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Background Congenital corneal opacities are comparatively rare diseases with high amblyogenic potential. Purpose The present work provides an overview of the diagnostics, clinical aspects and genetics of congenital corneal opacities. Methods A literature search was carried out to compile an overview and illustration with own clinical case examples. Results Differentiated diagnostics are of high importance in the treatment of patients with congenital corneal opacities. A close cooperation between the medical departments involved and also the parents is absolutely essential. The structured classification of congenital corneal opacities provides the basis for a targeted treatment. Discussion The causes and the clinical symptoms of congenital corneal opacities are manifold. The correct diagnosis should be made early and in an interdisciplinary manner. Based on this, conservative and surgical treatment measures can be planned and an impending development of amblyopia can be specifically counteracted.

Background The risk of allograft rejection following high-risk keratoplasty increases with the area of corneal neovascularization. Pharmaceutical and physical regression of corneal neovascularization before keratoplasty may offer the potential to reduce the risk of graft rejection after high-risk keratoplasty. Objective This article provides a review of the literature on the preconditioning of vascularized high-risk eyes using fine-needle diathermy and corneal cross-linking (preoperative preconditioning by lymphangioregression). Methods A literature search was carried out in PubMed and a summary of own data is presented. Results Animal experimental studies showed that both fine-needle diathermy and corneal cross-linking lead to a regression of corneal neovascularization and prolong graft survival after high-risk keratoplasty. Furthermore, studies from our institute provide first evidence that both procedures also lead to a reduction of corneal neovascularization in the clinical practice and thus potentially reduce the risk of allograft rejection after subsequent high-risk keratoplasty. Discussion Fine-needle diathermy and corneal cross-linking provide effective therapeutic approaches for angioregressive treatment and seem to prolong graft survival following high-risk keratoplasty. Larger prospective and controlled clinical trials are needed to further investigate these promising therapeutic approaches.

Background Descemet membrane endothelial keratoplasty (DMEK) is considered the gold standard for the treatment of corneal endothelial dysfunction and generally leads to good postoperative results. Recently, studies have also analyzed the outcome of DMEK in so-called high-risk eyes. Material and methods The relevant literature and own data on DMEK for graft failure after penetrating keratoplasty and in vascularized high-risk eyes are presented and discussed. Results A DMEK for the treatment of transplant failure after penetrating keratoplasty can be considered for eyes without stromal scars and without high astigmatism. A retrospective analysis of 52 patients with failed penetrating grafts showed that DMEK leads to a significant increase in visual acuity, albeit to a lesser extent than after primary DMEK. Rejection and transplant failure rates seem to be similar those seen after penetrating re-keratoplasty and are thus higher than after primary DMEK. A DMEK might also be a feasible option for eyes with corneal neovascularization and stromal edema without stromal scars. A retrospective analysis of 24 eyes with at least 2 vascularized corneal quadrants demonstrated that DMEK leads to a significant improvement in visual acuity and regression of corneal neovascularization. The rejection rate in this cohort was 4.2% and is therefore slightly higher than after low-risk DMEK in eyes without corneal neovascularization but still much better compared to penetrating keratoplasty. Conclusion Indications for DMEK are expanding and it can be a therapeutic option for transplant failure after penetrating keratoplasty with acceptable outcomes. Furthermore, DMEK seems to be a good option for the treatment of endothelial dysfunction in vascularized high-risk eyes without stromal scars.

Background In severely compromised and vascularized high-risk eyes, the Boston keratoprosthesis procedure is often the last treatment option. The transparency of the optics is not impaired by endothelial immune reactions. Methods This review article discusses relevant literature as well as our own data and experiences with the Boston keratoprosthesis in high-risk eyes. The relevant complications as well as the postoperative management are discussed. Results In more than 60% of the high-risk eyes a long-term increase in visual acuity can be achieved. Keratoprosthesis retention rates show a variable span with reported mean 5-year retention rates of 75%. The most common postoperative complications include the formation of a retroprosthetic membrane and keratolysis in up to 50% each. More than 70% of the eyes already suffer from (secondary) glaucoma preoperatively, so that glaucoma surgery is performed simultaneously in at least 20% of cases and in the postoperative course further antiglaucomatous surgery is necessary in up to 31%. Vitreoretinal complications include, in particular, sterile vitritis and infectious endophthalmitis but persistent hypotonia is also described in one third of patients. Conclusion The Boston keratoprosthesis is an alternative to conventional corneal replacement if the prognosis for allogeneic transplants is poor. Postoperative complications are common; therefore, postoperative management plays an important role. For vascularized high-risk eyes, however, it is often the only remaining option for visual rehabilitation.

