Category Archives: Retinal Disorders

What is the Difference Between Dry and Wet AMD?

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Age-Related Macular Degeneration: Dry or Wet?

What is the difference between dry and wet age-related macular degeneration?

Age-related macular degeneration (AMD) is the most common cause of visual loss in older Americans.  Doctors often talk about dry and wet types of AMD.  But what is the difference?  Simply put, dry AMD means there are no abnormal, leaky blood vessels.  Whereas, in wet AMD there are abnormal blood vessels that grow and leak under the macula.  However, this system of naming wet and dry AMD can be confusing.  Therefore, newer names for the stages of AMD have been proposed.

Why is it important to distinguish between wet and dry AMD?

Dry and wet AMD cause different symptoms and are treated differently.  Early and intermediate stages of AMD are often called dry AMD.  These stages usually cause few symptoms, which include the need for good lighting.  The treatment involves AREDS2 vitamins and a Mediterranean diet.  Advanced stages of AMD are called geographic atrophy (advanced dry AMD) and neovascular AMD (wet AMD).  Both types of advanced AMD are usually associated with decline in central vision, frequently affecting the ability to read, see the computer, and drive a car.  Geographic atrophy (advanced dry AMD) progresses slowly with blind spots that interfere with the ability to see.  Neovascular AMD (wet AMD) may advance more rapidly with distortion, blurred areas of vision, and blind spots.  It is possible to have both forms of advanced AMD…geographic atrophy (advanced dry AMD) and neovascular AMD (wet AMD).

What is the treatment of advanced AMD?

The treatment of the two forms of advanced AMD is different.  Furthermore, the treatment of one type of advanced AMD may aggravate the other form of AMD.  However, both forms of advanced AMD benefit from the use of magnifiers and other low vision aids.

What is the treatment of geographic atrophy (advanced dry AMD)?

The Meditteranean diet is protective against advanced AMD and appears to slow the enlargement of geographic atrophy. Additionally, Geographic atrophy (GA) is currently treated with medications that inhibit the complement systems of proteins in the body that normally cause inflammation to fight infection.  The two FDA-approved medications are Syfovre and Izervay.  They are both given as an injection into the eye.  They do not reverse the damage from GA and do not prevent GA from getting worse.  They do slow down the rate of worsening of GA.  There is no good evidence to show that one medication is better than the other.  Long-term injections are needed over the years.  And, unfortunately, both medications may cause wet AMD (neovascular AMD) to start.

What is the treatment of wet AMD (neovascular AMD)?

Wet AMD is treated with injections to stop the leaking blood vessels under the macula.  These medications (Avastin, Lucentis, Eylea, Beovu, and Vabysmo) prevent further worsening of vision from wet AMD in about 90% of cases over several years.  In about 40% of cases, the vision may improve.  Long-term injections are usually needed over the years.  There is limited evidence to suggest benefits of some medications over others.  There is also some concern that the treatment of wet AMD may aggravate geographic atrophy (advanced dry AMD).  

What do you do when an eye has both forms of advanced AMD?       

The presence of both neovascular AMD (wet AMD) and geographic atrophy (advanced dry AMD) poses a serious problem.  Both forms of advanced AMD may result in legal blindness (the loss of central vision).  However, advanced AMD almost never results in complete blindness.  Treatment is usually aimed at the greatest threat to vision.  Specific treatment plans are made on a case by case basis.  In some situations, injections are given on an alternating schedule for GA and neovascular AMD.  In end-stage situations in which the chance of further loss of vision is low, all injections may be stopped.  Low vision aids and assistance (family and social services) often results in high-level functioning and independence. 

By Scott E. Pautler, MD  

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2023 Designs Unlimited of Florida.  All Rights Reserved.

Will I Lose Vision from AMD?

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Will I lose vision from AMD?
anatomy of the eye (click on image to enlarge)

What are the risk factors of losing vision?

If you have been diagnosed with AMD, you may ask, “Will I lose vision from AMD?” There are many risk factors that lead to the worsening of age-related macular degeneration (AMD).  Some are genetic (inherited) and some are environmental.  However, one the greatest risk factors is age.  This is, the older you are with AMD, the greater the risk of loss of vision.  Genetic testing can reveal high risk genes, such as CFH and ARMS2.  Therefore, a family history of vision loss from AMD increases your risk.  Other important factors that affect the risk of losing vision include tobacco use and diet.

What are the stages of AMD?

There are several stages of AMD that affect the risk of severe loss of vision: early, intermediate, and late AMD.  Early AMD presents with medium sized deposits under the retina called drusen.  These eyes usually have no symptoms and the vision is good.  Intermediate AMD have large drusen and/or brown pigmentary changes under the retina.  These changes frequently result in slow recovery from bright light (like coming in from outdoors) and the need for good lighting while reading.  Late AMD causes distortion of straight lines and blind spots in the vision from geographic atrophy (loss of retinal tissue) and/or neovascularization (new blood vessels grow under the retina that bleed and cause blind spots from scar tissue).  Geographic atrophy is also called advanced dry AMD, whereas neovascularization is called wet AMD.  

How can I determine my risk of late AMD with vision loss?

A point system has been developed to determine your risk of loss of central vision from late AMD.  Exam findings in each eye are given a point value.  Then, all the points are added together from the two eyes to arrive at risk of progression to late AMD within 5 years.  

Exam FindingPoint Value
medium drusen0.5
large drusen1
pigmentary changes1
geographic atrophy or neovascularization2
Add the points from findings in each eye
From: Ferris FL 3rd, Wilkinson CP, Bird A, et al. Clinical classification of age-related macular degeneration. Ophthalmology. 2013;120(4):844-851. doi:10.1016/j.ophtha.2012.10.036

At total score of 1 yields a low risk of late AMD of under 5% in five years.  With 2 points, the risk is 12 percent.  Three points means a risk of 25% at five years.  And 4 points means there is a 50% chance of late AMD in five years.  Another way to calculate your risk of late AMD is to use an online calculator.  

How bad can the vision fall from late AMD?

Late macular degeneration may cause a large blind spot in the center of the vision in both eyes.  Although this renders an individual legally blind, it is important to remember that it is extremely rare to lose all vision from AMD.  The peripheral vision usually remains strong and this allows a person to get around a room and care for himself independently.  

Does everyone lose most of their central vision in late AMD?

No.  Many people retain useful vision.  Patients with advanced dry AMD may be treated with Syfovre or Izervay to slow the progression of geographic atrophy.  There are a host of medications that slow the loss of vision from wet AMD, including Avastin, Lucentis, Eylea, Beovu, and Vabysmo.  There is high expectation that further developments from ongoing research will offer even better odds of retaining useful vision in the future.  Those patients who do lose vision from AMD can be helped with low vision aids.  

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2023 Designs Unlimited of Florida.  All Rights Reserved.

Dome-Shaped Maculopathy

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dome-shaped maculopathy
anatomy of the eye (click on image to enlarge)

What is dome-shaped maculopathy (DSM)?

Dome-shaped maculopathy (DSM) is a type of myopic macular degeneration.  Myopic macular degeneration (MMD) is a degeneration of the center of the retina seen in some people who are myopic (near-sighted).  The cause of DSM and MMD is related to an elongation of the eye commonly seen in myopia.  MMD is not the same as age-related macular degeneration, which is a common cause of vision loss in the elderly. DSM is often mistaken for central serous retinopathy. 

What is the macula?

The macula is the area of the retina in the back of the eye that is responsible for seeing details in the central vision.  The retina is a thin layer of delicate nerve tissue that lines the inside wall of the eye like the film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.  Although macular problems affect the central vision, they do not affect peripheral vision— the ability to see objects off to the side when looking straight ahead.  This means that macular problems alone do not result in total blindness.

Why is it called dome-shaped maculopathy (DSM)?

As the name suggests, in dome-shaped maculopathy, the macula assumes the shape of a dome or elevation as seen on special imaging, called optical coherence tomography (OCT).  A dome-shaped macula is the result of the eye attempting to keep the eye focused at near.  The elongation of the eye may result in an irregular eye shape, including a dome or ridge shape.

In the sketch above, A represents the normal eye. B shows how the eye is elongated in near-sighteness (myopia). C and D show a focal out-pouching at the back of the eye. This out pouching is called a staphyloma. At the edge of the staphyloma (green arrowhead), the tissues inside the eye are stretched. This area appears as a dome on retinal imaging.

What causes fluid leakage under the retina in DSM?

 A dome-shaped macula may cause an abnormal leakage of clear fluid under the macula, which causes symptoms of blurred vision often associated with distortion or a round/oval dark spot in the central vision.  Other factors may aggravate the situation and contribute to a leakage of fluid beneath the macula.  Research suggests that hormones released under stress can affect the blood vessels beneath the retina. These vessels may leak an abnormal amount of fluid, which then works its way under the center of the retina. Sometimes, medical conditions (Cushing’s Syndrome, pheochromocytoma, sleep apnea), medications (pseudephedrine, prednisone, cold medication, diet pills, and medications for erectile dysfunction), and other agents (testosterone, cocaine, caffeine, amphetamines, etc.) may contribute to the development of leakage.

What is the treatment of DSM?

There is no universally effective treatment.  In some cases, the symptoms will disappear with time. Over a period of weeks to months, the abnormal leakage may stop with normal healing. In other situations, treatment may be needed to recover vision. Prescription medication (spironolactone or diamox) may be helpful if fluid leakage does not go away with time alone. Sometimes, eye drops can help. Other treatment that have been tried without uniform success include thermal laser photocoagulation, Visudyne photodynamic therapy (PDT), and medicine injections. The doctor can recommend the best course of action by taking special pictures of the eye called a fluorescein angiogram. The fluorescein angiogram shows where the leak is coming from, how active the leak is, and whether laser might be helpful. Optical coherence tomography (with and without angiography) may also be helpful to guide treatment.

In rare situations, blood vessels grow beneath the retina from the choroid (choroidal neovascularization, CNV).  When CNV is detected by fluorescein angiography or by optical coherence tomography, antiVEGF injections are the first time of treatment. 

What will happen to my vision?

Over a period of months to a few years, the vision may remain normal or near normal under observation without treatment. Sometimes over time, dome-shaped maculopathy may cause permanent loss of central vision.  More aggressive treatment may be considered in cases of persistent fluid leakage causing progressive loss of vision.  

