DAN: Dr. Sonkin, we greatly appreciate the time you are taking to share your knowledge and experience with macular diseases. Welcome to MDList, and I hope you will enjoy meeting the members of our Internet community. The session is now open for questions.

KAY: I was dxed with MD in both eyes on June 6. I look at the Amsler Grid daily. Everything is clear as can be, no wavy lines or blank spots. The retina specialist took tests of my eyes and many pictures. He looked at the pictures and said "You are lucky, you have dry MD in both eyes". He walked out of the room. End of discussion?? I had cataract surgery on May 7, and ten days later I saw a purple duck that came and went. I went to the doctor and he sent me to the retina specialist. The image lasted several days, and I haven't seen it since. My visual acuity is very good. No problems reading or driving. What is your opinion of this diagnosis? I am inclined to believe that the RS is wrong.

DR. SONKIN: The initial diagnosis of "dry" AMD is likely accurate. After cataract surgery, many things can occur that can cause temporary symptoms. It is normal to have inflammation inside the eye, and this usually resolves on its own and with the help of normal postoperative eye drops. This inflammation can cause symptoms consistent with what you described. It is also possible that you developed mild "swelling" in the macula, which we call cystoid macular edema or CME. This affects the same part of the retina as AMD, but is a different condition. It often gets better on its own, but sometimes requires special eyedrops or other treatments. This may have caused your symptoms. If your macular degeneration has converted to the wet form, i.e. you have developed abnormal blood vessels under the retina known as CNVM (choroidal neovascularization), then your symptoms would likely be worse and persistent. It is important to monitor your amsler grid daily. With any changes you should see your retina doctor asap. Catching the wet form before it reaches the center of the macula (the fovea) is very important and can often prevent or delay the more severe visual problems. Catching the wet form early rather than late if it develops right in the center will minimize the damage and visual loss. There are other things that might have developed in your eye, but the above are the most likely things. It is difficult to be more specific without actually seeing your eyes. You should follow-up with your retina doctor as scheduled and call him if you develop any new symptoms.

BARBARA: My sister and I have been diagnosed with adult vitillform dystropy, or Best's disease. Would you please explain the diagnosis further? What is the likelihood that my sons ( early 40's) or grandchildren will inherit the same vision problems?

Recently, I have noticed a severe light intolerance, which is reduced with UV protection in sunglasses and a wide brimmed hat. Is there anything else that can be undertaken to reduce the effect of bright light into the macula?

DR. SONKIN: Best's Disease is an autosomal dominant hereditary retinal disease. This means it is inherited, and there is a 50% risk of passage of the disease from an affected individual to an offspring. Each of your sons were at a 50% risk for the disease. Best's disease is usually easy to diagnose by exam and confirm by EOG (electrooculogram) at an early age. If a given son is not affected, the risk for that son's children for Best's disease is essentially zero, unless your daughter- in-law happens to have the disease, which would be very unlikely. If one of your sons is affected, each of his children would then be at a 50% risk.

No retinal disease is good to have, but if you had to pick one, Best's disease is not as bad as many. Usually the visual loss is not as severe and it usually occurs later in life. This is not true in every case, and I am not trying to minimize the effect it has had on you and your sister, but it does have a better prognosis. In contrast to the visual loss, the clinical appearance is very striking and impressive, with an "egg yolk"-like appearance to the macula. Other associated problems can develop late in the disease but are rare. This includes bleeding and choroidal neovascularization, much like we see with AMD.

You do have an inherited degeneration of your macula, but this is not the same as "age-related macular degeneration". Sensitivity to light is a nonspecific symptom, and is not necessarily related to your macular problem. It could be cataracts or normal light sensitivity.

JUDY: I received two photocoagulation laser treatments about two years ago, within six weeks of each other, due to a severe hemorrhage in my right eye.

As a consequence, I lost all central vision very quickly in that eye with a visual acuity of 20/800. I was told I had to have this surgery to avoid profound vision loss. Apart from "social blindness", how much more profound can you get with no central vision remaining in one eye?

Photodynamic therapy appears to be a better treatment option than photocoagulation, but is only performed on about 20% of the Wet MD eyes examined. Is that statistic correct?

Somehow I am under the impression that these two laser treatments are, in reality, only bandaid solutions, but superior to doing nothing. I am wondering if a saner approach would be to simply try harder to accept our vision loss rather than running after treatments that might or might not work.

