Other Conditions

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Other Conditions

Uveitis

Uveitis develops when inflammation targets the healthy tissue inside the uveal layer (or uvea) of the eye, the middle one between the retina and the sclera, the eye’s white part. Depending on the specific subtype, uveitis can be serious, as the uvea is home to components integral to vision. In severe cases, it may result in eye damage or vision loss, making regular, detailed detection essential. However, with early intervention, patients have a good chance of maintaining their vision.

Demographics

Globally, uveitis is the third leading cause of blindness, but it is still considered to be relatively rare. While anyone can develop this condition, it’s most common in people ages 20-60. Uveitis targets an estimated 38 out of 100,000 people in the United States, with about 43,000 new cases being diagnosed each year. 5-10% of patients are under the age of 16, yet more children will develop blindness than adults.

Uveitis can affect one or both eyes, and while it may quickly go away, it can return. It may also progress to a chronic condition. Uveitis may develop when your immune system is fighting off an infection, or if healthy eye tissue is attacked. This inflammation may be your eye’s response to disease, infection, injury, or toxins. In most cases, uveitis has no known cause, although the following risk factors may be associated:

• Previous eye surgery, damage, or injuries
• Underlying autoimmune illnesses, such as rheumatoid arthritis or lupus
• Bacterial, viral, or parasitic infections
• Family history
• Smoking

Clinical Appearance & Symptoms

There are four different uveitis subtypes, each diagnosed by the inflammation's location.

• Anterior uveitis, the most common subtype, involves swelling and inflammation in the eye’s front portion.
• Intermediate uveitis affects the eye’s peripheral retina, responsible for side and night vision, and the vitreous, the clear, jelly-like gel that makes up most of the eye’s volume.
• Posterior uveitis affects the eye’s back portion, including the choroid, a spongy, pigmented vascular layer of blood vessels and connective tissue connecting the sclera to the retina.
• Panuveitis involves inflammation in all of the uvea, and it may even affect the entire eye.

Among uveitis patients, symptoms can vary, depending on the inflammation site. They may occur gradually or quickly, once or multiple times, in stages, and in one or both eyes. As it can permanently affect your vision, you must tell your ophthalmologist as soon as these symptoms appear: eye redness, eye pain, floaters, blurriness, light sensitivity, and decreased vision.

Diagnosis

Uveitis is typically diagnosed by a complete, dilated ophthalmological eye examination, including your full medical history. Your doctor will determine if blood testing, X-rays, and an overall health evaluation are needed. This exam will include:

• An assessment of visual acuity, the ability to distinguish objects’ shapes and details at a given distance, to determine if your vision has worsened.
• Ophthalmoscopy (or fundoscopy), a thorough inspection of the eye’s back part (fundus), including the retina, optic disc, choroid, and blood vessels.
• Slit-lamp examination, which detects inflammation with an illuminating microscope.
• Tonometry, to check if a steady level of fluid is draining into your eye, ensuring stable intraocular pressure. If this drainage stops, fluid accumulates and pressure rises, damaging the optic nerve.
• Advanced imaging techniques, like optical coherence tomography (OCT) and fluorescein angiography.

Treatment

Uveitis treatment will depend on the inflammation’s source. You may be prescribed anti-inflammatory eye drops or oral medications, especially in more severe cases. Corticosteroid eye drops are usually prescribed to help reduce redness, inflammation, discomfort, and pain. Should symptoms return with medications, you may be referred to a specialist. Depending on your condition, you may be prescribed steroid tablets, injections, or immunosuppressive medications, or undergo surgery.

Lattice Degeneration

With lattice degeneration, the tissue of your retina’s peripheral (side) portions become thinner, forming a lattice-like pattern. This can lead to retinal tears, breaks, holes, and detachment. While there is no prevention or cure, treatment is usually unnecessary, although procedures may repair damage and avoid additional vision loss. Symptoms may not be apparent with this condition, so regular dilated eye exams are essential.

Demographics

An estimated 8-10% of the general population is afflicted with lattice degeneration, and no incidence in gender or race has been found. Should this condition develop in one eye, you’re more likely to develop it in the other. And while it targets peripheral vision, central vision is left intact.

