Philip Maier

This retrospective multicenter cohort study investigated the incidence, treatment, and prognostic impact of dysregulated intraocular pressure (DIOP; >24 mmHg requiring therapy) in patients with acanthamoeba keratitis (AK). Eighty-one eyes from 81 patients with confirmed AK were included; co-infections were excluded. DIOP and best corrected visual acuity (BCVA) were assessed at baseline, follow-up, and after penetrating keratoplasty (PKP). Overall, 28% of eyes were managed conservatively, 46% required one PKP, and 26% required two. DIOP occurred in 4% before AK, 11% after AK onset, and 10% after the first PKP. Eyes with DIOP prior to PKP had significantly worse final BCVA compared to those without DIOP (0.5 ± 0.7 vs. 1.8 ± 1.1 logMAR, p < 0.001) and required PKP more frequently (100% vs. 67%, p = 0.01). Time to correct diagnosis was significantly longer in the AK-associated DIOP group (64 ± 50 vs. 50 ± 68 days, p = 0.03). DIOP was also associated with higher rates of prior cortisone use and complications, including fixed dilated pupil, cataract, anterior synechiae, and anterior chamber irritation. Overall, DIOP represents a major complication in AK and is linked to poorer visual outcomes. Earlier therapy may help reduce DIOP and hence improve prognosis.

Background: Corneal dystrophies are inherited disorders that can lead to significant visual impairment and often require surgical intervention in advanced stages. Fuchs endothelial corneal dystrophy (FECD) is the most frequently diagnosed type in Western countries and remains a leading global indication for corneal transplantation. In contrast, non-Fuchs dystrophies represent a diverse group of less common entities, each with distinct clinical features, surgical considerations, and regional variations in incidence and management. Despite their relevance, long-term data on the full spectrum of corneal dystrophies remain scarce. This study aimed to evaluate the distribution and temporal trends in dystrophy types and associated surgical procedures over eight decades at a tertiary referral center in Germany. Methods: This retrospective analysis included 3 827 histopathologically confirmed corneal dystrophy specimens identified from an archive of 58 150 ophthalmic specimens collected between 1945 and 2024. Extracted data included dystrophy type, patient age at surgery, sex assigned at birth and associated surgical procedures. Distribution and temporal trends were analyzed descriptively. Results: FECD accounted for 90.3% (n = 3 455) of all cases, with a more than 15-fold increase in annual cases between 2003 and 2024. Its surgical management transitioned from exclusive use of penetrating keratoplasty (PKP) to posterior lamellar keratoplasty in over 99% of cases by 2024. Among non-Fuchs dystrophies (n = 372), granular (21.2%), macular (17.5%), and lattice dystrophy (17.2%) were most frequent. These exhibited greater surgical variability, reflecting their heterogeneity across 21 non-Fuchs dystrophy types in this study. Stromal and epithelial-stromal dystrophies were predominantly managed with PKP, whereas superficial epithelial and basement membrane dystrophies were increasingly treated with phototherapeutic or manual superficial keratectomy. Limbo-keratoplasty was introduced in the early 2000s for recurrent subepithelial and epithelial-stromal types. Conclusion: This study provides unique insights into the type distribution and surgical management of corneal dystrophies over eight decades in a German center, encompassing nearly all IC3D-classified entities. The marked increase in FECD specimen numbers and the shift toward lamellar keratoplasty reflect evolving clinical practices and rising demand on corneal transplantation services. The broader clinical spectrum and procedural diversity among non-Fuchs dystrophies underscore the ongoing need for pathology-specific management strategies tailored to population-specific needs.

Der Schwerpunkt der diesjährigen Sonderausgabe zu „Sklera, Konjunktiva und Kornea“ liegt in sehr seltenen Erkrankungen der Augenoberfläche bzw. (noch) selteneren Infektionen der Hornhaut. So wird die Inzidenz der Akanthamöbenkeratitis auf maximal 3 Betroffene pro Million pro Jahr geschätzt. Unter den infektiösen Keratitisfällen haben die mykotischen Infektionen nach Schätzungen je nach geografischer Lage einen Anteil von 1 und 44%, wobei aufgrund der weltweiten Erwärmung die Inzidenz auch in unseren Regionen zu steigen scheint. Die Inzidenz eines Stevens-Johnson-Syndroms bzw. einer toxisch epidermalen Nekrolyse wird auf 2 – 9 Betroffene pro Million pro Jahr geschätzt, wobei bei bis zu 90% auch die Augen von der fulminanten Autoimmunreaktion betroffen sind. Das okuläre Pemphigoid schließlich tritt bei etwa 2 Menschen pro Million pro Jahr auf. Trotz Ihrer Seltenheit haben die genannten Erkrankungen in der Ophthalmologie einen wichtigen Stellenwert, da den Betroffenen stets bei zu später, inadäquater oder insuffizienter Therapie die Erblindung droht. Aufgrund der Seltenheit der verschiedenen Erkrankungen gibt es in Deutschland für diese Entitäten spezielle Register, in denen die Verläufe der Betroffenen dokumentiert werden können, um so über die Auswertung eines größeren Kollektivs die Diagnostik und Therapie langfristig verbessern zu können. Article published online: 16 June 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

Epidermal necrolysis (EN) is a very rare, but life-threatening dermatological disease where the skin and various mucous membranes react to drugs (75%) or systemic infections (25%). If up to 10% of the body surface is affected, the disease is called Stevens Johnson syndrome (SJS); if more than 30% of the body surface is affected, it is called toxic epidermal necrolysis (TEN); if 10 – 30% of the body surface is affected, it is called SJS/TEN overlap. In the acute phase of the disease, 80% of cases show ophthalmic involvement (from mild conjunctivitis to total de-epithelialisation of the ocular surface). Therefore, patients should be seen by an ophthalmologist within 24 h after symptoms have started. Treatment of the acute ophthalmic problems has a very important impact on long-term eye complications. However, in the long run up to 50% of patients develop chronic sight threatening ocular surface disease which needs further intensive topical and surgical treatment (e.g. lubrification, topical anti-inflammatory agents, lid surgery, scleral lenses, corneal surgery). The indication for corneal transplantation (allogenic penetrating limbo-keratoplasty and keratoprostheses) should be given with due caution, as the prognosis is very poor.

