Abstrakt Makuläre Teleangiektasien Typ 2 (MacTel) ist eine seltene, bilateral auftretende neurodegenerative Erkrankung der Makula, die mit sekundären vaskulären Veränderungen einhergeht. Pathophysiologisch steht die Dysfunktion der Müllerzellen im Vordergrund. Ihr Verlust führt zur progressiven Degeneration zentraler Zapfen-Photorezeptoren und kann sekundär zur Entstehung makulärer Neovaskularisationen (MNV) beitragen, die etwa 10% der Patienten betreffen. Ein wesentlicher pathophysiologischer Mechanismus ist eine Störung im Serin- und Sphingolipidstoffwechsel. MacTel-Patienten weisen signifikant niedrigere Serinspiegel im Blut auf, was zur vermehrten Synthese neurotoxischer Deoxysphingolipide führt – bei Betroffenen um ca. 84% erhöht gegenüber Gesunden. Diese toxischen Lipide schädigen Müllerzellen, Photorezeptoren und retinale Ganglienzellen. Eine orale Serin-Supplementierung wird als vielversprechender kausaler Therapieansatz diskutiert. Erste Tierstudien zeigen reversible Netzhautveränderungen unter Serintherapie; eine Phase-3-Studie zur Supplementierung bei MacTel-Patienten ist in Vorbereitung. Klinisch imponieren in der OCT hyporeflektive Hohlräume und ein Verlust der Ellipsoidzone (EZ) als Marker der Photorezeptoratrophie sowie ein Verlust von Makulapigment in der Fundusautofluoreszenz. Die Fluoreszeinangiographie zeigt telangiektatische Kapillare temporal der Fovea in der Frühphase und diffuse Leckagen in der Spätphase. Symptomatisch klagen Patienten vor allem über Leseschwierigkeiten, Metamorphopsien und parazentrale Skotome; eine ausgeprägte Einschränkung des zentralen Sehvermögens tritt erst in späten Krankheitsstadien auf. Therapeutisch gilt die intravitreale Anti-VEGF-Therapie als Standardbehandlung bei exsudativer MNV, hat jedoch bei nicht-proliferativer MacTel keinen Nutzen und kann die Erkrankung sogar ungünstig beeinflussen. Einen Meilenstein stellt die im März 2025 durch die FDA zugelassene Therapie mit dem Encelto-Implantat (revakinagene taroretcel-lwey) dar. Das Implantat enthält allogene RPE-Zellen, die kontinuierlich den Ciliary Neurotrophic Factor (CNTF) produzieren und sezernieren, und gilt als erste krankheitsmodifizierende Therapie für MacTel. In zwei parallelen Phase-3-Studien (NTMT-03-A/B) reduzierte das Implantat den EZ-Verlust um ca. 55% bzw. 31% gegenüber Sham. Die europäische Zulassung durch die EMA steht noch aus. In diesem Übersichtsartikel werden aktuelle und zukünftige Therapiemöglichkeiten der Makulären Teleangiektasien Typ 2 systematisch dargestellt.
Abstract:BACKGROUND: Presbyopia-correcting intraocular lenses (IOLs) provide a complete spectrum of vision but frequently induce dysphotopsia due to multiple focal points and diffractive designs. Standard straylight assessments using a C-Quant meter at a 7° visual angle often show no significant differences between evaluated IOLs. Therefore, evaluating glare effects effectively requires device modifications to demonstrate differences across lower visual angles. This study evaluates glare from the diffractive designs of two presbyopia-correcting IOLs. Abstract:MATERIAL AND METHODS: This in vitro laboratory study assessed two IOL models: the Clareon PanOptix (Alcon) and the Tecnis Odyssey (J&J Vision). Straylight was measured using a modified C-Quant straylight meter, allowing evaluation at the standard 7° angle as well as lower angles of 2.5° and 3.5°. Abstract:RESULTS: At 7°, the Clareon PanOptix exhibited straylight of 0.29 ± 0.19 deg2/sr, while the Tecnis Odyssey showed an 8-fold higher value of 2.29 ± 0.60 deg2/sr. At 3.5°, the Clareon PanOptix measured 3.01 ± 0.88 deg2/sr, whereas the Tecnis Odyssey demonstrated more than twice the magnitude at 6.85 ± 0.68 deg2/sr. At the smallest angle of 2.5°, the Clareon PanOptix recorded 4.57 ± 0.12 deg2/sr. In contrast, the Tecnis Odyssey reached 9.34 ± 0.41 deg2/sr, approaching the reference value of a 65-year-old natural crystalline lens. Abstract:CONCLUSION: The Clareon PanOptix demonstrated substantially lower straylight values across all measured angles compared with the Tecnis Odyssey. This suggests a reduced potential for glare phenomena. Further research using validated patient-reported questionnaires is needed to correlate these objective findings with subjective visual disturbances. Abstract:QUINTESSENCE: in vitro straylight testing shows that the Clareon PanOptix IOL exhibits lower straylight levels than the Tecnis Odyssey across all measured angles. While clinical confirmation is needed, these findings suggest that the Clareon PanOptix may be associated with a lower risk of dysphotopsia related to its diffractive design.
