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Background: During the last decade Descemet membrane endothelial keratoplasty (DMEK) has been established as a surgical treatment even for complex cases of bullous keratopathy., Material and Methods: In a case series 9 eyes from 8 patients with chronic corneal edema caused by multiple intraocular operations underwent DMEK surgery. It was the sole surgical procedure in three eyes. In one case DMEK was combined with an intravitreal injection of bevacizumab, with opacified intraocular lens exchange in a second case, with transscleral cyclophotocoagulation with Iridex micropulse laser (Iridex, Silicon Valley, CA, USA) and intravitreal injection of bevacizumab in a third case and with the Tutopatch® (DMR srl, Italy) coverage of a scleral perforation in a fourth case. Additionally, DMEK was combined with trimming of the Ahmed valve tube length in two cases. The follow-up period was 12 months., Results: Out of 9 eyes 2 showed a persistent partial or total detachment of the graft with concomitant recurrence of bullous keratopathy despite repeated injection of 20% sulfur hexafluoride (SF6) in the anterior chamber. These 2 eyes were treated with penetrating keratoplasty and achieved satisfactory postoperative results including pain relief. Visual acuity did not improve in these 2 eyes due to optic atrophy. Visual acuity and pain improved in the first postoperative month in 7 eyes. Donor endothelial cell density decreased from 2465±147/mm 2 to 1295 ± 254/mm 2 and 1180 ± 197/mm 2 (p < 0.001, n = 7) after 6 and 12 months, respectively. Central corneal thickness decreased from 880 ± 232 μm to 571 ± 15 μm (p = 0.001, n = 7) after 12 months. Multiple rebubblings were performed in 2 of the 9 eyes because of a persistent corneal graft detachment., Conclusion: Although DMEK is a technically demanding surgical treatment for complicated cases of severe bullous keratopathy, it provides satisfactory results in terms of the improvement of visual acuity, pain relief and a shorter postoperative period., (© 2020. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Ocular graft-versus-host disease (GvHD) following allogeneic blood stem cell transplantation leads to immunologically induced alterations in many ocular tissues, particularly at the ocular surface. Within the framework of the main topic, this article focuses primarily on corneal complications in chronic ocular GvHD., Objective: This article aims to promote understanding of the influencing factors, diagnostics, and therapeutic options pertaining to corneal complications in ocular GvHD. Furthermore, the possibilities for prevention are discussed., Materials and Methods: This analysis is based on a literature review as well as on data from the Ophthalmology Clinic at the University Hospital Essen., Results: Corneal complications often occur secondarily in ocular GvHD, as a consequence of severe inflammatory alterations of the conjunctiva or eyelid. Spontaneous corneal perforations associated with only mild symptoms are less common during the course of disease. From the ophthalmologist's perspective, it is important that the inflammatory activity of all the different ocular tissues is considered. Treatment may follow a stepwise scheme that includes substitution, immunosuppression, and surgical rehabilitation., Conclusion: Systematic diagnosis of ocular GvHD helps to prevent corneal complications or support early therapeutic intervention. An interdisciplinary approach to diagnosis and treatment planning is recommended, in order to optimize local and systemic immunosuppressive therapy.

Background: Corneal transplantation in high-risk eyes remains a challenge. The Boston keratoprosthesis (B-KPro) is a final option for patients with end-stage corneal disease and a poor prognosis with conventional penetrating keratoplasty. In this article the results of the first 13 eyes that received a B-KPro type I at the Department of Ophthalmology, University of Cologne, Germany are reported and the usefulness of postoperative slit-lamp optical coherence tomography (SL-OCT) for control purposes is evaluated., Material and Methods: All recipients of a B-KPro type I between September 2013 and May 2015 were included in the study. The feasibility of the operation, clinical outcomes, complications and revision surgery were investigated. The visualization of wound healing by SL-OCT was analyzed., Results: The age of the patients ranged from 26 to 92 years (mean 57.3 ± 20.9 years). In all 13 eyes from 12 patients (6 males and 6 females) dense corneal opacification with vascularization and sometimes also conjunctivalization was present. Preoperative visual acuity was reduced and ranged from mere light perception up to a maximum of 1/35 eye chart. All 13 eyes could be supplied with a B-KPro type I without any intraoperative complications, in 6 eyes no significant postoperative complications occurred, whereas in 7 eyes various additional surgical interventions were required and 1 B-KPro could not be preserved. Postoperative visual acuity ranged from light perception to 20/32 and was significantly improved in 85 % of the treated eyes. The use of SL-OCT reproducibly allowed the postoperative assessment of stromal thinning., Conclusion: The B-KPro provides the possibility of visual rehabilitation in high-risk eyes that could never be achieved without artificial cornea replacement. Despite higher complication rates this technique represents a significant progress in the surgical treatment of complex corneal pathologies. Regular and intensive postoperative controls are necessary to achieve good long-term results.

