Klinische Monatsblatter fur Augenheilkunde · 2026
[Macular Telangiectasia Type 2 (MacTel) - Therapeutic Options and New Developments].
СтатьяОбзорАннотация на английскомНазвание и аннотация: Перевод в очереди
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Harten Julia, Raming Kristin, Goerdt Lukas, Holz Frank, Pfau Kristina
Аннотация
ABSTRACT: Macular telangiectasia type 2 (MacTel) is a rare, bilateral neurodegenerative disease of the macula associated with secondary vascular alterations. Pathophysiologically, Müller cell dysfunction plays a central role. Their loss leads to progressive degeneration of central cone photoreceptors and may secondarily contribute to the development of macular neovascularization (MNV), which affects approximately 10% of patients.
ABSTRACT: A major pathophysiologic mechanism is a disturbance in serine and sphingolipid metabolism. Patients with MacTel have significantly lower blood serine levels, leading to increased synthesis of neurotoxic deoxysphingolipids, which are elevated by approximately 84% in affected individuals compared with healthy controls. These toxic lipids damage Müller cells, photoreceptors, and retinal ganglion cells. Oral serine supplementation is discussed as a promising causal therapeutic approach. Initial animal studies have shown reversible retinal changes under serine therapy; a phase 3 study of supplementation in patients with MacTel is currently in preparation.
ABSTRACT: Clinically, optical coherence tomography (OCT) shows hyporeflective cavities and loss of the ellipsoid zone (EZ) as markers of photoreceptor atrophy, while fundus autofluorescence demonstrates loss of macular pigment. Fluorescein angiography reveals telangiectatic capillaries temporal to the fovea in the early phase and diffuse leakage in the late phase. Symptomatically, patients primarily report reading difficulties, metamorphopsia, and paracentral scotomas; marked impairment of central visual acuity usually occurs only in advanced disease stages.
ABSTRACT: Therapeutically, intravitreal anti-VEGF therapy is considered the standard treatment for exudative MNV but has no benefit in nonproliferative MacTel and may even adversely affect the disease course. A milestone is the Encelto implant (revakinagene taroretcel-lwey), approved by the FDA in March 2025. The implant contains allogeneic RPE cells that continuously produce and secrete ciliary neurotrophic factor (CNTF) and is considered the first disease-modifying therapy for MacTel. In two parallel phase 3 studies (NTMT-03-A/B), the implant reduced EZ loss by approximately 55% and 31%, respectively, compared with sham treatment. European approval by the EMA is still pending. This review systematically presents current and future therapeutic options for macular telangiectasia type 2.
ABSTRACT: A major pathophysiologic mechanism is a disturbance in serine and sphingolipid metabolism. Patients with MacTel have significantly lower blood serine levels, leading to increased synthesis of neurotoxic deoxysphingolipids, which are elevated by approximately 84% in affected individuals compared with healthy controls. These toxic lipids damage Müller cells, photoreceptors, and retinal ganglion cells. Oral serine supplementation is discussed as a promising causal therapeutic approach. Initial animal studies have shown reversible retinal changes under serine therapy; a phase 3 study of supplementation in patients with MacTel is currently in preparation.
ABSTRACT: Clinically, optical coherence tomography (OCT) shows hyporeflective cavities and loss of the ellipsoid zone (EZ) as markers of photoreceptor atrophy, while fundus autofluorescence demonstrates loss of macular pigment. Fluorescein angiography reveals telangiectatic capillaries temporal to the fovea in the early phase and diffuse leakage in the late phase. Symptomatically, patients primarily report reading difficulties, metamorphopsia, and paracentral scotomas; marked impairment of central visual acuity usually occurs only in advanced disease stages.
ABSTRACT: Therapeutically, intravitreal anti-VEGF therapy is considered the standard treatment for exudative MNV but has no benefit in nonproliferative MacTel and may even adversely affect the disease course. A milestone is the Encelto implant (revakinagene taroretcel-lwey), approved by the FDA in March 2025. The implant contains allogeneic RPE cells that continuously produce and secrete ciliary neurotrophic factor (CNTF) and is considered the first disease-modifying therapy for MacTel. In two parallel phase 3 studies (NTMT-03-A/B), the implant reduced EZ loss by approximately 55% and 31%, respectively, compared with sham treatment. European approval by the EMA is still pending. This review systematically presents current and future therapeutic options for macular telangiectasia type 2.