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:INTRODUCTION: Cystoid macular edema (CME) is a recognized complication associated with penetrating keratoplasty (PK) and Endothelial Keratoplasty (EK). The purpose of this study was to determine the incidence of acute CME within the first 6 months after PK, Descemet stripping automated EK, or Descemet's membrane EK. Abstract:METHODS: A retrospective cohort analysis of the TriNetX United States collaborative database was conducted. Patients with a diagnosis of Fuchs dystrophy or keratoconus who underwent PK or EK between January 1, 2004 and August 30, 2024 were included. Those with a history of uveitis, retinal vascular occlusion, diabetic macular edema, or prior vitrectomy were excluded. Outcomes and diagnoses were defined using current procedural terminology and International Classification of Diseases, Tenth edition (ICD-10) codes. Cohorts were stratified based on a history of prior PK or EK with >6 months follow-up. Propensity score matching was performed on both cohorts to control for age at index, sex, race, ethnicity, and the diagnosis of Fuchs dystrophy and keratoconus. The incidence of acute CME during a 6-month postoperative follow-up period was assessed. Odds ratios were calculated between cohorts. Abstract:RESULTS: A total of 2516 eyes were included in the study (PK: 1258; EK: 1258). At six months postoperatively, the incidence of acute CME in the EK cohort (46 of 1258) was significantly higher than in the PK cohort (20 of 1258) (P = 0.0012) (OR 0.426, 95% confidence interval 0.25-0.72). Abstract:CONCLUSIONS: Our findings suggest that the incidence of acute CME is higher in patients undergoing EK compared to PK.
Abstract:BACKGROUND: Childhood visual impairment is a significant public health challenge, with prevalence rates in sub-Saharan Africa exceeding those in high-income countries. While surgical intervention is the mainstay of treatment for many blinding paediatric conditions, access to these services is constrained in low-resource settings. We report a comprehensive analysis of demographic and geographic patterns of paediatric ophthalmic surgical services at a tertiary eye centre in Malawi. Abstract:METHODS: A retrospective analysis of paediatric patients who underwent ophthalmic surgeries at Lions Sight First Eye Unit, Queen Elizabeth Central Hospital (QECH) in Southern Malawi, between January 2015 and December 2022 was undertaken. Demographic variables included age group, sex and distance travelled to reach the facility. Descriptive statistics summarised trends, and a chi-square test assessed the association between sex and distance travelled. Abstract:RESULTS: Of 3,807 identified patient files, 3,263 (85.7%) were eligible for analysis. A male predominance was observed (61.0% male vs 39.0% female), with a mean age of 5.7 (±4.4) years. The majority of children (n = 2783, 85.3%) were from the Southern region. More than half of the patients (52.6%, n = 1717) travelled over 50 km to reach QECH. A statistically significant association was found between sex and distance travelled (χ2 = 14.46, p = 0.013). Annual surgical volumes plateaued during the study period. Abstract:CONCLUSION: Paediatric ophthalmic surgical services in Malawi are concentrated at a single tertiary facility, creating geographic disparities in access. The male predominance in the number of children accessing the surgical services across different paediatric eye diseases highlights the need for gender-sensitive community awareness initiatives to ensure equitable access to surgical eye care for all children. Furthermore, the surgical numbers failing to increase in line with the national population growth can be an indicator of an overwhelmed system. These findings emphasise the urgent need to establish additional specialised centres to overcome these barriers.
