a retinal detachment.  Floaters are common and may not be anything serious, but you won’t know unless that eye is examined.

Pain in the surgical eye one year later is uncommon, but I have encountered it.  Sometimes it is something simple that is not related to the retina—for example, a loose suture, dry eye, or an eroded scleral buckle. There are many possibilities, and you should get a comprehensive examination.

Gatorfrog5:    I am having extreme pain in my eyes. It comes and goes, and does not last long. I am wondering what could be causing this in my eye. I don't know if it is related to the retina or not, but I thought I would ask.
Dr_Yuan: Retinal problems do not usually cause eye pain because there are no pain receptors in the retina. I advise you to get a comprehensive exam from an ophthalmologist. Inflammatory conditions can cause the eye to ache and will cause light sensitivity.

 

Treatment of Detached Retina

miyashl: What are the best options for a detached retina?
Dr_Yuan: Detached retinas can be repaired by several methods, including placing a scleral buckle with a gas bubble in your eye, performing a vitrectomy, or performing a procedure called a pneumatic retinopexy. Combinations of these surgeries can also be performed. There isn’t a single best option. The decision depends on several factors. You should discuss your options with your retina doctor given your particular scenario.

JMartin: What are the special considerations for treating retinal detachment in patients with Marfan syndrome?
Dr_Yuan: Patients with Marfan syndrome often have weak zonules (fibers that connect to the lens), causing their lens to be subluxed (malpositioned). These patients also have a higher tendency to have retinal holes and tears. However, the approach to repairing a detached retina in a Marfan syndrome patient is no different than in any other patient—keeping in mind the likelihood of a subluxed lens. Often, the eye is filled with gas after retinal detachment surgery. If the lens is subluxed, then the gas could potentially worsen the subluxation, or can move from the vitreous cavity into the anterior chamber. (The gas should stay behind the iris and pupil, but sometimes it can move in front of the pupil from a subluxed lens.) Sometimes it becomes necessary to remove the lens completely, in which case a lens may be placed at a later time or one may be sutured back in place.

 

Scleral Buckle

liesel: In January 2012, I had surgery for a detached retina during which I received a scleral buckle (sponge sewn onto the sclera). I had an extremely long, painful recovery. I saw double immediately, and in February 2012 I had eye muscle surgery to help with left hypotropia. That surgery was not successful, and I still saw double. I had a second eye muscle surgery in September with a different surgeon. That surgery helped the double vision a lot, but I still see double when I look up. Also, first thing in the morning, my eye is in a somewhat locked downward position for about one-and-one half hours. I will not have any more muscle surgeries. I always feel an irritant, which I believe is the sponge. Can that sponge eventually cause problems? How great is the possibility of re-detachment or the buckle disintegrating? I am 70 years old. I did not have the gas bubble creation.
Dr_Yuan: We usually do not recommend removing the buckle. However, sometimes this can be done, and the retina will remain attached. You should be examined to make sure the scleral buckle has not eroded through the conjunctiva or the covering over the white part of the eye. With modern scleral buckle components, the likelihood of the band disintegrating is very low.

 

Intraocular Lenses

njgiraffe: I am a 49-year-old female with Marfan syndrome. I have had two detached retinas in one eye and one in the other. I've also had both natural lenses removed, along with a vitrectomy and a sclera buckle in one eye. I know that any surgery has risks, but do you think it would be safe for me to have intraocular lenses implanted since I have Marfan syndrome? (I didn't get them initially because the doctor did not have time to measure my eyes, since it was an emergency detached retina.) Is there a strong chance of having another detached retina from the surgery? I could wear regular contact lenses again (which I have worn for 20 years). However, they are now so uncomfortable since I have dry eye. What would you have me do?
Dr_Yuan: Patients with Marfan syndrome can have eye implants placed safely. Sometimes in an emergency surgery, a lens is not placed in your eye as is the case with you. You should come in for an evaluation. Surgery to implant lenses in your eyes might help you. Usually for Marfan syndrome patients, the lens implant will be placed in front of your iris instead of behind it.