My retinal surgeon thinks that macular translocation, once perfected in a few years, might be a better treatment choice for the future. Would you by chance have recent statistics on the validity of these two lasers as opposed to doing nothing, during this time frame, when neo-vascularization occurs?

DR. SONKIN: I am going to try to answer your question in sections.

Thermal laser was shown to be beneficial in a large study done approx 20 years ago called the MPS (macular photocoagulation studies). It showed conclusively that thermal laser was beneficial in preventing visual loss for wet AMD (CNV) not involving the fovea if it is a certain type called "classic" as defined by a fluorescein angiogram (FA). This still holds true today for nonfoveal classic CNV.

Before the approval of PDT, many retinal surgeons also used thermal laser to treat classic CNV that does involve the fovea. This was also shown to be better than observation only by the MPS trials. Thermal laser to the foveal region, however, does not improve vision or necessarily prevent further loss. The MPS study showed that it does reduce the likelihood of SEVERE visual loss (6 lines on the eye chart) versus observation only. The analogy I often used with my patients is that thermal laser to the fovea is similar to amputating a finger to save a whole arm. In other words, the thermal laser to the fovea results in an immediate and permanent blind spot, and often a drop in vision. But this blind spot is hopefully smaller and not as dense (or dark) as the natural blind spot that would have resulted from observation only.

Most retina surgeons now treat fovea-involving CNV with PDT because it minimizes the damage to the overlying retina and hopefully minimizes the visual loss. Neither thermal laser or PDT is a cure. Hopefully research will lead to ways to prevent CNV growth, rather than having to wait to treat after damage is done. Some studies with diode laser have shown promise. Our practice is part of the PTAMD study. There is also a lot of research on antiangiogenic drugs that may work. Our practice also does macular translocations, but this is also not a cure. It is a surgical procedure that moves the macula away from the CNV, but it has many inherent risks and is not done as much since the introduction of PDT.

Although I agree with your analogy that these treatments are somewhat like bandaids, I still feel strongly that they are better than no treatment when they meet proper indications. I know the frustration that I sometimes feel in treating macular degeneration is not at the same level of the patient, but it is definitely present. It is hard to accept that sometimes the best we can do is slow down a process rather than curing it. It is also, however, extremely satisfying when I have a patient improve after PDT. I have several patients that were legally blind that have improved to 20/50 or better after PDT, and I have monocular patients that also have had great responses to treatment. It is also, without a doubt, very satisfying to treat a nonfoveal CNV and prevent its spread to the fovea. This is more satisfying for me than my patients because they often have not lost vision yet and therefore are unable to comprehend what would have happened to the central vision without treatment. This also helps to emphasize the importance of the amsler grid, particularly for patients that have lost vision in one eye from AMD.

DOTTIE: My friend is going to have thermal photocoagulation treatment. Would photodynamic therapy be a better option?

DR. SONKIN: Thermal (hot) laser is still the treatment of choice for classic choroidal neovascularization (abnormal blood vessels under the retina that are well-defined; CNVM; wet type) that does not involve the fovea, or center of the macula. If the CNVM your friend has is mostly "classic" and involves the fovea (very central point of our vision), then most retina surgeons would argue that PDT is better than thermal laser. If the blood vessels do not involve the fovea on fluorescein angiogram, than the thermal laser he received would be the best option. Hopefully the physician that treated your friend is trained in making the proper distinction, which can often be very difficult.

ROB: My father (age 84) only has one eye that's got "useable" vision. The other has long term ARMD and other related problems that render it nearly useless. The remaining eye had it's first leakage a year ago that seems to have spared the fovea. The absorption/clearance of the leakage substances has been very slow and is, perhaps, still ongoing.

He is of the impression that PDT, in the event of another leakage event, could be a substantial risk to the remaining vision. Something about the condition of the rods and cones. I don't know where he got this idea but I didn't want a fatalist attitude as that might lead to ignoring danger signs and assuring loss of remaining vision.

I've read that the earlier one can catch and treat the leakage, the better chance there is of saving central vision.

DR. SONKIN: To fully answer your question I would need more information. In general, though, you are correct that early detection is very important. The location of subretinal fluid is not nearly as important as the location of the abnormal blood vessels (CNV) under the retina that are leaking and resulting in the "wet" AMD. This is determined by exam and fluorescein angiogram (FA). If the CNV is caught before it extends under the fovea and it is "classic" and well- defined, thermal laser can be used to "save" the fovea and the very central vision. If the CNV is "classic" and involving the fovea, PDT is often the best treatment choice. There is some risks with the treatment, but the risks of no treatment is greater. This was proven in large national clinical trials that resulted in FDA approval of the treatment. If the CNV is not mostly "classic", but instead is "occult" in nature (as determined by exam and FA), treatment is completely different. There are several treatments that retina surgeons offer, including TTT and in some cases PDT. Neither is FDA approved for that indication at this time.