Background: Suture fixation of intraocular lenses (IOLs) is a rare but essential procedure for patients with inadequate capsular support, offering crucial therapeutic benefits. This study analyzes a large cohort of patients undergoing IOL suture fixation, focusing on demographics, risk factors, and clinical outcomes. Methods: In this retrospective analysis of 332 eyes treated at the Eye Center of the University Hospital Freiburg (2008–2022), we evaluated immediate and long-term postoperative outcomes, including visual acuity and refraction. The follow-up averaged 2.5 years. A detailed analysis of the IOL positioning was performed in 111 patients. Results: The most common indications were pseudoexfoliation syndrome (33%), trauma (17%), and prior complicated cataract surgery (11%). The Zeiss CT27SF lens was used most frequently (91%). The mean spherical equivalent deviation from target refraction was −0.375 diopters overall. According to the detailed analysis, IOL tilt occurred in 6.3% of patients, and decentration occurred in 7.2%. Only three patients (3.6%) required additional ocular procedures specifically for IOL repositioning. Risk factors for further surgery included underlying systemic conditions or multiple previous surgeries. Conclusions: Suture fixation of IOLs proves to be an adequate and effective intervention for visual rehabilitation in patients with compromised capsular support, demonstrating good refractive outcomes and low complication rates. Even in cases where the postoperative visual acuity was comparable to the best-corrected preoperative acuity, the procedure improved refractive correction by reducing the need for extensive refractive aids, such as contact lenses required for aphakia.

Background: Anterior chamber–associated immune deviation (ACAID) is an active immunotolerance mechanism, which is induced by placing antigen into the anterior eye chamber as long as a major surgical trauma is avoided. For this reason, ACAID may be a major contributor to the favorable immunologic outcomes in Descemet membrane endothelial keratoplasty (DMEK). Rodent models have demonstrated the importance of a functional spleen for the development of an ACAID. Objective: This study aimed to investigate whether splenectomy leads to increased rejection rates after DMEK in humans. Methods: A retrospective evaluation was conducted on the course following DMEK at the Eye Center, Medical Center, University of Freiburg, for patients with a self-reported history of splenectomy compared to patients without this condition. Potential study patients were contacted by mail. A questionnaire to self-report splenectomy and the time thereof was sent out. The medical records of all consenting patients at the Eye Center were reviewed for graft survival and immune reactions. Results: We asked 1818 patients after DMEK to report their history of splenectomy. A total of 1340 patients responded and were included in the study. Of these 1340 patients, 16 (1.2%) reported a history of splenectomy (ie, 26 DMEKs, with 10 patients being transplanted in both eyes and 6 patients being transplanted in 1 eye; median age at surgery 73.7, range 66.7-76.1 y). The remaining patients (1324 patients, ie, 1941 eyes) served as controls, with 1941 DMEKs (median age at surgery 71.5, range 64.1-77.2 y). Five (19%) out of the 26 eyes from the splenectomy group required a second transplant due to dislocation (n=2.8%), failure (n=2.8%), and rejection (n=1.4%). Kaplan-Meier analysis revealed no relevant difference compared with controls. Conclusions: Our results suggest that splenectomy has no major effect on the outcome following DMEK. Subsequent, ACAID may not be the main reason for the favorable immunological outcomes in DMEK, or the camero-splenic axis may be subordinate in humans. However, we only included 16 patients who underwent splenectomy, so it might be possible that we missed a minor effect.

The microbiome of the ocular surface has been characterised, but only limited information is available on a possible silent intraocular microbial colonisation in normal eyes. Therefore, we performed next-generation sequencing (NGS) of 16S rDNA genes in the aqueous humour. The aqueous humour was sampled from three patients during cataract surgery. Air swabs, conjunctival swabs from patients as well as from healthy donors served as controls. Following DNA extraction, the V3 and V4 hypervariable regions of the 16S rDNA gene were amplified and sequenced followed by denoising. The resulting Amplicon Sequence Variants were matched to a subset of the Ribosomal Database Project 16S database. The deduced bacterial community was then statistically analysed. The DNA content in all samples was low (0–1.49 ng/µL) but sufficient for analysis. The main phyla in the samples were Acinetobacteria (48%), Proteobacteria (26%), Firmicutes (14%), Acidobacteria (8%), and Bacteroidetes (2%). Patients’ conjunctival control samples and anterior chamber fluid showed similar patterns of bacterial species containing many waterborne species. Non-disinfected samples showed a different bacterial spectrum than the air swab samples. The data confirm the existence of an ocular surface microbiome. Meanwhile, a distinct intraocular microbiome was not discernible from the background, suggesting the absence of an intraocular microbiome in normal eyes.