Various techniques for posterior lamellar keratoplasty have been established for the clinical routine and continuously improved during the last 15 years so that an extremely rapid recovery of vision is possible due to very thin transplants. Descemet membrane endothelial keratoplasty (DMEK) is the method of choice for simple corneal endothelial diseases and has already been applied in complex conditions of the anterior segment. The learning curve for DMEK is comparatively long and the risk of complications in complex anterior segment pathologies is higher than in Descemet's stripping automated endothelial keratoplasty (DSAEK); however, DMEK results in better visual outcome and less graft rejections than DSAEK. The latest evolution in posterior lamellar transplant surgery is ultrathin DSAEK (UT-DSAEK), where the grafted lamella is much thinner than in conventional DSAEK. Currently available data suggest that the resulting visual acuity after UT-DSAEK is close to the visual acuity seen after DMEK; however, studies comparing the results after DMEK and UT-DSAEK are so far lacking. Whether the transplantation of these very thin DSAEK grafts also results in endothelial cell densities and graft rejection rates comparable to DMEK has to be proven.

Background: Case report of a 6-year-old boy with bilateral choroidal osteoma., Case Report: The patient was followed up for the next 6 years and during this period bilateral tumor progression was observed. In one eye a staphyloma posticum developed in the area of the osteoma which led to secondary anisometric myopia., Conclusion: If undetected, this rare complication can lead to anisometric amblyopia; therefore, repeated cycloplegic refractometry is advisable in children with choroidal osteoma.

Deep anterior lamellar keratoplasty (DALK) offers tremendous advantages over penetrating keratoplasty (PK). Intraoperative safety is increased due to reduced opening of the eye interoperatively and the fact that no endothelial graft rejection can take place as the patient's own corneal endothelium is retained. Despite these advantages the number of DALK procedures performed each year in Germany remains constant at a low level. One reason could be that the DALK technique offers some complexity and at the same time intraoperative conversion to PK has to be performed in some cases due to rupture of Descemet's membrane. Moreover, interface-related and DALK-specific complications exist which can contribute to an unfavorable visual outcome. Most of these complications, such as incomplete attachment of Descemet's membrane or opacification within the interface between Descemet's membrane and the posterior corneal stoma can be resolved by adequate measures making PK for revision barely necessary. As visual acuity does not differ between PK and DALK, the benefits of DALK - lack of endothelial immune reaction and increased ocular stability during surgery - outweigh the risk of additional complications and DALK should therefore be performed whenever appropriate.

Background: Lamellar keratoplasties, e.g. Descemet's stripping automated endothelial keratoplasty (DSAEK) and Descemet membrane endothelial keratoplasty (DMEK) are considered the procedures of choice for corneal endothelial diseases. In comparison to penetrating keratoplasty (PK) they are associated with faster visual rehabilitation, a lower risk of complications and a decreased necessity for follow-up visits, which reduces the burden on quality of life in elderly patients. In order to advise patients regarding the indications for surgery and to facilitate the follow-up management, it is important to know the most important complications associated with these keratoplasty techniques., Results and Discussion: The most important preoperative complication is a delayed indication for the operation. In contrast to PK, DSAEK and DMEK surgery should be provided at an earlier stage of disease as chronic edema alters the stroma and reduces the speed of visual recovery. The most important complications during or early after surgery are detached lamellae, pupillary blocks with increased pressure or air bubbles in the vitreous cavity in patients with previous vitrectomy. The main long-term complications include chronic increased intraocular pressure and immune-mediated graft rejections in DSAEK patients after reducing or stopping topical corticosteroid therapy. This article describes the potential complications of endothelial keratoplasty and provides a detailed explanation of strategies to avoid these complications.

