Looking beyond visual acuity in macular telangiectasia

Is visual acuity enough to follow macular telangiectasia (MacTel), and what should clinicians do when a patient’s symptoms say otherwise? Those questions came up in a recent Modern Retina Clinical Forum held during the American Academy of Ophthalmology 2026 Annual Meeting in New Orleans, Louisiana, “Advancing Clinical Decision-Making in Macular Telangiectasia (MacTel).”

Roger A. Goldberg, MD, MBA, a vitreoretinal surgeon and partner at Bay Area Retina Associates in Walnut Creek, California, moderated the discussion, which centered on 2 cases. In an interview with Modern Retina after the discussion, Goldberg shared what he felt attendees took away from each case.

The evidence behind encapsulated cell therapy

Goldberg presented MacTel type 2 as a progressive neurodegenerative disease driven by Müller cell dysfunction rather than a primarily vascular process, with a reported prevalence below 0.1%.¹ He also reviewed revakinagene taroretcel-lwey (Encelto; Neurotech Pharmaceuticals), an encapsulated cell therapy that uses engineered retinal pigment epithelial cells to secrete ciliary neurotrophic factor and is approved by the FDA for MacTel type 2.

Approval was based on 2 identically designed, randomized, sham-controlled phase 3 trials (NCT03316300; NCT03319849) that included 228 patients in total.² The primary end point was the rate of ellipsoid zone area loss over 24 months. His slides reported a relative reduction in retinal degeneration of 30.6% to 54.8%, with no statistically significant difference in retinal sensitivity or reading speed.²

Tracking progression in a treated and untreated eye

The first case involved a woman aged 53 years whose left eye was enrolled in one of the phase 3 trials and randomized to receive the implant. The right eye also had eligible disease and was untreated, and both eyes had visual acuity of 20/30. Comparing baseline imaging with follow-up imaging, Goldberg said, showed marked progression of photoreceptor loss in the untreated eye and stabilization of disease in the treated eye.

Although MacTel is often thought of as slowly progressive, Goldberg said the case offered “a nice internal control between two eyes in the same head,” since genetic and systemic risk factors are identical. He said that allowed the case to highlight the efficacy of treatment in a controlled way.

When visual acuity stays stable but symptoms do not

The second case involved a woman aged 59 years with progressive vision loss in the right eye. She was screened for a study of the implant in April 2019 but did not qualify because hyperreflectivity from retinal pigment epithelium migration limited the reading center’s ability to measure ellipsoid zone loss. Her disease was bilateral and more advanced in the right eye, with significant breaks in the ellipsoid zone and loss of photoreceptors in both, and visual acuity was 20/80 in the right eye and 20/20 in the left.

She returned 2 years later with unchanged acuity but more difficulty reading, reporting, “I need to move my head around to search for the letters and words.” Optical coherence tomography, Goldberg said, showed marked progression of ellipsoid zone loss in both eyes.

The patient was highly symptomatic despite the stable vision. Goldberg said a key takeaway for attendees was that “visual acuity is not a good marker for disease severity or for symptoms.” He noted that clinicians in a busy retina clinic are not always accustomed to stopping to listen to a patient’s symptoms.

Misdiagnosis and progression in practice

Goldberg said attendees generally agreed that MacTel, although classified as a rare disease, is “not as rare as we think.” He said it became apparent that patients are often misdiagnosed until they reach a retina specialist. He added that the disease is not as slow as imagined, with patients experiencing progression and worsening symptoms.

References

1. Kedarisetti KC, Narayanan R, Stewart MW, Reddy Gurram N, Khanani AM. Macular telangiectasia type 2: a comprehensive review. Clin Ophthalmol. 2022;16:3297-3309. doi:10.2147/OPTH.S373538

2. Chew EY, Gillies M, Jaffe GJ, et al. Cell-based ciliary neurotrophic factor therapy for macular telangiectasia type 2. NEJM Evid. 2025;4(8):EVIDoa2400481. doi:10.1056/EVIDoa2400481