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2023 Designs Unlimited of Florida.  All Rights Reserved.

Central Retinal Vein Occlusion

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globe anatomy
anatomy of the eye (click on image to enlarge)

What is a central retinal vein occlusion (CRVO)?

Retinal vein occlusion means blockage of a vein in the retina.  A central retinal vein occlusion is a blockage of the main (central) vein on the retina. The retina in your eye is like the film inside a camera.  The retina “takes the picture” of objects you look at and sends the message to the brain.  The retina is a living tissue, which requires blood supplied by tiny vessels.  Retinal veins are blood vessels that drain blood from the retina. The veins form as branches that drain sections of the retina.  The branch veins all drain into the central retinal vein that exists the eye with the optic nerve.  An abnormal blood clot in a retinal vein causes a blockage (occlusion) of the blood flowing out of the retina.

Who is at risk of a central retinal vein occlusion?

Retinal vein occlusions are more common in people who have risk factors for hardening of the arteries, which may compress the veins where they come in contact in the retina.  Hardening of the arteries (arteriolar sclerosis) occurs in people who are overweight, use tobacco, or have diabetes, high blood pressure, or sleep apnea.  Glaucoma is also a risk factor as intraocular pressure spikes affect blood flow in the retinal veins, and slow blood flow promotes blood clots.  There are a number of blood disorders that may play a role in CRVO, especially in younger individuals.  Estrogens may increase coagulation of the blood and lead to CRVO. Less commonly, inflammation may cause retinal vein occlusion.  

What are the symptoms of a central retinal vein occlusion?

Blurring of vision may occur if excess fluid (edema) leaks from the veins into the center of the retina.  Floaters can look like tiny dots or cobwebs moving about in your vision.  They may be due to bleeding from the retina into the central gel (vitreous) of the eye.  Pain is rare and may be due to high pressure in the eye (neovascular glaucoma).

What treatment is available?

There is no cure, but treatment may improve vision or keep the vision from worsening.  Your doctor may allow time for the vein to heal, but this is rarely advisable if the vision is impaired.  Medicine injections may help recover vision and may be given with little discomfort.  The vision may not return to normal following treatment as there may be some permanent damage to the retina from the occlusion.  Injections must be repeated in order to maintain vision.  Over time, the retina does heal to some degree.  However, after three years of treatment about half of eyes continue to require injections.  In some cases when treatment cannot improve the vision, laser is used to prevent severe pain and complete blindness. Your doctor is going to order appropriate tests and recommend the best course of action to take at this time.  The retinal vein occlusion will not be worsened by your daily activities or by using your eyes. 

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2023 Designs Unlimited of Florida.  All Rights Reserved.

Choroidal Macrovessel

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Choroidal macrovessel is the term currently used for a rare choroidal vascular abnormality that may present as a small choroidal mass with or without symptoms.  It usually appears as a dilated, tortuous choroidal vessel beneath the temporal macula and may extend to the periphery. A dilation of the posterior ampulla may elevate the overlying retina (see OCT image below) and cause secondary changes in the retinal pigment epithelium, rarely with subretinal fluid.  Choroidal macrovessel is usually diagnosed during adulthood with a female predilection.  There is no known associated systemic vascular abnormality.

Another term that may be somewhat more specific for this condition is posterior aneurysmal choroidal varix. An aneurysmal varix is a markedly dilated and tortuous vessel, sometimes used to describe a dilated vascular channel due to a direct communication of an artery and a vein. In the example below, there appeared to be a direct communication between a short posterior choroidal artery and a choroidal vein (Haller vein) as seen on ICG angiography where there was early filling of the lesion in the arterial phase. On the color photo, the prominence of the lesion diminishes in appearance as the vessels track toward the vortex outflow. This is likely due to numerous collateral venous channels that are known to exist, which allows for a reduction in blood flow and intraluminal pressure. Thus, the peripheral choroidal venous channels appear unremarkable.

The most helpful diagnostic tests include optical coherence tomography (OCT) and indocyanine green angiography (ICGA).  OCT shows a small hyporeflective choroidal mass temporal to the fovea.  There may be disruption of the outer retinal bands and/or subretinal fluid.  ICGA shows early filling of the prominently dilated and tortuous choroidal vessel.  

As choroidal macrovessels are usually asymptomatic, no treatment is needed.  Their importance is primarily to differentiate them from tumors.  Rarely, they cause disruption of retinal pigment epithelium resulting in simulated “tracks”, which may be confused for ophthalmomyiasis (subretinal larva). 

By Scott E Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2023 Designs Unlimited of Florida.  All Rights Reserved.

HTLV1-Associated Uveitis

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HTLV1-associated uveitis
Anatomy of the eye (click on image to enlarge)

What is HTLV1?

HTLV1 is a virus that causes HTLV1-associated uveitis. It is an abbreviation for human T-lymphotropic virus type 1.  First isolated in 1980, HTLV1 belongs to the retrovirus group that also includes the virus that causes HIV/AIDS.  Retroviruses are called “retro” because they use a pathway to reproduce that is the reverse of what most organisms use.  The genetic map of retroviruses is RNA, which is converted inside host cells to DNA by a special enzyme (reverse transcriptase).  The host cell is then directed to produce more virus particles.  HTLV1 is called “lymphotropic” because it tends to infect lymphocytes, which are a type of white blood cell involved with immunity (see Legrand).  

How and where do you get exposed to HTLV1?

Because most people with HTLV1 infection remain without symptoms, they carry the virus and spread it to others by sexual contact (semen), shared blood (e.g. IV drug-shared needles, organ transplantation), and by breast milk.  HTLV1 is found in most frequently in people from Brazil, Japan, sub-Saharan Africa, Honduras, Iran and the Caribbean islands.  However, due to international travel, HTLV1 may be found anywhere in the world.

What problems does HTLV1 cause?

Many people who are exposed to HTLV1 develop no symptoms. However, because HTLV1 affects white blood cells, it may cause autoimmune conditions, as well as blood cancer. For example, autoimmune conditions include seborrheic dermatitis (infective rash), paralysis (tropical spastic paresis), and uveitis (see Schierhout). Examples of blood cancer include T-cell lymphoma and leukemia.

What is Uveitis?

Uveitis (pronounced, “you-vee-EYE-tis”) is a general term used to describe inflammation inside the eye.  The uvea is the name given to the layer of tissue in the eye that has a brown color (melanin pigment) and blood vessels, which serve to provide blood supply and protect the eye from excessive light.  The uvea can be divided into separate parts, which perform different functions in the eye: the iris, the ciliary body, the pars plana, and the choroid (see anatomy of the eye).  Therefore, in any one patient uveitis is usually given a more specific name depending on where most of the inflammation is located in the eye. Sometimes, uveitis affects tissues not considered a part of the uvea.

What type of uveitis is most common with HTLV1?

Intermediate uveitis is the most common type of uveitis caused by HTLV1. In intermediate uveitis the inflammation mainly centers in the vitreous gel (the clear gel that fills the eye). This type of uvetiis is called intermediate because it affects the middle or intermediate part of the eye. That is, the vitreous gel fills the eye and is located in an intermediate position between the front and the back of the eye. Vitritis and pars planitis are other names for intermediate uveitis.    

Who is most likely to develop HTLV1-associated uveitis (HAU)?

The age group most likely to be affected by HAU is between 20-49 years; however, any age group may develop HAU (see Mochizuki).  Female are affected by HAU twice as often as males (see Takahashi).  It appears that the eye inflammation (uveitis) is caused by the effect of HTLV1 infection on the behavior of white blood cells (lymphocytes), rendering them more likely to mistakenly attack the eye (see Mochizuki).  HAU may occur with or without other ocular inflammatory conditions, such as thyroid eye disease (see Nakao). Likewise, HAU may occur with or without non-ocular HTLV1-associated conditions, such as paralysis, rash, or blood cancer. 

What are the symptoms of HTLV1-associated uveitis (HAU)?

The most common symptoms include tiny floating spots which move or “float” in the vision.  They are usually numerous and may cause a veil-like appearance in the vision.  Sometimes blind spots, blurred vision, distortion, or loss of side vision occurs. The eye may be painful, red, tearing, and light sensitive if other parts of the eye are also inflamed (5-10% of cases).  Symptoms may be mild or they may be severe and disabling.  Only one eye is affected in about half of all cases of HAU (see Takahshi).

How is HTLV1-associated uveitis (HAU) diagnosed?

Diagnosis can be difficult.  Blood tests are performed to identify HTLV1 infection in patients with findings that suggest HAU.  One FDA-approved test is produced by MP Biomedicals Diagnostics: HTLV blot 2.4 (EIA).  Sometimes, accurate diagnosis requires multiple tests.  

How is HTLV1-associated uveitis (HAU) managed?

There is no cure for HTLV1 infection.  To limit the damage from inflammation, HAU is treated with anti-inflammatory medication in the form of eye drops, injections, or pills.  When pills are used, the eye doctor may coordinate medical care with the expert assistance of a rheumatologist.  Rarely, surgery is required to treat uveitis.  Episodes of inflammation may last from weeks to many years.  HAU is a serious eye problem and may result in loss of vision (see Takahashi).  However, by seeing your eye doctor and taking the medications exactly as recommended, damage to your vision can be minimized.  Most people with HAU keep good vision (See Nakao).  In some cases, HAU may go away, but return at a future date in about 50% of cases (see Takahashi).  Therefore, if you become aware of symptoms of uveitis in the future, do not hesitate to contact your doctor. 

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2023 Designs Unlimited of Florida.  All Rights Reserved.

Lattice Degeneration

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globe anatomy
anatomy of the eye (click on image to enlarge)

What is lattice degeneration?

Lattice degeneration is a condition in which the retina develops areas of abnormal thinning.  The thinning occurs as the retina stretches during growth of the eye.  The retina is a “tissue-paper” thin layer of nerve tissue, which lines the inside of the eye like the film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.  Lattice degeneration is most common in near-sighted people and affects about 7% of the general population.          

What causes lattice degeneration?