Techniques available for corneal lamellar refractive surgery are laser-assisted in situ keratomileusis (LASIK) using a microkeratome or femtosecond laser incision followed by excimer laser corneal ablation, and femtosecond laser-assisted refractive lenticule extraction (ReLEx). These treatments are nowadays considered to be safe and effective standard procedures for surgical correction of mild to moderate ametropia. Possible complications include too small or decentered optical zones, intraoperative flap cutting errors and postoperative inflammation (e.g. diffuse lamellar keratitis, DLK), epithelial or flap folds, epithelial ingrowths or iatrogenic ectasia. The occurrence of complications may be significantly reduced by compliance to corresponding standards of indication and treatment that are based on current scientific knowledge.

Descemet's membrane endothelial keratoplasty (DMEK) is a relatively new, but now established surgical procedure to cure corneal endothelial disorders. However, there are donor- and recipient-associated sources of potential complications, some of which can be identified and addressed prior to the procedure and others that may lead to intra- or postoperative problems. Preoperatively, risk factors for a limited visual outcome (ocular comorbidities, previous ocular surgery, subepithelial/stromal scars) and the specific indication for DMEK should be discussed with the patient. Intraoperatively, young donor age can be associated with a particularly elastic graft, which may be difficult to unfold. Such transplants are not appropriate for particularly difficult recipient situations (very opaque cornea, history of vitreoretinal surgery). Postoperatively, transplant dehiscence is the most common complication, which in many cases can be managed by reinjection of air (or a 20 % SF6 gas/air mix) into the anterior chamber. An elevation of the intraocular pressure after DMEK is often caused by a reaction to topical steroid therapy. Although immune rejections after DMEK are less likely than after Descemet's stripping automated endothelial keratoplasty (DSAEK) and perforating keratoplasty, adequate treatment with topical steroidal drugs is recommended. These common and several rare complications are discussed in this review.

The surgical treatment of congenital corneal diseases or corneal diseases occurring during infancy is demanding even for experienced corneal surgeons. Besides the need for frequent examinations under anesthesia during the postoperative follow-up in young children and infants (e.g. after corneal transplantation), the surgeon frequently encounters intraoperative and postoperative problems, such as low scleral rigidity, positive vitreous pressure and a narrow anterior chamber. Other problems include increased fibrin reaction, an increased risk of rejection in cases of allogenic corneal transplantation and frequent loosening of sutures necessitating replacement or early removal. Lamellar corneal transplantation reduces the risk of graft rejection and the risk of wound leakage. Moreover, posterior lamellar keratoplasty in children offers a faster visual recovery compared to penetrating keratoplasty and thus reduces the risk of amblyopia.

Background: The procedure of posterior lamellar keratoplasty has now become established as first choice therapy for the treatment of corneal endothelial diseases, with Descemet membrane endothelial keratoplasty (DMEK) as the most recent refinement of endothelial keratoplasty techniques. In this study the outcome of 400 consecutive cases after standardized no-touch DMEK was evaluated., Methods: This was a non-randomized retrospective clinical study of 400 consecutive eyes in 321 patients who underwent standard DMEK. Outcome measures included best corrected visual acuity (BCVA) preoperatively and postoperatively at 1, 3 and 6 months, endothelial cell density (ECD), pachymetry and complications within the first 6 months. In addition two subgroups were analyzed (subgroup I cases 1-200 and subgroup II cases 201-400)., Results: At 6 months postoperatively 80 % of all eyes had a BCVA of ≥ 20/25 (≥ 0.8), 44 % of ≥ 20/20 (≥ 1.0) and 14 % of ≥ 20/18 (≥ 1.2). There were no significant differences between the two subgroups. The mean ECD preoperatively was 2,542 (± 217) cells/mm(2) and 6 months postoperatively 1,622 (± 500) cells/mm(2). The mean ECD decrease was 36 % 6 months after DMEK. No difference between the subgroups was noted. In 5 % a re-operation was needed of which 75 % were performed in subgroup I., Conclusion: With the standardized no-touch DMEK technique a rapid and nearly complete visual rehabilitation as well as ECD values similar to earlier endothelial keratoplasty techniques can be achieved. Therefore DMEK may become the preferred treatment for corneal endothelial disorders.