 

Complications Following Vitrectomy

eyewonder: I had an epiretinal membrane removed in July. Following surgery, a cataract formed as predicted by my doctor. Since I only see out of this one eye, it's causing me a great deal of difficulty. How long after surgery should I wait for the cataract to ‘ripen’?
Dr_Yuan: Developing a cataract after vitrectomy surgery is common. There is no set timeframe for recommending cataract surgery. If your vision has declined and if the examination confirms it is due to cataracts, then you should have cataract surgery.

 

Reducing Risk of Retinal Detachment

BQ_1: How does one reduce the risk factors associated with retinal detachment?
Dr_Yuan: Most of the risk factors cannot be modified, including age, severe near-sightedness and certain genetic disorders. However, avoiding eye and head trauma can help. Eye surgery can also increase the risk for retinal detachments. If your vision could be helped by eye surgery, then the potential benefits are likely to exceed this risk.  Consult with your ophthalmologist for specific risk factors that may pertain to you.

 

Nutrition for Retinal Detachment

BQ: What impact does nutrition have on the prevention of retinal detachment?
Dr_Yuan: I am not aware of the effects of nutrition on preventing retinal detachments.

 

Pseudohole of the Macula

Diagnosis of Pseudohole of the Macula

Shalu: I have a ‘pseudo’ hole in my macula due to scarring. What causes this scarring and what can I do to improve my sight in that eye?
Dr_Yuan:
Pseudoholes can sometimes be caused by a wrinkle in the retina called an epiretinal membrane and I presume this is what you mean by ‘scarring’. Epiretinal membranes may be caused by inflammation, retinal holes or tears, or may be idiopathic (i.e., no known cause).  If the epiretinal membrane is causing severe distortion of the retina, then surgery can sometimes help to improve this.  Pseudoholes can also be caused from traction on the retina from the vitreous (gel), a condition called vitreomacular traction.  Surgery may be able to help in these cases as well.  A new drug has also been approved for the treatment of vitreomacular traction.  This drug is injected into the eye.  There are also some cases of pseudoholes that cannot be helped by surgery.  You should consult with your retina doctor.

Pseudoholes in the macula can sometimes affect vision, but often do not affect vision. If there is significant epiretinal membrane or scarring that can be removed by surgery, then one option is to remove the epiretinal membrane. You should have a full evaluation by a retina surgeon.

 

Macular Degeneration

Diagnosis of Dry Macular Degeneration

nance: In late 2011, I was diagnosed with early, dry age-related macular degeneration (ARMD). Since this time, I have been taking the AREDS 2 supplement every day. Last week I had cataract surgery on right eye to be soon followed by surgery on left eye. My vision is dramatically improved in the right eye and I expect the same will be true when the left eye is operated. My question concerns the progression of the dry ARMD. At 81 years of age, can I be hopeful that the progression of the MD is very slow and that I will enjoy my improved vision for a very long time before the macular degeneration begins to worsen? Is there anything more that I can do to slow the progression?
Dr_Yuan: There is a wide range of progression rates in patients with AMD.  Some of it depends on what stage dry AMD you were diagnosed with. AREDS original eye vitamin formula is recommended for patients with stage 3 or higher AMD. If you have early AMD or stage 2 AMD, then regular exams are recommended to see if your disease has progressed. The results of the AREDS 2 trial have not been released yet. (In the AREDS 2 study, the eye vitamin formulation differs from the original AREDS vitamins, with additional dietary supplements.)

auntlinnie66:            I have dry macular degeneration. Is there any way to improve my sight? I take vitamins, but I have not had a prescription change in four years.
Dr_Yuan: You should have a comprehensive examination to determine if you have other conditions that may be affecting your eye sight. A good refraction (eyeglass prescription) alone can sometimes help, but in severe cases of dry AMD, then a visit to your low-vision specialist can help.

auntlinnie66: If I was to use a program named ‘The Program for Better Vision™’ by Martin Sussman, would it help?
Dr_Yuan: I'm sorry; I am not familiar with this program.