Lattice degeneration appears to be an inherited condition that usually develops in childhood or early adulthood.  The eye, which is round like a ball at birth, becomes oval like an egg.  The elongation of the eye causes the retina to stretch.  As a result, thin areas develop in the retina similar to “stretch marks” that occur in the skin with growth or weight gain.    

Why is it important to know about lattice degeneration?

            Lattice degeneration in itself causes no symptoms or loss of vision.  It is important because lattice degeneration predisposes eyes to retinal detachment, which can cause permanent blindness without treatment.  With age, trauma, or inflammation, the vitreous gel that fills the eye begins to condense and pull away from the retina.  If the retina has become weakened by lattice degeneration, it is more likely to tear when pulled upon.  A tear in the retina allows fluid from the vitreous gel to seep under the retina as the retina detaches.  Fortunately, only one in 200 eyes with lattice degeneration ever develop retinal detachment.  Usually no treatment of lattice degeneration is needed, but symptoms of new floating spots or fibers should be reported to the doctor promptly. An examination may disclose breaks in the retina, which may be treated in the office with laser.  The appearance of a dark curtain or shadow from the side-vision like an eclipse of the moon is an even more serious symptom that may indicate retinal detachment and require major eye surgery to repair. 

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  ©2022 Designs Unlimited of Florida.  All Rights Reserved.          

Alport Syndrome and the Eyes

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globe anatomy
anatomy of the eye (click on image to enlarge)

Alport syndrome is an inherited condition that prominently affects the kidneys.  It may also cause hearing loss and affect the eyes.  It is due to an alteration in genes that codes for a protein called collagen.  Alport syndrome affects about 1 in 5,000 to 10,000 people in the US.  Depending on which gene is affected, there are three types of Alport syndrome:

1.) X-Linked Alport syndrome is the most common type (80% of cases) and it affects males more severely than females.  

2.) Autosomal recessive Alport syndrome must be inherited by an affected gene in both parents.  It is rare and affects males and females equally.  

3.) Autosomal dominant Alport syndrome is inherited from one parent with an abnormal gene.  It affects males and females equally and may be less severe.

How does Alport syndrome damage the kidney?

            Alport syndrome causes abnormal collagen to form.  There are many different types of collagen, which is deposited outside the cells to give structural and functional support to cells in the body.  The collagen (type IV) affected by Alport syndrome is important for kidney function.  It affects the glomerulus, which is the blood filtering unit of the kidney.  The decline in kidney function may eventually require kidney transplantation.  

What kidney symptoms does it cause? 

            Alport syndrome causes blood in the urine, the most common early sign of disease.  Urinalysis reveals protein in the urine, as well.  Related to damage in the kidney, Alport syndrome may also cause swelling (edema) of the extremities (especially, the legs).  High blood pressure is also common.  Other symptoms include weakness, fatigue, loss of appetite, poor digestion, excessive thirst, and frequent urination.

How are the eyes affected?

            Alport syndrome affects the cornea, lens, and retina of the eye.  The changes in the eye are more common with X-linked and autosomal recessive disease than in autosomal dominant cases.  

The cornea is the clear front window of the eye.  Alport syndrome may cause recurrent corneal erosions (similar to a scratched cornea, but without trauma). Symptoms include intermittent pain, redness, tearing, blurred vision.  PPCD is a corneal dystrophy that may occur and cause similar symptoms.      

The lens is positioned behind the colored iris and focuses light onto the retina in the back of the eye. Alport syndrome may cause an unusual bulging of the anterior surface of the lens (anterior lenticonus), which may require the use of glasses and may cause cataract formation.

What is the retina?

            The retina is a thin layer of delicate nerve tissue, which lines the inside wall of the eye like the film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.  The retina has two main areas.  The macula is the central area that gives you sharp, central vision and color vision.  The peripheral retina is the part of the retina that gives you side vision and night vision.

How is the retina affected in Alport syndrome?

            Alport syndrome causes abnormalities of the inner the retina, especially evident in the macula.  Inner retinal deposits cause no symptoms, but may be mistaken for dry macular degeneration on a routine eye exam.  These deposits rarely cause symptoms; though a hole in the center of the retina (macular hole) has been reported. 

There appears to be an increased risk of lattice degeneration in Alport syndrome. This may increase the risk of retinal detachment later in life.

How are eye problems in Alport syndrome diagnosed?

            An ophthalmologist may diagnose eye changes on a careful routine examination.  Sometimes, special scans and photographs are needed.  The patient may perform vision checks at home with the help of an Amsler grid.  Distortion of the grid indicates macular problems and warrants a prompt eye exam. 

How are eye problems associated with Alport syndrome treated?

            No treatment is needed if the vision is not threatened.  Corneal problems may be treated with eye drops and contact lenses.  Only rarely is surgery needed.  Lens changes may be compensated for with the use of prescription glasses.  In some cases, cataract surgery is needed.  Only in severe cases of retinal disease is treatment needed by eye injections or surgery.  

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2022 Designs Unlimited of Florida.  All Rights Reserved.

Retinitis Pigmentosa

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globe anatomy
anatomy of the eye (click on image to enlarge)

What is retinitis pigmentosa?

Retinitis pigmentosa (RP) is a group of inherited problems of the retina.  The retina is a thin layer of delicate nerve tissue that lines the inside wall of the eye like the film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.  Retinitis pigmentosa especially affects the peripheral part of the retina where there are many rod-shaped cells that normally provide side vision and night vision.  In advanced cases it may also affect the sharp, central, reading vision and color vision. 

What causes retinitis pigmentosa?

Retinitis pigmentosa is inherited through a variety of different genes.  Because it is hereditary, other members of the family may have retinitis pigmentosa.  However, sometimes the symptoms are mild and special testing may be required.  At other times the abnormal gene that causes retinitis pigmentosa may skip several generations.  In these families no other members can be found to have the problem.

What are the symptoms of retinitis pigmentosa?

Retinitis pigmentosa may cause no symptoms in the early stages. And symptoms may begin early or late in life.  Initially, night vision is impaired.  This makes it difficult to walk in unfamiliar surroundings in dim illumination.  When the peripheral vision (side vision) is affected, an individual with retinitis pigmentosa may accidentally bump into things that are not directly in view.  The severity of these symptoms slowly progresses over years and may impair safe driving vision or result in legal blindness.  The rate of progression tends to be consistent in each family affected.  Therefore, older family members may be able to provide useful information to younger family members.  Late in the course of retinitis pigmentosa, the central vision and color vision may be affected. Make a point to have trusted family and friends help to monitor your ability to drive safely and ask your doctor annually if you have sufficient vision to drive safely and legally.               

How is retinitis pigmentosa diagnosed?

The symptoms listed above may be the first clue to the diagnosis.  A routine dilated eye examination can detect the retinal changes of retinitis pigmentosa.  In some cases, a fluorescein angiogram is helpful.  In this procedure the ophthalmologist injects a dye into the vein of the arm.  Special photographs are taken of the eye, which show the degeneration of the retina. An electroretinogram (ERG) is also used to demonstrate faint electrical abnormalities in retinal function. In this test, electrodes are taped to the head and to contact lenses. A computer magnifies the electrical impulses generated by the retina in response to carefully programmed flashing lights.  Finally, genetic testing may be considered in order to identify a specific gene variant that may be responsible for causing the problem.  This may be important in confirming the diagnosis, genetic counseling, and treatment. Blueprint genetics offers free testing for US residents.  

How is retinitis pigmentosa treated?

There is currently no proven treatment for most forms of retinitis pigmentosa. One type of retinitis pigmentosa is caused by a gene called RPE65.  Patients with this gene variant may undergo gene therapy to treat this type of retinitis pigmentosa.  Future research offers the hope of treatment of additional genes that cause retinitis pigmentosa.  A mouth swab may be sent for free gene testing.    

What about vitamins and nutritional supplements?

For many years research indicated that high doses of vitamin A may be helpful to slow the progression of RP. However, in 2023 a carefully designed analysis revealed there is no benefit, in general. They did report a possible benefit in a rare type of RP seen in families with mutations in the RHO gene. High doses of vitamin A may cause liver damage and should be administered under the care of a physician.

Vitamin E appears to be harmful in high doses for people with RP. It appears to worsen the vision. Therefore, the AREDS vitamins that are recommended for people with age-related macular degeneration should be avoided in people with retinitis pigmentosa. However, routine use of multi-vitamins are safe in RP, as they usually contain less than 30 IU vitamin E per day.

There are other supplements that are often recommended. Lutein is often recommended in RP at a dose of 10-20 mg per day. Fish oil (DHA 200mg per day) may be a helpful antioxidant in RP. Much research needs to be done to prove they are helpful, but there is no compelling evidence to indicate that they are harmful.

Should patients with RP avoid sunlight?

There appears to be consensus among experts in the field, that sunlight tends to worsen the progression of retinitis pigmentosa. Therefore, most recommend avoiding excess exposure to sunlight. Wear a broad-brimmed hat and sunglasses when out-of-doors.

What support services are available?

A wide range of support services, rehabilitation programs, and devices are available to help people with retinitis pigmentosa continue with many of their favorite activities. The use of a white cane can be a valuable aid in navigating in unfamiliar surroundings. It also serves to notify other people to be more courteous. The Lighthouse for the Blind and the Watson Center have classes and specialists in providing prescription lenses to magnify printed material. The Argus II Retinal Implant was approved by the FDA in 2013 for patients with very severe loss of vision from retinitis pigmentosa. The Division of Blind Services provides rehabilitation services and financial aid for eye care in selected cases. Your doctor can give free access to the “talking book” library to make “books on tape” available. A form is available from your doctor that establishes legal blindness to be used for property tax and income tax deductions. As always, if you have any questions please do not hesitate to contact your doctor for more information.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright © 2021-2024 Designs Unlimited of Florida.  All Rights Reserved.

Diabetic Vitreous Hemorrhage

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Eye Anatomy

What is diabetic vitreous hemorrhage?

            Diabetic vitreous hemorrhage means blood has leaked into the vitreous gel of the eye as a result of diabetic damage. The vitreous is a clear gel that fills the center of the eye and helps to hold the retina in place against the eye-wall like wallpaper in a room.  The retina is a thin layer of delicate nerve tissue, which acts like film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.  The retina has many fine blood vessels that may become damaged from diabetes leading to bleeding into the vitreous.  Blood in the vitreous (vitreous hemorrhage) interferes with vision. 