Background: Patients undergoing corneal transplantation often suffer from postoperative reduced vision due to high astigmatism. This retrospective study analyzed the influence of heterotopic or orthotopic transplantation on astigmatism and visual outcome., Patients and Methods: In this study 373 eyes of 334 patients were analyzed. Group 1 (OT) contained 186 eyes, which underwent orthotopic transplantation (side of recipient and donor corresponded), whereas group 2 (HT) included 187 heterotopic keratoplasties (donor cornea placed in the recipient's contralateral side). After 1, 3, 6, 12 and 24 months the median of keratometric astigmatism, objective astigmatism, topographic astigmatism and best corrected visual acuity (BCVA) were assessed and compared between groups., Results: The long-term results showed no statistically significant differences regarding keratometric and objective astigmatism, whereas topographic astigmatism differed significantly (p = 0.04) after 3 months. We observed a lower astigmatism of 5.7 dpt (range 3.08-7.78 dpt) in group OT than in the group HT with 7.1 dpt (range 3.9-10.7 dpt). No differences were found at the other time points. The BCVA showed a significantly better effect after 1 month (p = 0.01) in the OT group of 0.2 (0.1-0.3) than in HT group of 0.1 (0.05/0.25). In the postoperative course no additional significant dissimilarities were documented., Conclusions: Heterotopic and orthotopic keratoplasty show no significant long-term differences in astigmatism and visual outcom.

Background: The aim of this study was to evaluate the effect of donor lamella thickness on postoperative visual acuity after Descemet's stripping automated endothelial keratoplasty (DSAEK)., Materials and Methods: A retrospective analysis of 65 eyes from 61 patients who underwent DSAEK surgery in cases of Fuchs' corneal dystrophy or bullous keratopathy between 2008 and 2011 was performed. The thickness of donor lamella was measured intraoperatively by ultrasonic pachymetry and postoperatively by anterior segment optical coherence tomography (OCT) and correlated to the visual acuity and number of endothelial cells., Results: The donor lamella thickness measured intraoperatively and postoperatively correlated significantly with each other (r = 0.874, p < 0.001). A significant correlation was found between postoperative corneal lamella thickness measured by anterior segment OCT and visual acuity (r = 0.273, p = 0.028) but not between intraoperative donor lamella thickness measured by ultrasonic pachymetry and visual acuity (r = 0.241, p = 0.103). The postoperative endothelial cell number did not show a correlation with either the intraoperatively or the postoperatively measured donor lamella thickness (r = - 0.059, p = 0.731, r = 0.024, p = 0.869, respectively)., Conclusions: Corneal lamella thickness < 120 µm was found to be correlated with a better visual outcome than in cases of thicker corneas > 120 µm. Despite greater difficulty in corneal transplant technique in cases of thinner lamella no increased damage of corneal endothelium was shown. Therefore, DSAEK with corneal lamella thickness < 120 µm is an interesting therapeutic alternative to DMEK.

Laser in situ keratomileusis (LASIK) is now considered to be a safe and effective standard procedure for surgical correction of low to medium ametropia. Nevertheless, as in any other surgical intervention complex courses may occur in single rare cases. Possible complications include, but are not limited to, optically effective errors, such as too small or decentered optical zones as well as intraoperative flap cutting errors and postoperative complications, such as inflammation (e.g. diffuse lamellar keratitis, DLK), epithelial or flap folds, epithelial ingrowths or iatrogenic ectasia. Compliance to standards for indications and treatment based on the current scientific knowledge can significantly reduce the occurrence of complications. These standards include adjustment of the optical zone to the mesopic pupil diameter, the application of state of the art eye trackers, careful performance of intraoperative and postoperative procedures by a surgeon experienced in the entire spectrum of ocular surgery, the possibility of treating complications directly on occurrence and, last but not least, conservative patient selection and choice of treatment.