 

Diagnosis of Wet Macular Degeneration

scbkgma_1: My retinologist has me coming back at four-month intervals, but I can come back sooner if I feel that I need to. How do I know when my eye is in the ‘wet’ stage, as I know this is very serious and should see a specialist immediately?
Dr_Yuan: You can monitor yourself with the use of an Amsler grid. If you see wavy lines, a smudge, or are missing small areas in your vision, then these can be signs of conversion to wet macular degeneration.

scbkgma_1: I have macular degeneration and was also diagnosed with atrial defibrillation for which I was prescribed warfarin. Since I have been taking that, my eye seems to be more blurry in the central vision and I cannot read nearly as well. Is that something that is happening normally, or is warfarin making it to deteriorate more quickly?
Dr_Yuan: If you have bleeding from wet macular degeneration, then the warfarin can make the bleeding worse. However, warfarin itself does not increase your chances of having wet macular degeneration or having a bleed. I do not believe your blurry vision is related, but you should be evaluated to make sure you do not have wet macular degeneration.

Phylkr            : I am getting check ups every four months by my retinologist. When do I know that my dry macular degeneration has become wet? I know it is very crucial to see a doctor immediately. I am also on warfarin and am noticing that my vision is diminishing faster. Is there a correlation between warfarin and macular degeneration?
Dr_Yuan: There is no correlation between warfarin and worsening of macular degeneration.  However, if you do develop a large subretinal bleed as a complication from your macular degeneration, warfarin may make the bleeding worse. Warfarin does not cause the bleeding to occur in the first place. Some signs for conversion to wet AMD might be a loss in your central vision, straight lines appearing as wavy lines, or a blur in your central vision. Your retina doctor will be able to confirm this through examination and special tests that are done in the office.  If you notice new symptoms, you should consult your retina doctor.

 

Light Sensitivity with Wet Macular Degeneration

Kasparov: I have wet macular degeneration and have received Avastin® (bevacizumab)injections for many years. I also receive Eylea® (aflibercept) injections every four to eight weeks. I have developed an extreme sensitivity to light and wonder if there is anything I can do about it except wear sunglasses.
Dr_Yuan: Sensitivity to light can be due to many factors—some of which are not related to macular degeneration and injections. You should ask your retina doctor to examine you for signs of inflammation or other causes for increased light sensitivity.

Kasparov: Can anything be done for extreme light sensitivity secondary to macular degeneration other than sunglasses?
Dr_Yuan: Other causes for light sensitivity should be ruled out first. If it is indeed due to macular degeneration, sunglasses with polarizers or regular glasses with polarizers are your only option.

 

Injection for Wet Macular Degeneration

fz4h4d: Is there any evidence to suggest that receiving injections for wet macular degeneration on a routine basis (even if the eye is dry) is more effective at preserving vision than receiving the injections on an ‘as-needed’ basis?
Dr_Yuan: A large randomized, controlled study by Cleveland Clinic Cole Eye Institute Chairman, Dr. Daniel Martin recently showed that monthly injections did have a small benefit in improving vision as compared to ‘as-needed’ injections. However, that difference was small and not likely to noticeable to most patients. The decision to treat monthly or ‘as-needed’ should be determined by your discussions with your retina doctor. Both regimens were shown to be highly effective and retina doctors use both methods.

 

Treatment for Macular Degeneration

cmcneil: Do you have treatments for myopic macular degeneration?
Dr_Yuan: You should be evaluated for choroidal neovascularization if you have had recent vision loss with myopic macular degeneration. This can be treated and is one cause for vision loss with this condition. Myopic macular degeneration in itself is not treatable. However, complications from this condition can be treated to help improve vision.

Davids: What is the best way to treat age-related dry macular degeneration?
Dr_Yuan: Currently, there is no cure for dry macular degeneration. AREDS original formula eye vitamins are recommended to prevent the progression to advanced AMD. (The AREDS 2 formula is new, and the results of that clinical trial have not been released yet.) There is lots of research in this area, and perhaps in the next few years we will see something promising.

