Klinische Monatsblatter fur Augenheilkunde最新文献

{"title":"[Foveal Red Spot Syndrome - Diagnosis on Sight?]","authors":"Niklas Schön, Patrick K Peschke, Warda Darwisch, Clara Eileen Englisch, Annekatrin Rickmann","doi":"10.1055/a-2604-4288","DOIUrl":"10.1055/a-2604-4288","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"927-929"},"PeriodicalIF":1.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144216294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Macular Telangiectasia Type 2: Current and Emerging Diagnostic Imaging Modalities.","authors":"Chantal Dysli, Nicola Sagurski, Lorenzo Ferro Desideri, Martin S Zinkernagel","doi":"10.1055/a-2937-4106","DOIUrl":"10.1055/a-2937-4106","url":null,"abstract":"<p><strong>Abstract: </strong>Macular telangiectasia type 2 (MacTel type 2) is a progressive neurodegenerative macular disease whose diagnosis, prognostic assessment, and longitudinal monitoring rely critically on the recognition of its characteristic clinical and imaging features. This systematic review provides a comprehensive overview of the most important diagnostic findings in MacTel type 2, encompassing both biomicroscopic clinical signs and the full spectrum of multimodal imaging modalities currently employed in clinical practice-including color fundus photography, fluorescein angiography, fundus autofluorescence, multicolor imaging, spectral-domain optical coherence tomography (OCT), and OCT angiography. For each modality, key diagnostic signs, their contribution to disease staging, and their value in monitoring disease progression are discussed. Additionally, a specific focus on emerging imaging techniques is provided with potential relevance to future diagnostic and research applications. In MacTel type 2, a multimodal imaging approach represents the most effective strategy for accurate diagnosis, reliable staging of disease severity, and meaningful prognostic assessment.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"901-910"},"PeriodicalIF":1.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical Management of Residual Infantile Esotropia Using Lyophilized Bovine Pericardium - A Case Series of 29 Patients.","authors":"Mohamed Elabbasy, Sebastian Pfeiffer, Maren Horn, Sabine Naxer, Michael P Schittkowski","doi":"10.1055/a-2670-1917","DOIUrl":"10.1055/a-2670-1917","url":null,"abstract":"<p><strong>Abstract: </strong>PURPOSE: This study aimed to assess the effectiveness of tendon elongation using Tutopatch as a secondary intervention for residual infantile esotropia, particularly in patients exhibiting limited abduction, for whom further conventional surgery might not yield successful outcomes.</p><p><strong>Abstract: </strong>METHODS: Design: This retrospective study analysed data from patients with residual deviation angles following previous surgery for infantile esotropia. Participants and intervention: All patients who underwent tendon elongation with Tutopatch between 2009 and 2023 were included in the study, excluding those with other ocular or strabismus diagnoses or those who refused to participate. Setting: The data were collected from patients operated at a single tertiary medical centre. The analysis included pre- and postoperative deviation angles recorded at one day, three months, and twelve months, if available. The dose-effect relationship, defined as the deviation angle in degrees per effective operative distance in millimetres, and overall success rates were determined. Primary and secondary outcome measures: The primary outcome measure was the effect of using Tutopatch on the deviation angle. The secondary measure was the effect on ocular motility.</p><p><strong>Abstract: </strong>RESULTS: Participants and intervention: The study included twenty-nine patients (ten females, 19 males) with a median age of 17 years (range: 5 - 59 years). The median preoperative deviation angle was + 15° (range: + 6° to + 27°), which decreased to - 1.5° (range: - 12° to + 7°) at the three-month follow-up in 22 patients. Subsequent long-term follow-up in ten patients, after a median duration of 34.5 months (range: 13 to 69 months), revealed a median deviation angle of - 1° (range: - 15° to + 15°). Postoperative adduction was slightly reduced, by a median of 5° (range: loss of 30° to gain of 15°). No postoperative complications were observed. Overcorrection necessitated re-operation in five patients, which was uneventful. The median postoperative deviation angle following re-operation was - 1° (range: - 5° to + 8°).