Stem cells are the ultimate source of the rapidly self-renewing corneal epithelium and are located in the basal layer of the limbal epithelium. Ocular surface defense mechanisms are important for the integrity of the ocular surface under normal and compromised conditions. A variety of diseases can compromise the stem cell pool causing an entity called limbal stem cell deficiency. Clinical symptoms can range from foreign body sensation and glare up to blindness. The exact diagnosis is crucial for adequate therapeutic measures.

Background: To prevent complications through the use of contact lenses the efforts spent on documentation will have to be increased to meet the demands of quality management. How can ophthalmologists benefit from the use of software-based graphical documentation?, Methods: To make a standardized documentation of contact lens complications possible a software module with a graphically intuitive visualization scheme of the Efron grading scale was developed. The Efron grading scale defines 16 different classes of findings scaled to 5 grades., Results: The software module shows a schematic view of the eye which allows an easy sectoral assignment of all 16 Efron findings. The designated sector can simply be marked with a mouse click. A subsequently appearing context menu offers all 16 Efron findings together with the corresponding 5 grades. Finally the chosen Efron grade is graphically visualized in the corresponding sector., Conclusion: The software module supplies a feasible way for high quality documentation in the clinical routine with illustrative visualization. The practical application of software tools to assist clinical documentation can help to meet the challenges of quality management in contact lens utilization.

We present two cases of Peters anomaly (Peters plus syndrome and a maximum manifestation variant) with abnormally thickened cornea and corneal staphyloma. Both patients presented to our hospital shortly after birth and were treated with perforating keratoplasty and lensectomy. Histological analysis showed marked thickening of the corneal stroma due to abnormal stromal connective tissue deposition. Additionally, both eyes showed the characteristic changes of Peters anomaly with corneal opacity, adherence of the iris stroma and anterior lens surface to the posterior corneal surface, absence of the corneal endothelium, Descemet and Bowmans layers. Peters anomaly with abnormally thick intracorneal fibrosis with or without congenital corneal staphyloma is a very rare manifestation.

We report the case of a 67-year-old woman who presented with a persisting corneal erosion after blunt injury. Six years later, a highly prominent corneal tumor had developed at the site of the initial erosion. Histological analysis revealed a malignant melanoma. This case provides evidence that malignant melanoma may be a long-term complication of corneal epithelial disorders.

Aim: Several methods permit the measurement of geometric parameters of the cornea, but until now biomechanical conditions of the cornea have been ignored (e.g. in refractive corneal surgery). Besides the geometric condition, biomechanical properties of the cornea have been shown to influence applanation measurement of intra-ocular pressure (IOP) and epidemiological studies have identified corneal thickness as an independent risk factor for the development and progression of glaucoma. The aim of this investigation was to characterize the biomechanical properties of the cornea using the ocular response analyzer (ORA)., Methods: The ocular response analyzer (ORA) is a new method available for non-contact measurement of the biomechanical properties of the cornea. We evaluated the reproducibility of measurements, the difference between static and dynamic factors and the impact of independent factors (e.g. IOP, age, CCT, swelling of the cornea) on 2,500 measurements of corneal hysteresis (CH) and corneal resistance factor (CRF)., Results: In a large sample size we observed changes in CH and CRF after refractive surgery procedures (LASIK, UV-A cross-linking, keratoplasty) and in other corneal disorders (keratoconus, corneal dystrophies)., Conclusions: CRF and CH changes may reflect structural changes of the cornea. Thus, the ORA provides valuable information for a better understanding and characterization of the biomechanical condition of the cornea, especially with regard to diseases such as keratoconus and glaucoma.

Diagnosis of fungal keratitis is only straightforward if it presents in its typical form with pyramidal-shaped hypopyon, prominent geographical corneal infiltration and satellites. However, in contact-lens wearers clinical presentation is untypical at early infection. Corneal debridement is therefore necessary, which simultaneously facilitates penetration of the antimycotic into the corneal stroma. At present, voriconazole is the medication of choice, if not changed after microbiological diagnosis to a specifically more potent substance - e.g. posaconazole or caspofungin. Amphotericin B may be used for repeated intracameral or intravitreal injection. Simultaneous application of steroids is useful.

