Idiopathic Juxtafoveal Telangiectasis (Macular Telangiectasia Type 2)

Idiopathic juxtafoveal telangiectasis, more commonly called macular telangiectasia type 2 (MacTel Type 2), is a rare retinal disease that affects the small blood vessels and nerve cells in the macula. The macula is the central part of the retina responsible for detailed vision needed for reading, driving, and recognizing faces.

In MacTel, tiny abnormal blood vessels develop near the fovea, the center of the macula. Over time, the disease can lead to gradual loss of central vision.

MacTel usually affects adults in middle age or later and often occurs in both eyes.

What Causes MacTel?

The exact cause of MacTel is not fully understood. Researchers believe the condition involves degeneration of specialized retinal support cells along with abnormal blood vessel changes in the macula.

Unlike diabetic retinopathy or retinal vein occlusions, MacTel is not primarily caused by poor circulation or blockage of blood vessels.

The disease tends to progress slowly over many years.

Symptoms of MacTel

Symptoms often begin gradually and may initially be subtle.

Common symptoms include:

Blurred central vision
Difficulty reading
Distorted vision
Missing or blank spots in vision
Trouble recognizing faces
Reduced contrast sensitivity

Many patients notice increasing difficulty with fine visual tasks even if standard vision testing remains relatively good early in the disease.

Peripheral vision is usually preserved.

How Is MacTel Diagnosed?

A retinal specialist can diagnose MacTel using a combination of examination and advanced retinal imaging.

Optical Coherence Tomography (OCT)

OCT is one of the most important tests for MacTel. It creates detailed cross-sectional images of the retina and may show:

Cavities or spaces within the retina
Retinal thinning
Disruption of photoreceptor layers

Fluorescein Angiography

This test uses fluorescent dye injected into a vein in the arm to identify abnormal retinal blood vessels and leakage.

OCT Angiography (OCTA)

OCTA provides detailed imaging of retinal blood vessels without dye injection and can help detect abnormal vascular changes associated with MacTel.

Fundus Photography

Retinal photographs help document progression over time.

Stages and Progression

MacTel often progresses slowly over years.

Some patients maintain useful reading and driving vision for a long time, while others develop increasing central vision impairment.

In advanced cases, complications may include:

Scar tissue formation
Retinal atrophy
Abnormal blood vessel growth beneath the retina (neovascularization)

Treatment for MacTel

Treatment depends on the stage of disease and whether abnormal blood vessel growth is present.

Observation and Monitoring

Many patients are monitored closely with:

Regular retinal examinations
OCT imaging
Vision testing

Because progression is usually slow, careful long-term follow-up is important.

Anti-VEGF Injections

If abnormal blood vessel growth develops beneath the retina, anti-VEGF injections may help reduce leakage and preserve vision.

Encelto™ Therapy

A major recent advancement in the treatment of MacTel is Encelto™ (revakinagene taroretcel), an implantable encapsulated cell therapy designed to slow retinal degeneration associated with MacTel Type 2.

Encelto works by continuously delivering protective growth factors to the retina over time. The goal of treatment is to help preserve retinal cells and slow progressive vision loss.

Patients with MacTel may benefit from evaluation to determine whether they are candidates for this therapy.

Our practice is proud to be one of the only centers in the region currently offering Encelto therapy for eligible patients with MacTel Type 2.

Because this is a highly specialized treatment, patients are carefully evaluated using advanced retinal imaging and visual testing before treatment recommendations are made.

What Is the Prognosis?

The outlook for MacTel varies from person to person.

Many patients:

Maintain functional vision for years
Experience gradual progression
Continue normal activities with visual aids or reading support

However, some patients develop more significant central vision loss over time.

Early diagnosis and ongoing monitoring are important because newer therapies may help slow progression.

Living With MacTel

Patients with MacTel may benefit from:

Regular retina specialist follow-up
Monitoring changes with an Amsler grid
Good lighting for reading
Magnification or low vision aids
Prompt evaluation of sudden vision changes

Supportive care and monitoring can help patients maintain quality of life and independence.

Frequently Asked Questions

Is MacTel the same as macular degeneration?

No. MacTel and age-related macular degeneration are different retinal diseases, although both affect the macula and central vision.

Can MacTel cause blindness?

MacTel usually progresses slowly and primarily affects central vision. Total blindness is uncommon because peripheral vision is generally preserved.

What is Encelto therapy?

Encelto is a specialized retinal implant therapy designed to help slow retinal degeneration in MacTel Type 2 by delivering protective growth factors to the retina over time.

More Information

For additional patient information, visit the American Society of Retina Specialists Macular Telangiectasia Page