 

Eye Nutrition and Supplements for Macular Degeneration

susievip: What are your feelings on the use of eye vitamins? If you eat a healthy colorful diet, should you waste your money on them? I am 55 years old and do eat a colorful diet. I have heard food is better than vitamins. What are your thoughts?
Dr_Yuan: AREDS original formula eye vitamins were found to be helpful for patients with diagnosed stage 3 or higher macular degeneration. If you have healthy eyes, I would not recommend the use of eye vitamins. Eating a healthy, well-balanced diet is, however, recommended for eye health.

Shalu: What foods and/or supplements are especially good for the eyes?
Dr_Yuan: For healthy eyes, I would recommend a balanced diet with plenty of fruits and vegetables. For patients with macular degeneration, eye vitamins have been shown to delay the progression to advanced stages.

nance: In late 2011, I was diagnosed with early dry age-related macular degeneration (ARMD). Since then, I have been taking Bausch & Lomb PreserVision® AREDS 2 Formula everyday to try to stop the progression of early dry age-related macular degeneration.  Last week I had cataract surgery on my right eye, and will soon have surgery on my left eye. My recent cataract removal has dramatically improved my vision in the right eye, and I expect the same will be true when the left eye is operated on. My question concerns the progression of the dry ARMD. At 81 years old, can I hope to enjoy my enhanced vision for a very long time? Is there a very slow progression of early dry macular degeneration? What more can I do aside from taking the PreserVision® to try and stall the progression of this disease?
Dr_Yuan: There is a wide range of progression rates in patients with AMD.  Some of it depends on the stage of dry ARMD you were diagnosed with. The AREDS original formula is recommended for patients with stage 3 or higher ARMD.  If you have early ARMD or stage 2 ARMD, then regular exams are recommended to see if your disease has progressed. The results of the AREDS 2 trial on dietary supplements have not been released yet. Therefore, no recommendations can be made about the AREDS 2 formula at this time.

PreserVision® and other AREDS original formulation eye vitamins have been shown to reduce the risk of developing advanced stage macular degeneration in patients who already have signs of macular degeneration. You should also eat a healthy, well-balanced diet and follow up regularly with your retina specialist. Finally, you should monitor your vision for sudden changes, such as wavy lines or missing spots in your vision.

Kellis:            Are there supplements which are known to decrease the likelihood of macular degeneration? If so, which ones do you recommend?
Dr_Yuan: I recommend the PreserVision® AREDS Formula (original formula), or any other brand of vitamins with the original AREDS formula until data is reported from the AREDS 2 trial.

 

Age-related Macular Degeneration Research

kathyrlab: Concerning age-related macular degeneration (AMD), have you read or heard of any results of the ARED2 trials? What is your understanding of taking low-dose aspirin with dry and wet AMD?
Dr_Yuan: The Age-related Eye Disease Study (AREDS) was a large trial evaluating the efficacy of vitamin supplementation in reducing the risk of advanced AMD. The trial found high-dose vitamins (i.e., vitamins A, C, E, and zinc) reduced the rate of advanced AMD in patients who were already at risk for developing advanced AMD. The AREDS vitamins do not prevent AMD in patients who do not already have the diagnosis.

AREDS 2 is a new study evaluating the efficacy of dietary supplements including lutein, zeaxanthin, omega 3 fatty acids (DHA), and eicosapentaenoic acid (EPA) in reducing the risk of advanced AMD. The results have not been reported yet, but interestingly many companies have started to sell supplements with these supplements. There is currently no proven efficacy to the AREDS 2 formula. Results are expected later this year and until then, no recommendations can be made regarding the AREDS 2 formula.