There are no known causes, although this condition may be linked with low or inadequate blood flow to the eyes. Often, lattice degeneration is found in patients with myopia (nearsightedness). While not passed down from parents, it may develop in people with a family history. It may also occur in rare conditions impacting eye and vision health, including Stickler syndrome and Marfan syndrome.

Clinical Appearance & Symptoms

Lattice degeneration has no symptoms, although it may, rarely, develop into a retinal tear or detachment. Research shows that an estimated 20-30% of patients with a rhegmatogenous retinal detachment, the most common type, also have lattice degeneration. But the reverse is not true, as lattice degeneration patients do not develop retinal detachments. Among detachment symptoms to be aware of are blurred vision, floaters, flashing lights, a curtain obscuring your peripheral visual field, and general vision quality issues.

Diagnosis

Lattice degeneration can only be diagnosed with an annual comprehensive, dilated eye examination. Typically, imaging tests are unnecessary, although your retina may be inspected and wide-angle retinal photographs may be taken to assist in monitoring. Scleral depression, slight pressure exerted around your eye, may also be used.

Treatment

Lattice degeneration treatment is typically unnecessary, other than regular monitoring. Rarely, doctors may recommend preventive steps to repair damage and prevent additional vision loss. Laser therapy or cryotherapy (freezing treatment) may be performed to strengthen the retina’s weakened areas.

Fortunately, most lattice degeneration patients do not develop retinal tears or detachment. But if they do develop, a retina specialist may perform laser therapy or cryotherapy. And should surgery be required, options include:

• Scleral buckle, in which a flexible piece of material is implanted to secure the retina in its proper place. Your surgeon will also remove any vitreous that’s seeped under the retina.
• Pneumatic retinopexy, in which a gas bubble is injected into the eyeball’s vitreous cavity to push and close the torn retina. While healing, new fluid is produced to fill the eye. This procedure is performed with laser therapy or cryotherapy.
• Vitrectomy, removal of the vitreous, enables the surgeon to repair the detached retina or remove the force pushing it out of place. The vitreous is typically replaced with a gas bubble.

Retinitis pigmentosa

A group of rare, genetic eye diseases targeting the retina, retinitis pigmentosa causes retinal cells to break down. Symptoms typically begin in childhood or adolescence, and over time, most vision is lost, although total blindness doesn’t occur. While no cure is known, patients may find that vision aids and rehabilitation programs help to make the most of their remaining vision.

Demographics

Retinitis pigmentosa, or hereditary retinal dystrophy, is the most common inherited retinal disease. It affects 1 in 4,000 people in the United States and 1 in 5,000 worldwide, and both men and women can develop it.

Generally, it's caused by changes in genes that control retinal cells. These altered genes are passed down in families. If you have retinitis pigmentosa, it may be helpful to speak with your doctor or a genetic counselor to learn more about your risk of passing it on to your children.

With this condition, the type and speed of vision loss varies, depending on its form. Retinitis pigmentosa may also rarely develop due to some medicines, infections, or eye injuries.

Clinical Appearance & Symptoms

Generally, the most common early symptom is loss of night vision, often starting in childhood. Parents may notice their children having difficulties adjusting to dim light or moving around in the dark.

You may experience loss of peripheral vision, making it hard to see out of the corners of your eyes. As this worsens, your field of vision narrows until you only have some central vision, known as tunnel vision. But retinitis pigmentosa can also cause central vision loss. Most patients eventually lose both their side and central vision, although the rate may vary. You may also experience sensitivity to bright light and the loss of color vision.

Diagnosis

A comprehensive dilated eye exam is required to diagnose retinitis pigmentosa. This enables ophthalmologists to monitor symptoms and help find treatments. Your exam will include a visual field test to check peripheral vision, along with such tests as:

• Genetic testing, to study blood or tissue samples to identify certain associated genes. It can also help determine a condition’s likely course or severity and whether gene therapy can replace faulty genes.
• Electroretinography (ERG), to measure the retina’s electrical activity and how it responds to light.
• ­Optical coherence tomography (OCT), an imaging test using light waves to take detailed retinal pictures.
• Fundus autofluorescence (FAF) imaging, where eye doctors use blue light to take retinal photos.