What symptoms does diabetic vitreous hemorrhage cause?

            Diabetic vitreous hemorrhage usually causes many new floaters in the vision.  Floaters may appear as round specks, hair-like or bug-like debris, or clouds moving in your vision as though they were in front of your eye.  They are more noticeable when looking at a blank surface and may interfere with the good vision in the fellow eye.  If vitreous hemorrhage is severe, the vision may be severely limited.  Patients may only see shadows or light, but no details.

            Flashes are brief streaks of light that are usually seen off to the side, especially at night when you turn your head or eyes.  Flashes are caused by vitreous gel pulling on the retina with eye movement.  They may be seen in the setting of diabetic vitreous hemorrhage, but are not worrisome in themselves.

Although many people have occasional floaters or flashes of light, the sudden onset of many new floaters with or without flashes is an important sign of abnormal pulling on the retina by the vitreous.  In some people with these symptoms, the retina may tear and detach resulting in loss of vision.  Therefore, these new symptoms warrant prompt evaluation.

What causes diabetic vitreous hemorrhage?

            Diabetes can cause vitreous hemorrhage by weakening the blood vessels in the retina and by causing the vitreous gel to shrink and pull on the retinal vessels.  Aging also causes changes in the vitreous gel and can cause it to pull on the retina.  In any given patient with diabetes, both weakened retinal blood vessels, as well as tugging on the blood vessels from the vitreous play a role in causing vitreous hemorrhage.  However, in some eyes weakened blood vessels may be the main reason and in other eyes the main reason for bleeding may be tugging from the vitreous.  This is an important issue as diabetic vitreous hemorrhage may be treated differently depending on its underlying cause.   

How is diabetic vitreous hemorrhage treated?

            The most important step is to have a thorough eye examination with ultrasonography.  The ultrasound machine uses sound waves to safely and effectively “look through” the blood in the vitreous to see if the retina is attached.  If a retinal detachment is found, surgery is required in an attempt to repair it.  If no retinal detachment is found on ultrasound exam, your doctor may allow the vitreous hemorrhage to clear on its own with time.  The ultrasound exam may be repeated periodically to assure the retina remains attached.  If the hemorrhage does not clear on its own, vitrectomy surgery as a one-day surgery in the hospital operating room may be considered.  The amount of visual return depends on several factors including the health of the underlying retina.     

            In an effort to prevent additional bleeding, the underlying diabetic retinopathy may be treated with medication injections (e.g. Avastin, Lucentis, or Eylea) into the eye.  These injections can usually be given without significant pain by using anesthetics.  The injections reduce the risk of future bleeding, but do not hasten the clearing of the bleeding that has already occurred.   These medication injections may be especially important if no previous laser (or insufficient laser) has been given for diabetic retinal damage (diabetic retinopathy) prior to the vitreous hemorrhage.  Medication injections do not help with tugging on the retinal blood vessels by the vitreous.  Indeed, in rare cases the injections may increase the tugging.  Therefore, if tugging from the vitreous is determined to be the main factor in causing the diabetic vitreous hemorrhage, injections may not be used.  Instead, vitrectomy surgery is more effective at relieving the tugging.  

            Once the vitreous hemorrhage has cleared over time with observation or with vitrectomy surgery, laser is often used to stabilize the retinal blood vessels that have been weakened from diabetes.  This helps reduce the chances of reoccurrence of vitreous hemorrhage in the future.

What should I be on the lookout for?

            After examination or treatment for a vitreous hemorrhage, you should notify your doctor if you have a burst of new floaters, a loss of side vision, or pain.  Sometimes, retinal tears or a retinal detachment occur at a later date after the examination.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright 2021 Designs Unlimited of Florida.  All Rights Reserved.

Sickle Cell and the Eyes

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anatomy of the eye (click on image to enlarge)

What is Sickle Cell Disease?

            Sickle cell disease is the most common genetic disease, affecting about 400,000 newborns each year. It is caused by a mutation in the gene that codes for the hemoglobin protein that carries oxygen in the blood stream. Sickle hemoglobin tends to clump into a sickle shape when it gives up its oxygen molecule to the tissues it supplies. When this occurs, the red blood cells lose their flexibility and tend to block the small blood vessels in the body. The retina in your eye is like the film inside a camera. The retina “takes the picture” of objects you look at and sends the message to the brain. The retina is a living tissue, which requires blood supplied by tiny vessels. These blood vessels may be damaged in people with sickle cell disease.

Who is at risk of eye problems in sickle cell disease?

            Although more extensive blockage of retinal blood vessels occurs in sickle cell disease, more severe complications (bleeding and retinal detachment) occur in people with a combination of sickle hemoglobin and hemoglobin C (called Hemoglobin SC disease). Some studies suggest men are more likely than women to have loss of vision.

What are the visual symptoms of sickle cell disease?

            Blurring of vision may occur if excess damage occurs to the retinal blood vessels.  Floaters can look like tiny dots or cobwebs moving about in your vision. They may be due to bleeding from the retina into the central gel of the eye. Retinal detachment may cause a dark shadow to appear off to the side (in the peripheral visual field) and may progress to total loss of vision. Pain is rare and may be due to high pressure in the eye (neovascular glaucoma).

What treatment is available?

            There is no cure, but treatment may improve vision or keep the vision from worsening.  If bleeding occurs inside the eye due to blocked blood vessels, floaters are seen by the patient. Medicine injections may help recover vision and may be applied without pain in most cases.  Laser may stabilize or improve the vision. In some situations, surgery is required. The vision may not return to normal following treatment as there may be some permanent damage to the retina. The earlier retinal problems are found, the better the outcome of treatment. Therefore, annual exams are important and it is critical to report new floaters without delay.

By Scott E Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2021 Designs Unlimited of Florida.  All Rights Reserved.

Vision Loss in ARMD

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anatomy of the eye (click on image to enlarge)

Why am I losing vision despite treatment for age-related macular degeneration?

            Age-related macular degeneration (ARMD) is the most common cause of visual loss in older Americans.  Vision may be lost from dry-type or wet-type ARMD: 

  • Dry-type macular degeneration is the most common type and involves the disintegration of the light-sensitive tissues in the macula.  Loss of vision is usually gradual in dry macular degeneration.  Small blind spots interfere with reading numbers in a row and all the letters of a word.  Over time, these blind spots usually enlarge and take away most of the central vision.  
  • Wet-type macular degeneration accounts for about 10% of all cases of ARMD.  It occurs in patients with dry-type ARMD when abnormal blood vessels grow under the macula and cause fluid leakage, bleeding, and scarring of the macula.  Vision loss may be rapid and severe.  Straight lines may appear distorted and the central vision appears blurred early in wet-type ARMD.  Over time, a large blind spot may develop in the center of the vision.  If one eye develops wet ARMD, there is about a 50% chance the other eye will be affected within the next five years.

            There is currently no proven drug treatment to stop dry-type ARMD.  Eye vitamins are prescribed for dry-type ARMD.  However, the main purpose of the vitamins is to stave off the start of wet-type ARMD.  Although AREDS eye vitamins appear to slow the start of blind spots in the vision from dye-type AMD, they do not slow the progression of blind spots once they start. A Meditteranean diet appears to reduce the onset and progression of blind spots from dry-type macular degeneration. Therefore, it is important to limit red meat intake to once per week, eat two servings of whole fruit per day, include fish in the diet, and rely on olive oil rather than other oils with saturated fatty acids. There are a number of on-going research studies to find a treatment to slow or prevent loss of vision from dry-type ARMD.  There is even hope for treatment to reverse the loss of vision from dry-type ARMD.  Your doctor can put you in touch with study centers if you are interested in learning more about or participating in these research studies.  Age-related macular degeneration appears to be an inherited condition.  However, it may be aggravated by factors that cause hardening of the arteries like high blood pressure, high cholesterol, overweight, physical inactivity, and tobacco use.  Efforts to control these factors may be helpful in slowing loss of vision in dry-type ARMD. 

            There are fairly good treatment options for wet-type ARMD.  Injection therapy (Avastin, Lucentis, Eylea, and Beovu) is the first-line treatment for wet-type ARMD.  Lasers are second-line treatment options.  Lasers include photocoagulation (which is rarely used currently) and Visudyne photodynamic therapy.  Treatment of wet-type ARMD is effective at slowing the loss of vision.  Unfortunately, current treatments do not completely prevent the loss of vision from ARMD.  There are several reasons why patients may continue to lose vision during treatment of ARMD:

1. Insufficient treatment

Some eyes require injection therapy every four weeks to optimal effect.  If treatment is given less often, the wet-type ARMD may progress with loss of vision that may be irreversible.  This is an important reason to continue monthly injections in some eyes (as determined by the retinal specialist).  

2. Bleeding despite treatment

Bleeding under the retina from ARMD usually results in some degree of permanent scar tissue and loss of vision.  Bleeding may occur if treatment is not given frequently enough and appears more likely in patients who take blood thinners.  Blood thinners (including aspirin) are usually prescribed to prevent heart attack or stroke.  If they are prescribed, the benefits likely outweigh the risks.  However, if blood thinners are not prescribed for a patient with ARMD, they may be best avoided to reduce the risk of bleeding from ARMD.

3. Progression of dry-type ARMD while wet-type ARMD is being treated

Many patients are not aware that ARMD always starts with the dry-type.  Wet-type ARMD develops later.  Therefore, patients with wet-type ARMD may lose vision over time even though their wet-type ARMD is well controlled.  That in, they may lose vision from a worsening of dry-type ARMD over time.  Complicating this issue is the concern that the very treatment of wet-type ARMD may, in some cases, worsen the dry-type ARMD.  

4. New eye problems develop during treatment of ARMD

The ophthalmologist will look for other problems that may cause a loss of vision unrelated to ARMD.  Common causes of vision loss include cataract, which is treatable with surgery.  Other problems include glaucoma, retinal vein occlusion, and diabetic retinopathy. 

What is to be done about the continued loss of vision? 