Several previous studies have found no additional risk for advanced AMD in patients taking aspirin.  Recently, aspirin use was found to be associated with a risk of wet AMD in a retrospective study (i.e., a study where the risk factors were looked at after the patients were already identified with the disease). Caution must be taken when interpreting these results. They found no ‘dose response’ related to the risk of AMD (meaning that patients who take a higher dose of aspirin were not at any higher risk), but they did find an association with regular aspirin use with wet AMD. This may not be a causal relationship (meaning one precedes and causes the other). More studies will need to be done to answer this question. Retrospective studies can have many biases. (For example, perhaps the patients who were not taking aspirin died at an earlier age and thus never had a chance to develop advanced AMD. Since age is the leading risk factor for developing advanced AMD, this could be a bias). What we do know is aspirin saves lives by having many cardiovascular benefits.  I believe the choice to stop taking aspirin in patients at risk for AMD should be individualized. You should discuss the risks and benefits with your primary care doctor or cardiologist.

 

Retinitis Pigmentosa

Retinitis Pigmentosa Diagnosis

ChBy: What causes flickering lights in the peripheral vision in retinitis pigmentosa (RP)? This was my first obvious symptom, which has persisted for 15 years.
Dr_Yuan: The retina—as a light sensing tissue—relays sensory information as light. Stimuli to the retina would be interpreted by the brain as light even if that stimulus is mechanical or traumatic. Although the exact mechanism is unknown, it might be related to the loss of photoreceptors (cells that are sensitive to light) in RP.

 

Retinitis Pigmentosa Treatment

LucyAnn: What course of treatment, if any would you pursue if you were diagnosed with RP?
Dr_Yuan: There are some clinical trials for treatments for RP. However, there is no treatment currently available. If you are eligible for these studies, please consider participating. Please see the posting on research for the website to check on these trials.  Just announced last week was the approval of an artificial retina for some patients with RP. You should consult your retina doctor for more information.

 

Retinitis Pigmentosa Research
Hale:  About three years ago I was diagnosed with retinitis pigmentosa. I would like to know if there are any clinical trials or nutritional studies being done for this eye condition.
Dr_Yuan: Try this website http://clinicaltrials.gov and query retinitis pigmentosa.  You will get a list of all the trials that are currently recruiting patients.

 

Stargardt Disease

Stargardt Disease Diagnosis

Dwald: My 14-year-old son has been diagnosed with Stargardt disease, and I have a 16-year-old son with no symptoms. Is he in the clear? What can my 14 year old expect?
Dr_Yuan: Stargardt disease usually presents itself early in life—in the teenage years or earlier. However, there are variants of the disease with late onset that have been reported. You should have your 16 year old evaluated. Most forms are recessive (meaning an affected individual must inherit the mutation from both parents), but some dominant forms have been described.

Your 14 year old will be able to lead a normal healthy life. His vision will likely be impaired, but children are capable of adapting in remarkable ways. He should have regular eye examinations to optimize his vision and wear eye protection. If his vision is severely affected, he should visit your local low-vision specialist who can make further recommendations.

Krauser: My father has been diagnosed with Stargardt disease. He is 84 years old. We were told not to use vitamin A any longer. We are waiting for a study to become available for his treatment at University of Michigan Health System. He has great peripheral vision, but his central vision is dark. He has to turn his head to see, and uses a hand-held magnifier of 6X for reading. Is there anything we can do to help him? Do you have any studies at Cleveland’s Cole Eye Institute that would help him?

Additionally, my sister has been diagnosed with Usher syndrome (because she is deaf). She is 55 years old. Yet, the DNA shows only one of the genes that is a known part of Usher syndrome (MY07A) and the other gene has not shown up in any of the known research. She was born deaf, but didn’t start losing her sight until she was 50 years old. This isn’t consistent with the literature I have read. She recalls her younger days of seeing and being able to drive her car. She has lost her peripheral vision, so they call it ‘tunnel vision’ now. She has good days and bad days. She is considered ‘legally blind,’ but she still has vision to see people and TV. She is still able to clean the house, sometimes missing a spot, due to the vision. Doctors are calling it retinitis pigmentosa (RP). The room lighting has to be bright for her, but sunlight bothers her, even on cloudy days. Could she lose all of her vision? Will RP go into remission? How can I help her?

