Idiopathic Perifoveal Telangiectasia

Idiopathic Perifoveal Telangiectasis is commonly referred to as macular telangiectasia, a retinal disorder that affects the center most part of the macula. This poorly understood disease is most common in people between 40 to 60 years old. Dilated and leaky retinal capillaries develop around the temporal part of the foveal area and may eventually encompass the entire area, causing pigmentation to migrate below the retina and into the vitreoretinal interface. As this progresses, visual clarity decreases.

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What Is Parafoveal Telangiectasia?

The term parafoveal telangiectasia has a wide variety of names, including macular telangiectasia, idiopathic parafoveal telangiectasia, idiopathic perifoveal telangiectasia, perifoveal telangiectasia, juxtafoveal retinal telangiectasia, telangiectasis, and MacTel. The condition is quite rare and impacts the small blood vessels in the retina, leading to abnormal dilation (enlargement).

Parafoveal telangiectasia is usually divided into a few types, with different blood vessel changes for each. Based on Dr. Yannuzzi’s study, which reviewed the frequency and nature of idiopathic macular telangiectasia and classified the disorders based on new clinical and imaging observations, there are 2 distinct types of this condition, including:

Type 1 aneurysmal telangiectasia is characterized by enlarged or bulging blood vessels that leak and cause macular edema formation, which leads to vision loss. Usually occurring in one eye, it is more prevalent in younger individuals (typically males) and is believed to be a variation of Coats disease.
Type 2 perifoveal telangiectasia is the most common form of the condition, which also involves swelling and leakage of blood vessels. Sometimes the retina can develop new, abnormal blood vessels that leak fluid or bleed, affecting your central vision and leading to scarring of the central retina with a risk of deteriorating your eyesight even further. It is common in people in their 40s and 50s, typically occurring in both eyes and affecting each differently.

There is also a third type of macular telangiectasia called occlusive telangiectasia which is extremely rare and poorly understood. It is characterized by progressive visual loss with more diseased vessels and occlusion of the capillaries of the fovea in both eyes.

What Are the Symptoms of Parafoveal Telangiectasia?

The symptoms of Macular Telangiectasia can vary depending on the stage and severity of the condition. Early stages may not involve any symptoms or lead to only mild visual changes.

Symptoms you may develop as the condition progresses include:

Loss of sharpness and clarity in the central vision
Difficulty seeing objects directly in front of you
Trouble distinguishing colors or colors appearing less bright or vivid
Struggle to read the small print
Flashes of light or visual disturbances

If you are experiencing any of these symptoms, it is important to see a telangiectasia doctor for a comprehensive exam to slow the progression of the disease and preserve your vision.

What Are the Causes of Parafoveal Telangiectasia?

Parafoveal Telangiectasia is a rare retinal disorder, and its exact cause is not fully understood.

However, some factors believed to result in the condition include:

Mutations in the COL4A2 and COL4A4 genes. These genes are involved in the development and maintenance of the basement membrane, which gives structural support to the blood vessels in the retina.
Irregular blood flow in the retinal vessels. Reduced blood flow and oxygen supply to the fovea may result in the growth of abnormal blood vessels as well as fluid and blood leaks into the surrounding tissue.
External factors such as a toxin or radiation exposure, as well as a history of smoking or diabetes. More research, however, is required to understand the impact of these factors fully.

What Are the Treatment Options for Parafoveal Telangiectasia in NYC?

Though there is no effective cure for macular telangiectasia at the moment, your doctor can help control symptoms and prevent further vision loss.

Some of the treatments the specialist may recommend include:

Intravitreal injections of anti-vascular endothelial growth factor (VEGF) medications delivered directly into the eye to help reduce inflammation and swelling and improve vision.
Laser treatment can help seal off leaky blood vessels and reduce the formation of new ones, slowing the progression of the disease and stabilizing the vision. Laser treatment is often used in combination with other telangiectasia treatments, such as intravitreal injections, to provide the best possible outcomes.
Surgery such as Vitrectomy may occasionally be necessary. Vitrectomy involves the removal of the vitreous gel inside the eye in cases where there is significant bleeding or scarring. The procedure can help restore vision and improve the overall health of the eye.

As the best telangiectasia specialist in NYC and the entire country, Dr. Yannuzzi can precisely determine the stage and severity of your condition and find the most appropriate treatment options to preserve your eyesight. Contact VRMNY and achieve healthy eyes.

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Updated on Apr 24, 2023 by Ophthalmologists & Retina Specialists of Vitreous Retina Macula Consultants of New York