            There are a number of actions that may be taken in response to continued loss of vision during treatment of ARMD.  The retinal specialist will look for other causes of loss of vision and start appropriate treatment.  The frequency of treatment may be changed in response to changes in the retina.  Low vision aids (optical and electronic magnifiers) may be helpful.  Specially-trained social workers may help make changes in the household to make it easier to remain self-sufficient.  It is helpful to remember that the peripheral vision is rarely taken by ARMD.  Therefore, although a patient may be determined to be legally blind, total blindness is rare.  Most patients with advanced ARMD are able to ambulate and retain independence.

By Scott E Pautler, MD 

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2021-2022 Designs Unlimited of Florida.  All Rights Reserved. 

Retinal Angiomatous Proliferation

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Anatomy of the eye (click on image to enlarge)

What is retinal angiomatous proliferation (RAP)?

            Retinal angiomatous proliferation means there is a growth (proliferation) of abnormal blood vessels (angiomatous) in and under the retina (specifically, under the central part of the retina called the macula).  Retinal angiomatous proliferation (RAP) is a sub-type of wet age-related macular degeneration (ARMD).  Wet ARMD affects the central vision in older patients due to abnormal blood vessels growing under the macula.  The macula is the area of the retina in the back of the eye that is responsible for seeing details in the central vision.  The retina is a thin layer of delicate nerve tissue that lines the inside wall of the eye like the film in a camera.  In the eye, light is focused through the lens onto the retina, which “takes the picture” and sends the image to the brain. 

What causes retinal angiomatous proliferation (RAP)?

            Retinal angiomatous proliferation (RAP) appears to be caused by the release of blood vessel growth factors in the retina in response to age-related changes.  The age-related changes include the accumulation of cellular waste products under the retina (call subretinal drusenoid deposits).  The build-up of waste products (SDD) under the retina interferes with retinal function.  For one thing, the build-up of SDD separates the retinal cells from the normal blood vessels that nourish them.  Without proper nourishment the retinal cells do not work well.  Furthermore, when the retinal cells perceive that they are not getting enough oxygen and nutrients, they release growth factors (including one growth factor called VEGF).  These growth factors stimulate the growth of new blood vessels to assist in delivering oxygen and nutrients.  In other parts of the body, new blood vessels may grow to help or replace old blood vessels and it is a helpful response to poor blood supply.  However, in RAP the new blood vessels cause loss of vision due to fluid leakage, bleeding, and scarring of the macula.  

What are the symptoms of retinal angiomatous proliferation (RAP)?

            Retinal angiomatous proliferation (RAP) may cause no symptoms in its early stages.  Over time, symptoms may include blurred central vision, distortion of straight lines and/or a central, gray spot in the vision.  In its advanced stages without treatment, RAP may cause a large permanent blind spot in the center of vision.  At this stage no treatment is possible and low vision aids are used to compensate for loss of vision.  If one eye develops wet AMD, there is about a 50% chance the other eye will be affected within the next five years.

How is retinal angiomatous proliferation (RAP) diagnosed?

            Retinal angiomatous proliferation (RAP) is diagnosed in patients with known dry-type age-related macular degeneration.  Before the proliferation or growth of blood vessels under the retina develop in RAP, subtle yellow deposits may be identified under the retina.  These deposits are called subretinal drusenoid deposits (SDD).  SDD develop many years before RAP occurs.  A dilated eye examination can detect SDD and alert the retinal specialist to be on the lookout for RAP.  Retinal angiomatous proliferation is suspected when a patient with SDD develops blurred vision and swelling (edema) is present in the retina on a retinal scan called OCT (optical coherence tomography).  The diagnosis may be confirmed on a more extensive test called fluorescein angiography.  This is a procedure where the ophthalmologist injects an organic dye into the vein of a patient’s arm.  Then, photographs of the retina show the presence and location of the leaking blood vessels marked by the organic dye.

Why is this diagnosis important?            

It is important to recognize RAP because it guides treatment recommendations.  This type of wet ARMD is especially sensitive to antiVEGF therapy (injections with Avastin, Lucentis, and Eylea).  RAP is so sensitive to antiVEGF therapy that the medication injections are sometimes not required as often as they are in other types of wet age-related macular degeneration such as PCV.  Older treatments such as photocoagulation and photodynamic therapy historically do not work well in RAP and can be avoided.  Treatment rarely returns vision to normal, but may limit the amount of vision loss from blood vessel growth and leakage.  Frequent office visits and photographs are needed.  It may be useful to stop smoking, avoid becoming overweight, exercise daily, and control blood pressure and cholesterol. Aspirin should only be used if required to treat disease as recommended by a doctor. Relatives should be checked for macular degeneration, as well.

By Scott E Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2021-2022 Designs Unlimited of Florida.  All Rights Reserved.

Giant Cell Arteritis and the Eyes

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anatomy of the eye (click on image to enlarge)

What is giant cell arteritis (GCA)?

Giant cell arteritis (also called cranial arteritis or temporal arteritis) is an inflammatory condition affecting medium to larger arteries in people over the age of 50 years.  GCA may cause headache, neck stiffness, tenderness of the temple, and/or cramping of the tongue or jaw while eating or talking.  It may also cause fever, fatigue, weight loss, depression, night sweats and general feeling of illness or feeling unwell.  

What causes giant cell arteritis (GCA)?

The cause of GCA is not very well understood.  The inflammation from GCA is not due to infection.  In GCA, it appears the body’s immune system attacks itself, in some ways similar to rheumatoid arthritis.    

How is giant cell arteritis (GCA) diagnosed?

The diagnosis is first suspected by typical symptoms in an older patient.  Blood tests may include complete blood count (CBC), sedimentation rate (ESR), and C-reactive protein (CRP).  Biopsy of the temporal artery is necessary in most cases to confirm to diagnosis.  Firmly establishing the diagnosis is critical, because treatment needs to be started quickly and continued for a long time in GCA. 

How does giant cell arteritis (GCA) affect the eyes?

Giant cell arteritis (GCA) causes loss of vision due to retinal artery occlusion or ischemic optic neuropathy.  Early diagnosis is critical to begin treatment and limit the damage from blood vessel inflammation, which includes complications of brain stroke. 

 How is giant cell arteritis (GCA) treated?

Prompt steroid treatment is important to stop inflammation from causing further damage with loss of vision and brain stroke.  Prednisone is continued at a low dose for a year or more to prevent the inflammation from returning.  In an effort to avoid or manage side-effects of steroids, methotrexate is sometimes used.  Tocilizumab was approved in 2017 for use in GCA.  It avoids the use of steroids, but is very expensive.

By Scott E Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2021-2022 Designs Unlimited of Florida.  All Rights Reserved.

Inflammatory Bowel Disease and Your Eyes

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Anatomy of the eye (click on image to enlarge)

What is inflammatory bowel disease?

Inflammatory bowel disease includes conditions such as ulcerative colitis and Crohn’s disease. They are inflammatory conditions not caused by infection.  Instead, the immune system appears to mistakenly attack the bowel.  The same immune cells may also attack the eye in these bowel conditions. Ocular inflammation is called uveitis.  And the most common type of uveitis caused by inflammatory bowel disease, is intermediate uveitis.

Uveitis (pronounced, “you-vee-EYE-tis”) is a general term used to describe inflammation inside the eye.  The uvea is the name given to the layer of tissue in the eye that has a brown color (melanin pigment) and blood vessels, which serve to provide blood supply and protect the eye from excessive light (see anatomy of the eye).  The uvea can be divided into separate parts, which perform different functions in the eye: the iris, the ciliary body, the pars plana, and the choroid.  The part of the uvea in the front of the eye is called the iris (the round, blue or brown part of the eye that you can see in the mirror).  Behind the iris is the ciliary body, which produces the fluid that fills the eye.  The pars plana serves as the boundary between the ciliary body and the choroid.  The back part of the uvea that lies under the retina (the “film” in the eye that “takes the picture”) is called the choroid.  Therefore, in any one patient uveitis is usually given a more specific name depending on where most of the inflammation is located in the eye. In intermediate uveitis the inflammation is primarily located in the vitreous gel that fills the eye, which is located in an intermediate position between the front and the back of the eye. It is sometimes referred to as vitritis. 

What are other causes of intermediate uveitis?

Uveitis may be caused by an infection, an injury from trauma, a disease in the body outside the eye, or sometimes for unknown reasons.  Infection by a virus, bacteria, fungus, or other parasite may cause uveitis.  Infections may be limited to the eye or may involve other organs as well. In intermediate uveitis, infection may be caused by syphilis, tuberculosis, Lyme disease, cat scratch disease, Whipple’s disease, toxocariasis, human lymphotrophic virus (HTLV-1), or toxoplasmosis.

In other situations, uveitis is caused by inflammation without infection.  For example, multiple sclerosis and sarcoidosis may cause intermediate uveitis. Pars planitis is a sub-type of intermediate uveitis that often starts early in life during childhood. Its cause is unknown.

Uveitis commonly occurs following an injury to the eye.  Very rarely, cancer or cancer-fighting drugs may cause intermediate uveitis. In some cases, no underlying cause can be found to be the cause of uveitis.  Tobacco may be an aggravating factor and should be discontinued.     

What are the symptoms of intermediate uveitis?

The most common symptoms include tiny floating spots which move or “float” in the vision.  They are usually numerous and may cause a veil-like appearance in the vision.  Sometimes blind spots, blurred vision, distortion, or loss of side vision occurs. The eye may be painful, red, tearing, and light sensitive if other parts of the eye are also inflamed.  Symptoms may be mild or they may be severe and disabling.

How is intermediate uveitis managed?

Treatment of the underlying inflammatory bowel disease will also help treat the uveitis.  Separately, it is important to find any other underlying cause whenever possible.  Take some time to carefully review and report to your doctor any unusual or unexplained symptoms such as rashes, back and joint problems.  Tell your doctor if you travel abroad, spend time in rural settings, or may be exposed to animals or infections.  Heredity may also play a role. You should tell your doctor about any family members with inflammatory disorders anywhere in the body. Also, review and report your ancestry (for example, Asian, Mediterranean, or American Indian ancestry).  When the doctor diagnoses uveitis, laboratory tests may be ordered to help determine its cause.  Occasionally, a surgical biopsy is needed for diagnosis.   If infection is found, antibiotics are prescribed.  To limit the damage from inflammation, intermediate uveitis is treated with anti-inflammatory medication in the form of eye drops, injections, or pills.  When pills are used, the eye doctor frequently coordinates medical care with the expert assistance of a rheumatologist.  Rarely, surgery is required to treat uveitis.  In some cases, intermediate uveitis may be long-lasting. In these cases, years of therapy are needed to preserve vision.  Intermediate uveitis is a serious eye problem and may result in loss of vision or blindness.  However, by seeing your eye doctor and taking the medications exactly as recommended, damage to your vision can be minimized.