</p><p><strong>Abstract: </strong>CONCLUSION: Tendon elongation with Tutopatch is effective in managing significant residual infantile esotropia, particularly in complex cases, with minimal impact on duction limitation. Long-term overcorrection may occur, necessitating re-operation that can be performed without difficulty.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"937-943"},"PeriodicalIF":1.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144804356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"PAX6 and PAX6(5a) Transcript Levels in Conjunctival and Corneal Epithelial Samples and in Epithelial Cell Models of Congenital Aniridia.","authors":"Swarnali Kundu, Fabian Norbert Fries, Dalya Yapar, Lorenz Latta, Zhen Li, Nagy Zsolt Zoltán, Berthold Seitz, Thomas Tschernig, Nóra Szentmáry, Tanja Stachon","doi":"10.1055/a-2800-7868","DOIUrl":"10.1055/a-2800-7868","url":null,"abstract":"<p><strong>Abstract: </strong>PURPOSE: PAX6 haploinsufficiency plays a crucial role in the development of aniridia-associated keratopathy (AAK). Our purpose was to analyse PAX6 levels and the presence of different PAX6 isoforms in conjunctival and corneal epithelial samples and in epithelial cell models of congenital aniridia.</p><p><strong>Abstract: </strong>METHODS: PAX6 and PAX6(5a) mRNA levels were analysed in the following control and aniridia-derived samples: (a) primary limbal epithelial cell cultures from healthy donors (Ctrl-LECs) and aniridia patients (AN-LECs); (b) LECs transfected with control (siRNA Ctrl-LECs) or PAX6-targeting siRNA (siRNA PAX6-LECs); (c) immortalised limbal epithelial cells (t-LSCs) and genome-edited t-LSCs PAX6±; (d) conjunctival impression cytology from healthy individuals (Ctrl-CjECs-IC), aniridia patients (AN-CjECs-IC), and post-mortem donors (Pm-CjECs-IC); (e) corneal impression cytology from healthy living (Ctrl-CECs-IC) and post-mortem donors (Pm-CECs-IC); and (f) corneal epithelium (Ctrl-CECs) from healthy subjects. mRNA was extracted and reverse-transcribed to cDNA. PAX6 and PAX6(5a) were quantified via TaqMan qPCR with dual-labelled probes.</p><p><strong>Abstract: </strong>RESULTS: PAX6 mRNA was significantly reduced in AN-LECs, siRNA PAX6-LECs, t-LSCs, and t-LSCs PAX6± versus Ctrl-LECs (p = 0.022, 0.019, 0.006). In contrast, expression was significantly higher in Ctrl-CjECs-IC and Ctrl-CECs versus Ctrl-LECs (p < 0.001). Total PAX6 was significantly lower in AN-LECs, siRNA PAX6-LECs, t-LSCs PAX6±, and AN-CjECs-IC compared to their respective controls (p ≤ 0.039). PAX6(5a) levels were significantly lower than total PAX6 in certain examined groups (p ≤ 0.035).</p><p><strong>Abstract: </strong>CONCLUSIONS: PAX6 mRNA levels vary significantly across different epithelial cell types and models of congenital aniridia, with reduced expression observed in aniridia-derived samples and PAX6-deficient cell lines. The alternative isoform, PAX6(5a), is expressed at markedly lower levels than full-length PAX6 in most analysed tissues. This highlights the importance of both total PAX6 and PAX6(5a) expression in ocular surface epithelial homeostasis and provide further insight into the molecular alterations underlying AAK.</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146086303","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Type 2 Macular Telangiectasia: Pathogenesis-The Interplay Between Neurodegeneration and Metabolic Changes].","authors":"Malte Jung, Felicitas Bucher","doi":"10.1055/a-2936-0118","DOIUrl":"10.1055/a-2936-0118","url":null,"abstract":"<p><strong>Abstract: </strong>Macular telangiectasia type 2 (MacTel type 2) is a rare, chronic neurodegenerative macular disease that typically occurs bilaterally in the second half of life. Patients report paracentral scotomas and reading difficulties early in the disease course. Over time, limitations in daily life progressively increase.