BackgroundDescemet membrane endothelial keratoplasty (DMEK) has become the standard surgical treatment for Fuchs' endothelial corneal dystrophy (FECD) in Germany. In addition to classical FECD, there are endothelial disorders amenable to DMEK associated with complex changes in the anterior segment of the eye, such as anterior synechia of the iris, larger iris defects, after glaucoma surgery, after vitrectomy and after trauma.ObjectiveThis article provides an overview of the possibilities to treat patients with complex anterior segment situations in the presence of corneal endothelial decompensation with DMEK.Material and methodsDescription of problems and current surgical techniques when performing DMEK in complex situations.ResultsReconstruction of the iris or the iris/lens diaphragm often needs to be performed either simultaneously or in aprevious operation to treat patients with complex anterior segment alterations with DMEK. Both very shallow and very deep anterior chambers pose amajor problem. A DMEK graft from older donors (above 70years) is prone to less rolling and should be preferred in patients with adeep anterior chamber (e.g. after vitrectomy). The graft size should also be adapted to the available space, e.g. asmaller graft diameter should be selected for eyes with anterior synechia.ConclusionThere are only afew evaluations of DMEK under complex conditions; however, it appears that the rate of secondary graft failure, endothelial cell loss and the rebubbling rate are increased in this very heterogeneous group of diseases. Further studies are needed to identify risk factors for anegative progression after DMEK in complex eyes.

Background and objectiveDescemet membrane endothelial keratoplasty (DMEK) has increased in popularity since its introduction over 10years ago. This article presents asummary of the experiences of the past years collected at the Department of Ophthalmology at the University of Cologne.MethodsA literature review of DMEK studies primarily from the Department of Ophthalmology at the University of Cologne, Germany was carried out. Own experiences in the fields of donor selection and graft preparation, DMEK surgery, complication management and postoperative treatment are summarized.ResultsSince the introduction of DMEK experience has been gained and ongoing improvements have occurred ranging from donor-recipient allocation to postoperative follow-up. These led to abetter reproducibility of the intervention for the surgeon, to abetter postoperative result and to a reduction of the complication rate.DiscussionThe DMEK represents asafe and individualized procedure for endothelial transplantation for corneal endothelial dysfunction. Continuing development of the method leads to optimization and safer results.

BackgroundKeratoprosthetics refer to the replacement of human corneal tissue with an artificially produced exchange product. The most widely implanted keratoprosthesis worldwide is the Boston TypeI Keratoprosthesis (BI-KPro, Massachusetts Eye and Ear Infirmary, Boston, MA, USA).MethodsThis video article demonstrates the surgical technique of BI-KPro implantation as routinely performed at this institution. In addition, an overview of the surgical indications, patient consent, anesthesia and positioning, postoperative treatment and complications as well as the evidence of the technique is provided in the article based on experience with 31 BI-KPro implantations.ResultsThe indications for BI-KPro primarily include patients in ahigh-risk keratoplasty setting particularly after repeatedly failed keratoplasty. A thorough patient examination and assessment of the indications as well as detailed disclosure of information to the patient about the individual prognosis, the risk profile, the postoperative therapy and follow-up enable apositive postoperative result.ConclusionThe BI-KPro provides an established and relatively standardized option for surgical treatment of eyes in ahigh-risk keratoplasty setting. Patients should be preoperatively informed with respect to the individual prognosis, complication profile and life-long follow-up.

Background and purpose. Mycotic keratitis is a serious but relatively rare disease. No targeted data collection in Germany existed until the foundation of the German Pilz-Keratitis Register in 2015. Patients and methods. The inclusion of retrospective and prospective patients was carried out. Inclusion criteria: diagnosis confirmed by the polymerase chain reaction (PCR), culture, histology or confocal microscopy (IVCM). Collected parameters: date of symptom onset, date and method of diagnosis, risk factors, visual acuity and findings at admission and at follow-up, conservative and surgical treatment. Results. By January 2018, a total of 102 eyes from the years 2000-2017 were reported from 16 centers (64.3% female, mean age 52 years, range 18-95 years). The initial diagnosis was made correctly in only 20.6% of cases. The mean time to correct diagnosis was 31.7 +/- 46.9 (0-296) days. The diagnosis was confirmed in cultures in 74.5%, histologically in 30.4%, by PCR in 38.2% and IVCM in 27.4%. Fungal species identified were: 36.7% Fusarium spp., 35.8% Candida spp., 6.4% Aspergillus spp. and 21.1% other. The most important risk factor was the use of contact lenses. The most commonly used antifungal agent was voriconazole (64.7%) followed by amphotericin B (37.2%). Penetrating keratoplasty was performed in 65.7% of the cases and 8.8% of the affected eyes had to be enucleated. The visual acuity of the entire study population increased from the initial 0.16 +/- 0.25 (0.001-1.0) decimal to 0.28 +/- 0.34 (0-1.0) decimal. Conclusion. The correct diagnosis of fungal keratitis is often significantly delayed. The treatment can be very difficult and keratoplasty is often necessary. In order to gain a better understanding of this disease, to recognize previously unknown risk factors and, if necessary, a change in the spectrum of pathogens and to identify approaches to treatment optimization, the fungal keratitis registry will be continued.