In some cases, intermediate uveitis may go away, but return at a future date.  Therefore, if you become aware of symptoms of uveitis in the future, do not hesitate to contact your doctor. Preliminary evidence suggests that tobacco use may be an aggravating factor in some cases of uveitis. Also, vitamin D supplements may be helpful. 

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

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Copyright  © 2021-2022 Designs Unlimited of Florida.  All Rights Reserved.

Polymyalgia Rheumatica and your Eyes

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Anatomy of the eye

What is polymyalgia rheumatica (PMR)?

Polymyalgia rheumatica is an inflammatory condition affecting the hips and shoulders of people over the age of 50 years.  PMR may cause sudden shoulder and/or hip pain, especially after or during rest from activity.  It usually affects both sides of the body.  It may also cause fever, fatigue, weight loss, depression, and night sweats. 

What causes polymyalgia rheumatica (PMR)?

The cause of PMR is not very well understood.  The inflammation from PMR is not due to infection, though various infections may need to be excluded before a diagnosis of PMR is made.  In PMR, it appears the body’s immune system attacks itself, in some ways similar to rheumatoid arthritis.    

How is polymyalgia rheumatica (PMR) diagnosed?

The diagnosis is first suspected by typical symptoms in an older patient.  Blood tests may include complete blood count (CBC), sedimentation rate (ESR), and C-reactive protein.  If needed, ultrasound may detect bursitis in the shoulder.  PET/CT scan is also very sensitive and specific for PMR.    

How does polymyalgia rheumatica (PMR) affect the eyes?

When PMR is associated with inflammation of the blood vessels, the eye can become affected.  This closely-related blood vessel inflammation is called giant cell arteritis (also called cranial arteritis or temporal arteritis).  Giant cell arteritis (GCA) causes symptoms similar to PMR with the addition of headache, neck stiffness, tenderness of the scalp, and cramping of the tongue or jaw when speaking or chewing.  These symptoms indicate inflammation of the blood vessels of the head.  Loss of vision may occur due to retinal artery occlusion or ischemic optic neuropathy.  Early diagnosis is critical to begin treatment and limit the damage from blood vessel inflammation, which includes complications of brain stroke. 

By Scott E. Pautler, MD 

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2021-2022 Designs Unlimited of Florida.  All Rights Reserved.

Anatomy of the Eye

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Anatomy of the human eye. Image courtesy of Caitlin Albritton.

The eye is a specialized organ to provide sight.  The various parts of the eye work to assist in this process.  There are two eyes per human (many more if you are a spider or a scallop).  The paired human eyes allow for improved depth perception.  It takes two eyes for a human to best determine how far an object lies in front of him or her.  Each eye has a slightly different view and the difference between the two images is used by the brain to make a three-dimensional (3-D) image.  Test: you can see the difference in depth perception by trying to thread a needle or perform a similar fine task with one eye compared with two eyes. If the two eyes are not lined up straight, a person sees two images (double vision).

Shape of the eye

The shape of the eye is fairly round like a ball.  This design helps the eye determine what direction light is coming from (see video: evolution of the eye).  In near-sighted eyes, the eye becomes somewhat elongated (like an egg).  This appears to be due to an adaptive response of the eye to aid in focusing at near.  However, the elongation of the eye causes the tissues to stretch and this predisposes the eye to retinal detachment.

The Tear Film

This is an often-overlooked part of the eye.  The tears form a smooth surface over the front of the cornea and help avoid scattering of light as it enters the eye.  The tear film also provides nutrition, protection (antibacterial), and cleansing of the surface of the eye.  The tear film is composed of three parts/layers: the aqueous (water), mucin, and lipid layers.  The aqueous component is produced by lacrimal glands that rest in the eye socket behind a bone under the eye brow.  The mucin layer is produced by specialized cells (goblet cells) in the conjunctiva.  The mucin mixes with the aqueous layer and adds structure to the tears helping them to remain on the eye surface.  The lipid layer (outer surface) produced by meibomian glands along the edge of the eye lids. The lipid helps protect against evaporation of the tears from the surface of the eye.  Various conditions and diseases interfere with one or more layers of the tear film leading to dry eyes and reduced vision.

The Conjunctiva

The conjunctiva (not labelled on image) is a smooth moveable outer “skin” overlying the white sclera (see below).  The conjunctiva acts as a physical barrier against entry of infectious organisms, and contains many blood vessels which dilate and become inflamed if infection or foreign objects threaten to damage the eye.  The smooth surface of the conjunctiva and loose attachment to the underlying sclera helps the eye turn smoothly.

The Sclera

The sclera is the tough outer coat of the eye.  It provides structural support and protection of the sensitive tissues (like the retina) inside the eye.  The sclera does allow for the outflow of water that is produced by the ciliary body.  It also allows medications to pass into the eye.  Nerves and blood vessels penetrate the sclera to reach the inside of the eye.

The Cornea

The cornea is the clear window at the front of the eyeball that allows light to enter the eye.  It is continuous with the sclera.  Most of the focusing of light happens at the corneal surface/tear film.  As this outer layer of the eye is so critical for sight, there are many nerve endings in the cornea making it the most sensitive part of the eye to touch.  The outer-most layer is the epithelium: a smooth surface to transmit light with tightly layered cells difficult for infections to penetrate.  The epithelium acts as a barrier to keep the water-filled tears from entering the cornea as this would make the cornea lose its clarity.  The middle layer of the cornea is called the stroma.  It occupies most of the cornea with orderly layers of protein fibers to transmit light and minimize scattering.  There is very little water in the stroma because the inner-most layer of the cornea (the endothelium) pumps out water from the cornea into the eye.  When the cornea becomes hazy or opaque, a corneal transplant may be performed.

The Iris and Pupil

The iris is responsible for the color of the eye as seen from the outside.  A circular opening in the iris is called the pupil, which is seen as a black spot in the center of the iris.  Iris muscles expand and contract to change the size of the pupil and alters the amount of light entering the eye.  In bright light, the pupil constricts so as to prevent too much light from entering the eye.  There is no specific function of iris color and iridology is not a true science.  However, lighter-colored irises (irides) may not block as much light as dark irides.  From a medical perspective, eyes with lighter color eyes may be at higher risk of developing macular degeneration.  Darker eyes are seen among people who evolved nearer the equator.  In birds, iris color appears to play a role in the mating ritual.

The lens

The lens inside the eye is made of specialized crystalline protein fibers that help to focus light and allow for flexibility of the lens in order to focus light from different distances from the eye.  With age the eye loses its ability to focus light; therefore, reading glasses are needed by most people around the age of 40 years.  When the clear lens turns foggy, the lens is called a cataract.  Surgery may be performed to remove a cataract and replace it with a clear plastic lens implant.  

The ciliary body (SILL-ee-air-ee)

The ciliary body has two functions inside the eye.  It contains muscles to focus the lens (the ciliary muscles are attached to the lens by fine fibers called zonules).  The ciliary body also has a pump that produces aqueous (water).  The aqueous pump works to keep the eye inflated.  The aqueous also provides nutrition to the structures inside the eye.  There is a constant flow of aqueous into the eye via the ciliary body and out of the eye through the sclera (i.e. the trabeculum).  If the trabecular drain becomes blocked, the pressure in the eye goes up and damage may occur to the optic nerve.  This condition is called glaucoma.

The Vitreous

The vitreous is a clear gel that fills most of the eye.  There are no blood vessels and very few cells in the vitreous.  The clarity of the vitreous is important in order to allow light to pass from the lens to reach the retina.  The vitreous is mainly composed of water, but there are fine protein fibers and a gel (hyaluronin) providing a unique structure.  If the eye becomes cut from an accidental trauma, the vitreous gel may plug the hole in the sclera keeping the eye from deflating.  The vitreous also appears to protect the lens; there are anti-oxidants in the vitreous gel that help keep the lens clear.  

With age, the vitreous loses its gel-like quality and the protein fibers begin to clump together.  When this happens, fiber-like floaters may appear in the vision.  These floaters occur earlier in life in near-sighted eyes and in eyes following inflammation or trauma. Occasionally, the vitreous fibers will pull on the retina causing brief, streak-like flashes of light in the vision.  The pulling may cause a retinal break (retinal tear), which may lead to retinal detachment.  In other situations, the vitreous fibers may pull on the macula resulting in distortion of vision from vitreo-macular traction syndrome, epiretinal membrane, or macular hole.     

The Retina

The retina is a light-sensitive layer of nerve tissue that lines the inside of the eye wall.  It acts like the film in a camera.  The central portion of the retina is called the macula.  The macula is designed for central vision.  When you read or see fine details, you move your eye so that light focuses on the macula.  The rest of the retina is used for peripheral vision (side-vision).  The peripheral vision is essential for walking around a room without bumping into things.  The peripheral retina is also sensitive to detect movement in the environment.  The blood supply to the inner retinal layers (ten layers in all) comes from retinal blood vessels that enter and exit the eye through the optic nerve.  The outer retinal layers are supplies by blood vessels in the choroid (see below).  Blockage of the blood supply (retinal artery occlusion or retinal vein occlusion) causes a blind spot in the vision or blurred vision.  If the retina becomes detached from the eye wall, it does not function well, and surgery is required to recover vision.  Aging may result in macular degeneration. 

The Choroid

The choroid is a layer of tissue under the retina filled with blood vessels.  This important blood vessel layer provides oxygen and nutrition to the retina.  It also evacuates waste materials from the outer retina and acts as a heat sump, keeping the retina from overheating.  The choroid may be affected by a number of problems including inflammation, blood vessel blockage, and central serous retinopathy.   

The Optic Nerve

The optic nerve connects the nerve tissue of the retina to the nerve tissue in the brain, like wire in an electric circuit.  The optic nerve is sensitive to the pressure inside the eye.  High intra-ocular pressure may cause loss of vision or blindness from glaucoma.  The optic nerve may also be affected by blocked blood flow, inflammation, and pressure from outside the eye (tumors and aneurysms).  