Treatment

As over 100 genes cause retinitis pigmentosa, it has no single treatment. Researchers are working to develop potential gene therapies, cell therapies, and medications. For example, the ARGUS II implant, a type of “artificial retina,” may help patients with severe vision. And one form, caused by a specific genetic defect, is now being treated with a drug injected under the retina during vitrectomy surgery.

People with retinitis pigmentosa may be more likely to have other eye problems. Regular eye exams help ophthalmologists to diagnose and treat these earlier, helping to preserve remaining vision. These conditions include:

• Refractive errors, such as nearsightedness (myopia), farsightedness (hyperopia), astigmatism (far-away and nearby objects look blurry or distorted), and presbyopia (up close issues)
• Cataracts, clouding of the eye's lens
• Cystoid macular edema, small pockets of fluid in the retina’s center

Research suggests that certain supplements — including vitamin A, lutein, and fish oil — may help slow the resulting vision loss of retinitis pigmentosa. Low vision aids and devices, along with rehabilitation (training) programs, may help patients make the most of their remaining sight.

Retinoschisis

Retinoschisis is a rare condition in which the retina splits, usually at the center, although it also at its periphery (outer edges). There are two different forms, based on their causes. One is due to genetics, affecting boys and young men, while the other is age-related, developing in men and women. Retinoschisis can be serious, resulting in vision problems and retinal detachment. However, it may also be mistaken for detachment, requiring immediate surgery. But regular dilated eye exams enable early detection.

Demographics

The genetic form of this condition, known as congenital X-linked, or juvenile retinoschisis, affects one in 5,000-25,000 people. Typically, it develops in male infants, boys, and young men. While women can develop it, they usually retain their normal vision. The age-related form, known as degenerative, senile, or acquired retinoschisis, affects about 1-4% of patients over age 50. While younger patients may develop this condition, it generally occurs in men and women in their 50s-70s.

With juvenile retinoschisis, the cause is unknown, and the only risk factor is heredity. This condition lasts throughout patients’ lives, and while vision may stabilize, it often worsens in late adulthood, making periodic eye exams mandatory. Degenerative retinoschisis also has no known cause, but risks increase with age, especially after 40.

Clinical Appearance & Symptoms

While juvenile retinoschisis generally has no symptoms, should they develop, you’ll likely experience central or peripheral vision loss. Other common symptoms and complications include:

• Cataracts
• Crossed eyes (strabismus)
• Abnormal eye movements (nystagmus)
• Lazy eye (amblyopia)
• Farsightedness
• Vitreous hemorrhaging (heavy bleeding)
• Total blindness

Degenerative retinoschisis also has no symptoms. But should it worsen, you may experience:

• Floaters and flashes of light
• Distorted images
• Central or peripheral vision loss, depending on the split’s location.

Retinoschisis symptoms are very similar to those for retinal detachment. Retinoschisis patients are also more likely to develop detachment. Their symptoms are so similar that peripheral retinoschisis is often mistaken for retinal detachment.

Diagnosis

The typical diagnostic tests for juvenile retinoschisis include:

• Genetic tests
• Optical coherence tomography (OCT)
• Electroretinography (ERG)
• Ultrasound, in which soundwaves create pictures in the eye.

For degenerative retinoschisis, common diagnostic tests include:

• Visual acuity
• Ophthalmoscopy, including dilation
• Slit lamp exam
• Field of vision

Due to the similarities between retinoschisis and retinal detachment, regular comprehensive ophthalmologic exams are vital.

Treatment

Both retinoschisis forms have no cure, although in many cases, patients will not require treatment. But they may lead to considerable vision loss, even legal blindness. Patients may manage this by wearing special glasses for refractive errors. The use of low vision aids and adaptive technologies may be particularly helpful for juvenile retinoschisis patients. It’s important to always let your ophthalmologist know about any vision changes.

Should retinoschisis develop into retinal detachment, this requires immediate medical treatment, typically laser therapy or cryotherapy. If surgery is needed, common procedures include:

• Scleral buckle
• Pneumatic retinopexy
• Vitrectomy

Researchers are exploring potential treatments for juvenile retinoschisis. These potential therapies include gene therapy, stem-cell therapy, and a drug, dorzolamide.