</p><p><strong>Abstract: </strong>Although the macular vascular changes give the condition its name, MacTel type 2 is now considered a primarily neurodegenerative disease with secondary vascular changes. In a small number of patients (<5%), the disease can be causally attributed to mutations in serine metabolism. Genetic defects in serine biosynthesis should in many cases be understood more as a type of risk factor, without these genomic alterations necessarily being present in every affected individual. Furthermore, the prevalence of type 2 diabetes mellitus is significantly higher in MacTel patients than in the healthy general population.</p><p><strong>Abstract: </strong>Pathomechanistically, reduced serine levels lead to aberrant sphingolipid synthesis with the accumulation of neurotoxic deoxysphingolipids. This leads to degeneration of Müller cells, the retinal pigment epithelium, and photoreceptors, culminating in a breakdown of the blood-retinal barrier with secondary vascular changes. Neurodegeneration remains confined to a circumscribed area around the fovea, the MacTel zone.</p><p><strong>Abstract: </strong>Therapeutically, the only established treatment in Europe at present is for secondary choroidal neovascularization, using intravitreal anti-VEGF therapy. A neuroprotective therapeutic approach, in which a novel cell-based depot system continuously releases ciliary neurotrophic factor, thereby slowing down photoreceptor degeneration, is currently approved only in the U.S. Complementary strategies to slow the underlying disease progression are urgently needed. In animal models, oral serine supplementation has been shown to slow neurodegeneration. Phase 2 and 3 clinical trials in humans are already underway, although they have not yet fully completed (ClinicalTrials.gov: NCT04907084).</p>","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":"894-900"},"PeriodicalIF":1.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148840566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Multimodal imaging correlation in bilateral diffuse uveal melanocytic proliferation (BDUMP)].","authors":"Sebastian Mohr, Horst Helbig, Teresa Barth","doi":"10.1055/a-2932-2956","DOIUrl":"https://doi.org/10.1055/a-2932-2956","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148818765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Sekundäre korneale Amyloidose bei Keratokonus: klinische und histopathologische Erkenntnisse einer Fallserie von zwei Patienten].","authors":"Charlott Fuß, Berthold Seitz, Elias Flockerzi, Andrea Hasenfus, Fidelis Flockerzi, Tim Berger","doi":"10.1055/a-2937-4405","DOIUrl":"10.1055/a-2937-4405","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148723360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe Exacerbation of Granular Corneal Dystrophy Type 1 With Interface Opacification After Superficial Anterior Lamellar Keratoplasty (SALK).","authors":"Stefan Weber, Berthold Seitz, Elias Flockerzi, Fidelis Flockerzi, Yoo-Jin Kim, Loay Daas, Tim Berger","doi":"10.1055/a-2932-4681","DOIUrl":"10.1055/a-2932-4681","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148689605","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Herpes Encephalitis-Associated Hemorrhagic-Occlusive Retinal Vasculitis: A Case Report.","authors":"Peter Wolfrum, Marc Brockmann, Norbert Pfeiffer, Alexander K Schuster","doi":"10.1055/a-2932-6122","DOIUrl":"https://doi.org/10.1055/a-2932-6122","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148794935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Familial Exudative Vitreoretinopathy (FEVR) Associated with a CTNNB1 Mutation].","authors":"Melanie Michelczak, Marian Liegl, Sabine Aisenbrey","doi":"10.1055/a-2913-3388","DOIUrl":"10.1055/a-2913-3388","url":null,"abstract":"","PeriodicalId":17904,"journal":{"name":"Klinische Monatsblatter fur Augenheilkunde","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148422127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}