By Scott E. Pautler, MD

Copyright © 2021 Designs Unlimited of Florida.  All Rights Reserved.

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Cotton-Wool Spots

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Anatomy of the globe

What is a cotton-wool spot?

            A cotton-wool spot is the name given to a small white spot in the retina that resembles cotton wool (raw cotton). The retina in your eye is like the film inside a camera. The retina “takes the picture” of objects you look at and sends the message to the brain. The retina is a living tissue, which requires blood supplied by tiny vessels called arteries. If the blood supply to the retina becomes impaired, a small white spot may develop in the inner retina resembling cotton or wool.  This cotton-wool spot is a sign of stressed retina.

What causes a cotton-wool spot?

            There are a wide variety of causes of cotton-wool spots.  Most commonly, they occur along with other changes in the retina from diabetes, high blood pressure, or retinal blood vessel blockage.  However, they may occur due to a great number of other problems including heart diseases, vascular diseases, blood diseases, inflammations, trauma, medications, and infections.  Sometimes, no underlying condition can be identified.  

What are the symptoms of a cotton-wool spot?

            Often cotton-wools spots cause no symptoms at all.  Sometimes they cause a blind spot or dark arc in the vision just off to the side of central vision.  The symptoms come on suddenly and painlessly.  The loss of vision may be temporary or permanent.  Regardless, it usually fades over weeks to months.

What testing is needed?

            Testing depends on the finding on the eye examination.  Sometimes the underlying problem that caused the cotton-wools spots can be determined by the eye exam.  If not, blood tests and scans may be ordered by the ophthalmologist.

What treatment is available?

            Although there is no specific treatment for cotton-wool spots, treatment is directed toward any underlying conditions that caused the cotton-wool spots to appear.  The spots themselves fade away over several months’ time. 

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Retinal photograph showing two cotton-wool spots

Copyright  © 2021 Designs Unlimited of Florida.  All Rights Reserved.

Polypoidal Choroidal Vasculopathy

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globe anatomy
anatomy of the eye (click on image to enlarge)

What is polypoidal choroidal vasculopathy (PCV)?

            Polypoidal choroidal vasculopathy (PCV) is a type of age-related macular degeneration (AMD), the most common cause of visual loss in older Americans.  The macula is the area of the retina in the back of the eye that is responsible for seeing details in the central vision.  The retina is a thin layer of delicate nerve tissue that lines the inside wall of the eye like the film in a camera.  In the eye, light is focused through the lens onto the retina, which “takes the picture” and sends the image to the brain.  PCV is a disease that affects the central vision. It does not affect peripheral vision— the ability to see objects off to the side when looking straight ahead.  This means that PCV alone does not result in total blindness.  

            In PCV, abnormal blood vessels grow under the macula from a deep layer of normal blood vessels (the choroid).  The normal blood vessels in the choroid are usually separated by a tissue membrane from the macula.  However, in PCV abnormal blood vessels start growing from the choroid and invade the tissue beneath the macula.  These abnormal vessels leak fluid and blood under the macula causing loss of vision.  

Image of the retina with bleeding due to PCV causing a blind spot in the vision

What causes polypoidal choroidal vasculopathy (PCV)?

            Polypoidal choroidal vasculopathy appears to be an inherited condition.  PCV may occur in anyone, but it is more common in people who descended from Asia or Africa.  Therefore, genetic factors likely play a role in the cause of PCV.  It may be aggravated by factors that cause hardening of the arteries like aging, high blood pressure, high cholesterol, overweight, physical inactivity, and tobacco use.  

            Before abnormal blood vessels grow under the macula, there are usually findings that predict eyes that are at risk of developing PCV.  For example, the choroid (normal blood vessel layer under the macula) is usually thicker than average.  A thicker choroid may result in higher blood flow beneath the macula that may cause the growth of abnormal blood vessels.  In addition, pale deposits (drusen) may appear under the macula prior to the development of abnormal blood vessel growth.  These deposits may contain waste products of cellular function, as well as cholesterol.  Perhaps, newly growing blood vessels are called on by the macula to clear away the waste deposits.  Regardless, the abnormal blood vessels threaten loss of vision due to leaking, bleeding, and scarring beneath the macula.           

What are the symptoms of polypoidal choroidal vasculopathy (PCV)?

            Polypoidal choroidal vasculopathy may cause no symptoms in its early stages, especially if the abnormal blood vessels are located away from the center of the macula or if they have not begun to leak significantly.  Eventually, symptoms may include distortion of central vision or a blind spot in the vision.   

How is polypoidal choroidal vasculopathy (PCV) diagnosed?

            A dilated eye examination can often detect changes in the macula before visual loss occurs from PCV.  The hallmark of PCV, as well as other forms of macular degeneration, is the presence of drusen—tiny yellow deposits of waste products from the retinal cells that appear as spots under the retina.  After the diagnosis is made, a fluorescein angiogram may be needed.  This is a procedure where the ophthalmologist injects an organic dye into the vein of a patient’s arm.  Then, photographs of the retina show the presence and location of the leaking blood vessels marked by the organic dye.

How is polypoidal choroidal vasculopathy (PCV) treated?

            There is evidence that taking vitamin/mineral supplements in specific dosages decreases the risk of visual loss from PCV.  For high risk eyes, the following supplement is recommended: Preservision Soft Gels AREDS 2 Formula one capsule twice-a-day. To avoid toxic side effects, be careful about taking additional vitamins or zinc.  However, you may take calcium, iron, and vitamin D if recommended by your doctor for problems not related to your eyes. Check pricing of Preservation on Amazon.

            People with PCV can often be helped with medication injections and a special laser (PDT) performed in the office.  The Everest Study found that the combination therapy with medication injection and PDT (photodynamic therapy) was more effective than medication injection alone. The combination treatment group recovered more vision and required fewer treatments by injection. This treatment regimen differs from other types of age-related macular degeneration.

Treatment rarely returns vision to normal, but may limit the amount of vision loss from blood vessel growth and leakage.  Frequent office visits and photographs are needed.  It may be useful to stop smoking, avoid becoming overweight, exercise daily, and control blood pressure and cholesterol. Aspirin should only be used if required to treat disease as recommended by a doctor. Relatives should be checked for polypoidal choroidal vasculopathy, as well.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

NOTE: As an Amazon Associate I may earn from qualifying purchases.  You pay no additional fees by accessing the link.  These funds help defray the costs of maintaining this website. Thank you for supporting this blog.

Copyright  © 2021 Designs Unlimited of Florida.  All Rights Reserved.

Asteroid Hyalosis

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Eye
Vitreous is the gel that fills the eye (click on image to enlarge). Image courtesy of Caitlin Albritton.

See Anatomy of the Eye

What is asteroid hyalosis?

Asteroid hyalosis is a fairly rare, harmless eye condition in which calcium crystals form inside the eye.  The name comes from the fact that the calcium crystals look like asteroids on the examination by the eye doctor and are seen in the vitreous gel (also known as the hyaloid) that fills the eye.  These calcium crystals form slowly over time, usually in just one eye.  Asteroid hyalosis is not considered an eye disease and rarely causes problems with the vision.  

What causes asteroid hyalosis?

 The cause is not known.  Limited research has been carried out because asteroid hyalosis does not harm the eye.  It is usually well-tolerated and may be observed by the eye doctor.   Asteroid hyalosis is not associated with calcium-related problem outside the eye.   

What symptoms may be seen?

Many patients have no symptoms at all, but sometimes floaters are seen.  Floaters are small specks, fibers, or bug-shaped objects that may appear to move in front of your eye.  At times they may appear like a veil or cloud moving in the vision.  They are frequently seen when looking at a blank wall or blue sky.  Usually, they can be ignored and tolerated.

What should be done about the symptoms?

The most important step is to have a thorough dilated eye examination.  The eye doctor will check for the presence of a tear in the retina.  If the retina is stable, the asteroid hyalosis may be observed without treatment.  If the floaters become a problem for the patient and interfere with vision, vitrectomy surgery may be considered to remove the asteroid hyalosis.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Photograph of asteroid hyalosis appearing as white clumps inside the eye (vitreous) obscuring the view of the retina in the background.

Copyright 2021-2022 Designs Unlimited of Florida. All Rights Reserved.

Birdshot Chorioretinopathy

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What is birdshot chorioretinopathy?

Eyeball Anatomy

See Anatomy of the Eye

Birdshot chorioretinopathy (BSC) is a type of uveitis (pronounced, “you-vee-EYE-tis”), a term used to describe inflammation inside the eye.  BSC mainly causes inflammation of the choroid and retina, but may affect other parts of the eye as well.  The choroid is the part of the uvea that lies under the retina, which is the “film” in the back of the eye that “takes the picture” of objects you look at.  BSC is fairly rare form of inflammation affecting both eyes of men and women, usually starting in middle age.

What causes birdshot chorioretinopathy?

Birdshot chorioretinopathy (BSC) is strongly related to genetics.  Most people with BSC have inherited a cell protein called HLA-A29.  However, most individuals with HLA-A29 do not develop BSC; it appears to be triggered by an external event, such as an infection that “awakens” the immune response, which then abnormally attacks the eyes.  BSC is most common in people of European ancestry.       

What are the symptoms of birdshot chorioretinopathy?

Birdshot chorioretinopathy (BSC) usually presents with the slow-onset of floaters and blurred vision in both eyes.  The floaters appear as tiny floating dots, which move or “float” in the vision and are seen especially well in bright environments.  Shimmering lights may also be reported. Some patients note difficulty seeing at night. Symptoms may be very bothersome despite normal vision as measured on the eye chart.  Over many years without treatment, the vision deteriorates further with loss of contrast, color vision, peripheral vision, and central vision.  The symptoms vary from person to person and some have more rapid and severe deterioration than others.

How is birdshot chorioretinopathy diagnosed?