My eyes are good. Do I need to be concerned with my eyes as I grow older with family members having these two conditions?
Dr_Yuan: It is very unusual to see both Stargardt and Usher syndromes in the same family. They are inherited in different ways. The appearance of both Stargardt and some forms of retinitis pigmentosa or cone/rod dystrophies, however, can be similar. I wonder if there may be a connection between your father’s and sister’s diseases. I advise you to bring yourself, your father and your sister in for evaluation with Dr. Elias Traboulsi at Cleveland Clinic Cole Eye Institute. As the Director of the Center for Genetic Eye Diseases at Cleveland Clinic's Cole Eye Institute, Dr. Traboulsi is an expert on retinal dystrophies and has agreed to see your family if you would like. He can be contacted at 216. 444.7152.

 

Vitelliform Macular Dystrophy (Best Disease)

Vitelliform Macular Dystrophy (Best Disease) Diagnosis

IslandGirl12: What is the difference between macular degeneration and vitelliform dystrophy? The same treatment is used for both and my understanding is that vitelliform dystrophy can be stopped from getting worse with the same shot.
Dr_Yuan: Age-related macular degeneration is completely different from vitelliform dystrophy. Vitelliform dystrophy (or Best dystrophy) is an inherited disorder causing central vision loss late in life. Symptoms can start to appear in your 30s, but good vision is usually maintained until much later. The similarity is there is a loss of the central vision while peripheral vision is maintained. Age-related macular degeneration affects elderly patients and the mechanism is completely different from vitelliform dystrophy.  Wet macular degeneration is treated with injections into the eye, lasers or, in rare cases, surgery. Radiation therapy is also currently being evaluated for the treatment of wet macular degeneration. These treatments are not used to treat vitelliform dystrophy. The one exception is if a complication from vitelliform dystrophy occurs—known as choroidal neovascularization, then some of these treatments may be helpful.

Brandy-S: I have been diagnosed with Best disease based on a specialist examining my eyes with a camera and a dye test.  I want to know if there is a way to be sure this is what I have, and if there is anything at all I can do for this?  I want to be 100 percent sure that I have this genetic disease. I just turned 40 years old, and do have vision loss in the central vision of the right eye. However, I am told I have it in both eyes, and now I am starting to see some signs in left eye! What is the progression rate of this?
Dr_Yuan: Your specialist can perform an electro-oculogram (EOG) to make the diagnosis.  There is also a genetic test available. A sample of your blood can be sent for this test, if the EOG and other tests are not confirmatory. The disease progresses through several stages, and your central vision may become affected at the latter stages. Usually, vision does not decline until late in the disease process. Some patients maintain good vision late in life. Severe vision loss is usually from atrophic lesions (damaged areas) or sometimes from choroidal neovascularization (new blood vessel creation).  If choroidal neovascularization is present, some treatments may be available.

 

Nutrition and Supplements for Vitelliform Dystrophy

IslandGirl12_2: Are eye vitamins helpful with vitelliform dystrophy?
Dr_Yuan: Not that I am aware of.

 

Branch Retinal Artery Occlusion

Branch Retinal Artery Occlusion Diagnosis

Kahuna: I have been diagnosed with a branch retinal artery occlusion in my right eye in December 2012. In November, I was diagnosed with moderate-to-severe aortic valve disease. I had a carotid ultrasound on January 30, but I do not have the results. I am 76 years old, and in otherwise good health. Please advise me of my best course of action.
Dr_Yuan: Patients with a history of branch artery occlusion should be followed by their ophthalmologist for the development of complications such as glaucoma from neovascularization. You should have regular follow-up visits with your ophthalmologist to determine if further treatment such as laser photocoagulation is necessary. You should also follow up with the results of your carotid ultrasound, and consult with your primary care doctor or vascular surgeon if necessary.

 

Choroidal Nevi

Choroidal Nevi Diagnosis

Elizabeth: During my eye examination last week, the doctor discovered a small nevus on the left retina. It is new this year. I also have dry eyes, blepharitis, and swelling of tissue around the eye and face on my left side. My left eye waters and is photosensitive.  I have had melanoma with surgery on my right collarbone area in 2009. Is the nevus any cause for concern considering the melanoma diagnosis? How often should I be followed by an ophthalmologist?
Dr_Yuan: Choroidal nevi are relatively common. These small, flat, low-risk lesions can be followed for changes. It is not likely to be related with the melanoma on your collarbone.  Choroidal melanoma are primary tumors of the eye (meaning they originate in the eye). Follow-up intervals should be individualized.