With regards to the status of the rods and cones, your dad may have another retinal condition on top of the AMD. With regards to the slow and chronic leakage, this may indicate an "occult" CNV or other variations of AMD, such as serous or fibrovascular pigment epithelial detachments (PEDs). Treatment varies depending on many factors.

The bottom line is to emphasize to your dad the importance of regular exams and home amsler grid evaluation. With any changes, he should see his retinal surgeon ASAP. If he develops a change that necessitates treatment, it is important to let him know that the goal can be to improve the vision. In many cases, however, it is to prevent further loss of vision or to slow down vision loss, both of which are still very valuable.

SHARON: I am now 55 yrs of age and had a sudden onset of eye problems. First diagnosis was central serous retinopathy, then CNV, then the conclusive diagnosis was determined by use of ICG.

I have a variant of idiopathic polypoidal choroidal vasculopathy. For two years, I requested the opportunity to have phimotion angiography and was told it was not suitable. I lost the vision in one eye due to scar tissue from a bleed. I again asked for phimotion angiography when a hemorrhage recently occurred in the other eye. I asked a different RS this time. He was enthusiastic and arranged for the consult and treatment to cut off the feeder vessels.

1. Why are Doctors so reluctant to refer a patient to treatments they are unable to personally provide?

2. If we cut off the feeder vessels of which there are two, does this mean the progress of the disease is halted?

3. Even if the CNV is halted will the macular degeneration progress?

4. Is there an explanation as to why transpupillary thermotherapy (TTT) could be painful in one spot on the eye?

DR. SONKIN: It does sound like you have a complicated retinal history. I will try to answer some of your questions. Central serous retinopathy (CSR) can lead to the development of choroidal neovascularization (CNV). Idiopathic polypoidal choroidal vasculopathy is a complicated disease process, but in simplest terms it is a multifocal growth of CNV abnormal blood vessels under the retina. We do not know the best treatment, but most retina surgeons I know will watch this, unless it is causing problems or progressing. If the CNV is not foveal, thermal laser is often a good choice. If the CNV involves the fovea, some prefer PDT.

Feeder vessel treatment (aka phimotion angiography) has been shown in some studies to be beneficial if a feeder vessel is identifiable, which it often is not. There are risks with feeder vessel treatment, just as there are with any type of laser. If a feeder vessel is successfully treated, there are times when this will result in regression of the entire CNV with stabilization or improvement of vision. It does not cure the underlying problem (AMD or polypoidal or CSR), and persistence or recurrence of the CNV is definitely possible. In answer to your question, halting a CNV membrane does not stop or cure AMD, but it may prevent or delay visual loss.

TTT laser is a large spot diode laser that more frequently causes discomfort because it's wavelength is absorbed deeper to the retina.

With regards to doctors referring patients to other doctors that offer different treatments, that discussion could take weeks. I always refer if I feel someone else is better suited to help a given patient, and I take no offense to my patients obtaining second opinions and I sometimes recommend it. At the same time, I feel very protective of my patients and if I feel a certain type of treatment holds more risk than benefit, that is what I tell them.

I hope your better eye continues to do well. I saw a patient of mine this morning that has polypoidal choroidal vasculopathy. Her left eye has had two thermal lasers and remains 20/20. These lasers were done approximately one year ago, because the extrafoveal CNV were leaking fluid into the fovea and reducing the vision to 20/200. In her right eye, the initial thermal laser was also successful. Approximately four months ago, she developed a CNV in the right eye that involved the fovea, and we are in the process of trying to control it with PDT. Hopefully this will also do well.

DOE DOE: According to a recent report, PDT treatment for "occult only subfoveal choroidal neovascularization" now qualifies for reimbursement under Medicare. Does the qualifying condition include all forms of MD or is "occult only subfoveal choroidal neovascularization" a special type of MD, leaving the others excluded?