The diagnosis of birdshot chorioretinopathy (BSC) may be delayed due to the slow onset of symptoms and the subtle findings on the eye exam.  A retinal specialist or uveitis specialist may be needed to perform sophisticated testing and make the diagnosis.  Inflammation may be detected in many different parts of the eye, but the most typical findings include numerous pale spots inside the back of the eye.  Blood testing for HLA-A29 is positive in the vast majority of patients with BSC.  However, not all patients with uveitis who are positive for HLA-A29 have birdshot chorioretinopathy.  Therefore, it is necessary to exclude other diseases that may simulate BSC including lymphoma, sarcoidosis, tuberculosis, syphilis, and cancer medications such as pembrolizumab and others.    

How is birdshot chorioretinopathy managed?

Birdshot chorioretinopathy (BSC) usually requires management by an experienced retinal or uveitis specialist.  In most cases, systemic treatment (pills or injections into the skin) are needed to control the inflammation.  In a small subset of patients, localized treatment to the eye is sufficient.  This is more often the case in older patients at onset of symptoms.  When pills are used, the eye doctor frequently coordinates medical care with the expert assistance of a rheumatologist (a medical specialist with expertise in auto-immune diseases, like rheumatoid arthritis).  In BSC the rheumatologist monitors the patient for medication side-effects that may develop outside the eyes. In many cases, the uveitis may be long-lasting. In these cases, years of therapy are needed to preserve vision.  

Your doctor will choose from a variety of medications. Steroids (pills, eye drops, and injections) may be used at the start of treatment to gain rapid control of inflammation.  However, long-term steroid treatment in high doses is usually avoided to prevent side-effects of steroid therapy.  For long-term control methotrexate (MTX) pills or skin injections may be given weekly.  MTX has a long record of safety and is affordable.  If MTX fails or causes side-effects (liver or bone marrow), CellCept is another suitable medication, though it may cause diarrhea.  Cyclosporin has been used effectively, but is fraught with a high incidence of problems with hypertension (high blood pressure) and kidney toxicity.  Humira is a new biologic treatment given as an injection into the skin every two weeks.  It has been approved by the FDA for treatment of uveitis, such as BSC.  All medications used to treat BSC may have adverse effects and must be monitored for effectiveness and safety in a given patient. 

Birdshot chorioretinopathy is a serious eye problem and may result in loss of vision or blindness.  However, by seeing your eye doctor and taking the medications exactly as recommended, damage to your vision can be minimized.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2020-2023 Designs Unlimited of Florida.  All Rights Reserved.

Eye Care by Internet & Phone

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Questions?

Do you need eye care, but are fearful of COVID-19?

Do you need a second opinion on your eye problem?

Do you need an accurate diagnosis?

Do you need to know the best treatment options?

How can we help?

Now you can obtain high quality information on the phone, on videoconference (Facetime, Skype, etc), and by e-visit on a confidential computer portal.  

We offer assessments and treatment recommendations.  We review your history directly, examine your eyes with a video device, and review photos and charts from past exams.  If you live near Tampa, we are available to see you in the office.  If you are distant, we can put you in contact with superb physicians closer to you.  

What does it cost?

There is no charge to you with Medicare and most insurances.  If you have no insurance, the cost is $35 for the first visit.  Additional fees may apply if there is a need for review of photos and medical records. 

Does it matter where I live?

Although it is best if you live near Tampa, we may be able to help even if you live afar.  If you are local, we are available to examine your eyes directly if needed. If you live distant from Tampa, we can refer you to a reputable eye-care specialist in your area if you need an examination.

How do I make contact?

Call 1-888-622-8521.  Ask the receptionist for a virtual appointment with Scott E. Pautler, MD.  They will establish a patient account and arrange for a call-back within 24 hours.  

Send a secure email to Dr Pautler: spautler@retinavitreous.intellechartdirect.net 

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Indicate your interest in a virtual exam and briefly state your eye problem.  We will return your email or contact you by phone at your preference.

Serine and MacTel

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What is MacTel?

MacTel (Macular Telangiectasia) is a degeneration of the center of the retina (called the macula) that affects central vision. The macula is a type of nerve tissue that works to give sharp central vision to read and see fine details. There is evidence that an amino acid called serine plays a role in the cause MacTel.1

How does serine relate to MacTel?

Serine is an amino acid that is used by the body to build proteins and lipids. If this building block is not used properly by the body, abnormal nerve lipids (deoxysphingolipids) may accumulate and damage nerve cells.

 In an inherited condition (hereditary sensory and autonomic neuropathy type 1) an abnormal enzyme causes abnormal nerve lipids in the body and can cause nerve damage. Peripheral nerve damage may cause numbness and tingling of the hands and feet. Autonomic nerve damage may interfere with internal organ function (e.g. intestines, bladder, heart). In addition, these patients frequently develop MacTel.  

Even without this inherited condition of neuropathy, patients with MacTel often have low blood levels of serine that result in high blood levels of abnormal nerve lipids. These abnormal nerve lipids have been shown to damage retinal cells and likely play a role in loss of vision in MacTel.

 What can be done with this information?

At present (1-2020) the authors of the research paper advise against starting treatment based on their paper. They caution that more research is needed. However, the FDA found that over-the-counter L-serine supplements to be generally safe. One study found the use of L-serine (400mg/kg/day) safely lowered the abnormal nerve lipids in a case of hereditary sensory and autonomic neuropathy.2 Side effects of taking L-serine include stomach discomfort, diarrhea, constipation, and frequent urination. Most supplements come in the form of capsules containing L-serine 500mg. It is unknown what dosage might be most effective for MacTel. A patient may wish to take the dosage recommended on the bottle by the manufacturers.

Check for current prices of L-serine on Amazon.

Another option is the use of fenofibrate, a prescription medication that can lower the abnormal nerve lipid levels. This option may be especially useful in patients with MacTel who have abnormal cholesterol and/or triglycerides because fenofibrate has already been approved for use in the treatment of these conditions apart from potential benefit for MacTel. 

In general, patients with MacTel who also have symptoms of sensory or autonomic neuropathy should notify their retinal specialist and internist for additional testing and consider treatment.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

References:

1. Gantner, et al. Serine and lipid metabolism in macular disease and peripheral neuropathy. N Eng J Med 2019;10:1422-1433.

2. Auranen et al. Clinical and metabolic consequences of L-serine supplementation in hereditary sensory and autonomic neuropathy type 1C. Cold Spring Herb Case Stud 2017;3:6.

Please note: As an Amazon Associate I may earn from qualifying purchases.  You pay no additional fees by accessing the link.  These funds help defray the costs of maintaining this website. Thank you.

Pentosan (Elmiron) and Your Eyes

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See Anatomy of the Eye

What side effects can pentosan polysulfate have on my eyes?

Although pentosan polysulfate (PPS) is of proven benefit for interstitial cystitis, it may cause damage to the eyes.  Early symptoms may be subtle.  Blurred vision, especially with reading, is common.  Straight lines may appear wavy or distorted.  There may be a slow adaptation from light to dark environments.  Blind spots or missing areas may occur in the central vision.  These symptoms are due to retinal damage; however, they are not specific to pentosan polysulfate damage.  The Eye MD (retina specialist) must use special tests to determine whether vision symptoms are due to pentosan polysulfate or other types of retinal conditions such as macular degeneration.    

Who is at risk of losing vision?

Ocular side effects appear to be related to a build-up of medicine in the body over years.  The longer a person has been on PPS, the greater the chances of developing retinal damage.  Although the average duration of use at the time of diagnosis is 15 years, some patients develop symptoms as early as three years after starting the medication.  With continued use of PPS, additional permanent damage occurs that may result in loss of vision.

What can I do to protect myself?

Pentosan polysulfate is an effective medication for control of pain with interstitial cystitis.  However, it is important to monitor your eyes for side effects that might indicate the need to stop the medication.  The Amsler grid chart should be checked at least once a week testing each eye separately, using glasses if needed.  Look for a missing part of the grid either above or below the central dot while looking only at the center of the grid.  Additionally, your Eye MD should examine your eyes every year with specific testing to look for early signs of retinal changes.  It may be useful to see a retinal specialist who has training in this area.

What happens if I develop retinal changes from pentosan polysulfate?

If early retinal changes are found, pentosan polysulfate may be discontinued.  By discontinuing pentosan polysulfate at an early stage, vision may be saved.  Continued examination is important to monitor the eyes for further changes.  There is no specific treatment for retinal toxicity from pentosan polysulfate. However, if blood vessels grow under the retina, treatment may be helpful as with wet macular degeneration.

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2019-2022 Designs Unlimited of Florida. All Rights Reserved.

Retinoschisis

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See Anatomy of the Eye

What is the retina?

            The retina is a “tissue-paper” thin layer of nerve tissue, which lines the inside of the eye like the film in a camera.  In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain.  The retina has many layers of cells and fibers that normally adhere together for the retina to work properly.

What is retinoschisis (pronounced “retino-SKEE-sis”)?

            In Retinoschisis the retina splits into two layers that separate from each other.  The outer layer of the retina usually remains attached to the inside of the eye wall.  The inner layer of the retina floats inside the eye as fluid fills the space between the two layers.  It is similar to what happens to multi-ply tissue paper in a sink.  The layers of tissue paper may float apart from each other.   

What causes retinoschisis?

            Retinoschisis is caused by a weakness of the cells that normally hold the layers of the retina together.  Retinoschisis is more common in people who are far-sighted.  This tendency may be hereditary.    

What are the symptoms of retinoschisis?

            Retinoschisis usually causes no symptoms.  It is usually found on a routine dilated eye exam and becomes an important issue because it resembles retinal detachment.  

How is retinoschisis treated?

            Retinoschisis does not usually require treatment because it rarely causes any visual symptoms or problems.  However, retinal detachment may be caused by retinoschisis. Therefore, it is important to report any loss of side vision, like a curtain or shadow.  These symptoms may be a sign of retinal detachment, which does require treatment to prevent blindness. 

By Scott E. Pautler, MD

For a telemedicine consultation with Dr Pautler, please send email request to spautler@rvaf.com. We accept Medicare and most insurances in Florida. Please include contact information (including phone number) in the email. We are unable to provide consultation for those living outside the state of Florida with the exception of limited one-time consultations with residents of the following states: Alabama, Arkansas, Connecticut, Georgia, Minnesota, and Washington.

Copyright  © 2019-2022 Designs Unlimited of Florida.  All Rights Reserved.