 

Center Serous Retinopathy

Center Serous Retinopathy Treatment

Infiniti72: I live between Harrisburg, PA and Baltimore, MD. Is there anyone who can recommend the best facility to go to for treatment and advanced assistance with center serous retinopathy?
Dr_Yuan: Probably the best place is Wilmer Eye Institute at Johns Hopkins Hospital in Baltimore.

 

Center Serous Retinopathy Research

Infiniti72: I am 40 years old and I have damaged retina cells from center serous retinopathy. How positive do you feel that there will be something developed to cure or replace these damaged retina cells in the upcoming years?
Dr_Yuan: There is great interest in regenerative therapies for the retina, and lots of research in this area. I think we could potentially see progress in this area in the upcoming years.

Infiniti72: Has there been any new development with any type of telescopic implant devices?
Dr_Yuan: There is a device approved for macular degeneration, but I am unaware of its use in patients with central serous retinopathy. If you have additional questions about this, you might want to discuss this with your ophthalmologist.

Infiniti72: Has there been new research that has brought us closer to a cure of any kind for center serous retinopathy?
Dr_Yuan: Recently, mineral corticoid receptor blockers such as Inspra® (eplerenone) were found to be effective in treating central serous retinopathy.

Infiniti72: What progress has been made with the engineered photo-switch for nerve cells in the eye and brain known as MPC088?
Dr_Yuan: I’m sorry; I am not familiar with this particular technology, but the possibilities for a photo-switch are intriguing—especially if they can be delivered to surviving cells in the retina. Interestingly, last week an artificial retina was approved for patients with retinitis pigmentosa. This device might be beneficial in other patients with diseases where the photoreceptors are affected, but the other neurons in the retina are left intact (perhaps in very severe central serous retinopathy).

 

Other Diagnoses

Fuchs Endothelial Corneal Dystrophy

Sebastian:  My doctor says that I am a Fuchs patient. What does that mean?
Dr_Yuan: It is difficult to answer this question because Fuchs was a very prolific ophthalmologist with many different diseases named after him.  A few examples include: Fuchs endothelial corneal dystrophy, Fuchs heterochromic iridocyclitis, Fuchs adenoma, Dalen-Fuchs nodules, and Fuchs spots. Usually a Fuchs patient refers to Fuchs endothelial corneal dystrophy, which causes blurred vision that is sometimes worse in the morning.  You should consult with your ophthalmologist for clarification.

 

Cataracts

asgalian: When one has white halos around lights at night, is there specific treatment or certain glasses that can help reduce this vision impairment?
Dr_Yuan: You should be evaluated to make sure you do not have cataracts.

 

Optic Atrophy

Paula-M: My daughter was diagnosed with optic atrophy of unknown cause. She is now totally blind in her left eye. The doctors want her three young sons tested as well. I have an extremely large family, and no one has this. What may have caused this? Other than telling me that my daughter has ‘pale nerves’ and should be checked, no explanation was given! What research is being done? Is there any hope for reversal? Could an injury have caused this? As her mother, my only eye condition is extreme near-sightedness (-11/50).
Dr_Yuan: Optic atrophy is a very broad term and not a specific diagnosis. A clinical description of a pale nerve suggests permanent damage to the optic nerve. There could be many causes. Once atrophy has set in, then there is little chance for reversal. Injury is one possible cause for optic atrophy, but there are many other causes. Your daughter should be seen by a neuro-ophthalmologist.

 


Closing

Cleveland_Clinic_Host: I'm sorry to say that our time with Cleveland Clinic specialist Alex Yuan, MD, PhD is now over. Thank you, Dr. Yuan, for taking the time to answer our questions today about retinal disease.
Dr_Yuan: Thank you for your questions.


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