DR. SONKIN: When characterizing "wet" AMD on fluorescein angiography, the abnormal blood vessels fall under one of two catagories, either "classic" or "occult". Making this determination requires extensive experience with retinal diseases and treatment, and usually is only done by retina surgeons. In a given eye, the CNVM (wet form) can be completely classic, completely occult, or a combination of the two. Visudyne has been approved for completely classic, predominantly classic (greater than 50%), and now also completely occult. This still leaves out a small group of patients that have a combination of the two that is predominantly occult. Visudyne may also help these patients, but studies have not revealed this yet.

GEOFF: Many thanks for taking the time to answer our questions. I wonder if you have any experience with Central Areolar Choroidal Dystrophy (CACD)? Also, I see there have been studies that indicate antioxidants and zinc may be beneficial. I wonder if this also applies to hereditary types of MD, or is their progress already pre-programmed so that supplements may not have the same effect?

DR. SONKIN: Central areolar choroidal dystrophy (CACD) is an uncommon hereditary degenerative retinal disease. Symptoms of decreasing vision and loss of central vision usually arise in the teenage years through the 20's and 30's, and the rate of subsequent visual loss is very variable. Clinically, the macula develops a "mottled" appearance early on, which is then followed by a very distinct sharply bordered macular atrophy. We believe the underlying problem is the circulation beneath the retina and RPE known as the choriocapillaris. It is unknown whether vitamins will help slow progression of CACD, but given the proposed mechanism, it is less likely to help than in an age related degenerative process such as AMD. It is also important to keep in mind that CACD looks extremely similar to other conditions (too many to list), some of which carry a better prognosis and some worse. Also, there is no definitive test for CACD. Diagnosis is based on clinic appearance, clinic course, and FA. Best of luck, and be sure to follow-up with a retina specialist at least once a year. As your condition evolves, the diagnosis will become more certain. Also, I strongly recommend seeing a good low vision specialist. Although your central vision may become worse, fortunately CACD will not completely blind you and you will retain normal peripheral vision and some central vision. You certainly will want to optimize your ability to use the vision that you do have.

ROSIE: I'm a 62-year old female and have had high myopia since I was 9 years old. I had cataract surgery on both eyes in 2000 and macular hole surgery on my left eye in April 2001. In Aug/Sep 2001, vision in my right eye turned worse. My corrected visual acuity is a fuzzy 20/100 in my left eye and a fuzzy 20/50 in my right eye. [Lists specific results of examinations, not included here.] I have the following questions:

1. Is retina pigment epithelial changes with no drusen dry age-related macular degeneration?
2. Is it the same as RPE - retina pigment epithelial detachment?
3. Can it go from dry to wet without first drusen developing?
4. The new study on zinc supplements mentions that supplements are most successful when taken by persons with somewhat advanced dry macular degeneration. Am I at this stage?
5. Why can my myopia not be correct with glasses?

DR. SONKIN: Given the normal appearance of the right macula, and your history of macular hole surgery on the left, odds are that the RPE changes seen in your left macula are the result of the old macular hole. This is very common and likely is the cause of the reduced vision at 20/100. Although we can close macular holes greater than 90% of the time, this does not always result in vision near 20/20. I tell my patients that the main goal of closing the macular hole is to improve the vision, but the degree of improvement can vary greatly. It depends on the size of the hole, the duration of the hole, the health of the underlying RPE and the surrounding retinal tissue, the overall health of the patient, and last but not least, nature. Not every eye heals the same way. It is possible that the changes in your left macula is AMD, but this seems less likely to me. It is difficult, however, to tell you with any certainty given that I am unable to actually examine your eyes.

Regarding your specific questions:

1. RPE changes without drusen can be a presentation of AMD, but not always. You have to consider the person's age, the appearance of the other eye, the medical history, and the past ocular history (e.g. prior surgery). It is a clinical decision.

2. RPE detachments are a specific subtype of AMD. They are often classified as either serous or fibrovascular, the latter being a form of "wet" occult AMD.

3. Drusen are not always clinically visible, and you can develop wet AMD without first having visible drusen, although this is not common.

4. Vitamins may or may not help your condition. I would recommend a good balanced diet and multivitamin. This is not, however, based on any science.

5. General myopia is correctable with glasses, unless the myopia has resulted in functional damage to the retina or underlying tissues. The analogy I often use when asked why glasses won't always fix visual loss is that changing glasses is like cleaning the windshield of a car that isn't running very well. If the problem is with the engine of the car, it doesn't matter if the windshield is crystal clear and clean, the car won't run any better. The same holds true for the eye. If the problem is with the retina (the "engine" of the eye), new glasses (or cleaning the "windshield") unfortunately won't help.

MAGGIE: About three years ago, I began to find it uncomfortable to open my eyes in the morning. I had the sensation that opening them would cause discomfort - rather like trying to open an eye glued down with matter- although there was no matter in my eyes. Usually, just one eye was affected. Sometimes it was the left and sometimes the right. Within a short time, I experienced several incidents of difficulty in opening one eye, followed by intense discomfort. My eye hurt as if someone had poked it with a sharp stick. This pain would last for about an hour, then disappear.

In the past couple of years, the condition has grown worse. I cannot open my eyes without massaging them, gently, first. If I do this and open my eyes very slowly, I have no problem. Once in a while, I forget and open my eyes rapidly. Usually one eye seems to get stuck to the lid and I am in agony for an hour afterward. The eye actually puffs and waters for this period. Afterwards, it returns to normal, but my vision in it is blurry for a couple of days or more and my eye is very sore.

I have angioid streaks, but they have never caused any problems, and my vision has not deteriorated. I am curious about what causes the pain I experience when I open my eyes suddenly.

DR. SONKIN: With regards to the angioid streaks, it is important to continue follow-up with your retina doctor. They predispose you to CNV (abnormal blood vessels under the retina), much like those found in wet AMD. Also make sure you have been evaluated for sytemic diseases that can cause angioid streaks. The pneumonic we use for causes of these streaks is: PEPSI = pseudoxanthoma elasticum, ehlers danlos, paget's disease, sickle cell anemia, and idiopathic ("bad luck, no cause"). Idiopathic is the most common.

With regards to your other symptoms, I would recommend seeing a corneal/external disease specialist. It is a bit out of my area of expertise. The most likely things I would think of is a corneal dystrophy (which can sometimes be hard to diagnose and there are several different ones), recurrent corneal erosions, abnormal palpebral conjunctiva (under the lids), blepharitis, etc.

SHARON: In your response to Judy you wrote, "There is also a lot of research on antiangiogenic drugs that may work." Would you please be so kind as to list these drugs? Are there known severe contraindications? We would certainly appreciate any insight into drug therapy.

DR. SONKIN: The use of antiangiogenic drugs for AMD is still in the future. There have been a few studies done, including thalidomide and other drugs. That study was stopped due to systemic side effects. Most of the research is still in the basic science stages, although some clinic studies do exist. We can all remain hopeful. Certainly, preventing the problem before it develops is the ultimate solution.

ALAN: About 2 months ago, I suddenly developed what has been diagnosed as posterior vitreous detachment. Is this related to, or caused by, my dry MD? Could it be a precursor to development of wet MD in that eye? Can I expect continuing detachment of the vitreous in that eye?

DR. SONKIN: Our eyes are filled with a jelly-like substance called vitreous behind our natural lens. The vitreous fills the posterior compartment of the eye. As we get older, the vitreous undergoes many changes. One change is that it separates from the surface of the retina. This normally occurs without any major problems except for visible floaters. It can also cause retinal hemorrhages, vitreous hemorrhages, retinal tears, and retinal detachments. I am sure your doctor checked for these things.

Posterior vitreous detachment (or separation), also called a PVD, is not related to macular degeneration. The symptoms will likely persist, but may change and fluctuate. If you develop any sudden changes including a sudden increase in the floaters, flashes, or curtains/veils over your vision, return to your retina doctor for a recheck.

GIDEON: I was treated only once with PDT on a "classic" leak in January 2000. Since then, despite at least 4-5 additional leaks, but never under the fovea, my RS has decided to let it dry alone, as it really happened. My vision stayed stable since then.

My question is what according to your opinion is the dynamic "life circle" of the sickness? In the beginning, they said one year, then it became one and a half, now we talk about two and more.

The second part of my question is what is supposed to happen after one cycle, should/must we expect others? Or is PDT too new to answer this? How was it before PDT? My first eye, with hot laser (including your finger-hand phenomena) stays still static the 11th year now.

DR. SONKIN: Whether wet AMD goes untreated, or if it is treated with PDT or thermal laser, no two eyes behave the same. I tell all my patients that persistence and recurrence is, unfortunately, always a possibility with any treatment, but that hopefully those are bridges we won't have to cross. It is almost impossible to predict the risk of recurrence in a given eye. I have patients that have been treated only once with both thermal laser and/or PDT, and no recurrences have appeared. There are also patients who have been treated multiple times with no resolution. I have seen patients who were treated with thermal laser 15 years ago with absolutely no recurrences.