Modern Ophthalmology is currently experiencing a boom in anterior segment analysis tools that can increasingly gather information with one short measurement. These systems are based e.g. on so-called Scheimpflug (Pentacam, Galilei), slitlamp, and Placido technologies (Orbscan IIz) and other measurement systems. They help in analyzing the cornea in detail, including calculation of corneal wavefront. In addition, morphological and anatomical parameters such as anterior chamber depth, pachymetry, chamber angle, and lens densitometry can be analyzed. That makes these new technologies of interest for refractive surgery as well as for screening procedures such as Glaucoma, Keratoconus and others. This survey focuses on the two models, Oculus Pentacam and B&L Orbscan IIz as representative of all the other systems, as they are the most successful machines in this field.

Background: Cetuximab (Erbitux), a monoclonal epidermal growth factor receptor (EGFR) antibody, has been used for the treatment of advanced colorectal carcinoma over the last two years. Inhibition of EGFR also influences corneal wound healing as EGF stimulates the proliferation of epithelial cells., Method: Extensive corneal erosion was seen in both eyes of a 62-year-old patient under treatment with cetuximab for a metastasized colorectal carcinoma. Progression was fast despite vigorous conservative treatment. The application of autologous serum could not be considered because of the antibody treatment. Human EGF was applied topically several times daily in order to utilize the proliferative effect on corneal epithelial cells and to antagonize the inhibition of EGF receptors., Results: Improvement was seen shortly after the onset of therapy with EGF eye drops.. The epithelial defect was closed 7 days (left eye) and 19 days (right eye), respectively, after the onset of therapy. During this time treatment with cetuximab was continued., Conclusions: Cetuximab (Erbitux) can cause persisting epithelial defects. Patients with an impairment of corneal wound healing under cetuximab treatment can benefit from the topical application of human EGF. Consequently, surgical measures or complications such as infections can be avoided.

Various diagnostic procedures have been developed for analyzing the corneal surface. Corneal topographical measurements can be performed by classic Placido disc topographers and tomographers creating three-dimensional corneal models from cross-sectional images. Using a slit scanning system or the Scheimpflug technique additional information about the anterior segment can be obtained. New systems give us a wide range of diagnostic tools. Beside the measurement of the corneal anterior and posterior surfaces and point-to-point-pachymetry they enable analysis of corneal aberrations and measurement of the whole anterior segment, including the anterior chamber and the position of implants, pupil diameter, horizontal white-to-white and the kappa angle. Densitometry, statistical programs for the evaluation of corneal pathology and assistance with intraocular lens calculation following excimer laser treatment are further options available. This article is intended to give an overview of the principles and limitations of the various diagnostic systems.

In surface ablation, haze is the most frequent complication. After LASIK, microkeratome-related complications are rare today and usually resolve without sequelae, provided no ablation was done. Postoperative flap complications such as flap slippage usually occur during the first few days after surgery and should be treated as early whenever present. Interface complications are a new diagnostic entity as the interface between flap and stroma presents a space where fluid or cells can accumulate. Diffuse lamellar keratitis usually occurs within the first few postoperative days and should be treated aggressively to avoid scarring. Epithelial ingrowth is another rare complication usually requiring treatment. Corneal hydrops with fluid accumulation in the interface is a very rare but important phenomenon related to steroid-induced glaucoma but presenting with false low tonometry readings. Corneal ectasia is extremely rare and in most cases related to thin stromal beds. However, its pathogenesis is not yet completely understood, and it may occur in "normal" eyes, too. Dry eye syndrome is the most frequent complication after LASIK. It is usually benign but may cause significant visual impairment in rare cases.

Purpose: Vitamin A has been identified as a substantial substrate for ocular surface integrity. Vitamin A deficiency, which can be caused by different etiologies, may lead to severe complications including corneal perforation., Patients: Case reports of three patients with vitamin A deficiency. The observed etiologies leading to vitamin A deficiency were: (1) primary biliary cirrhosis, (2) malnutrition induced by alcohol abuse, and (3) malabsorption syndrome caused by a graft-versus-host disease of the intestine after bone marrow transplantation., Results: In all three cases vitamin A serum levels were below normal values. In two patients retinol binding protein was also reduced. Two of three had corneal perforation, which required penetrating keratoplasty (pKP). In one of these cases, pKP was performed three times on both eyes due to a rapid corneal stromal melting leading always to corneal perforation. The third case developed severe corneal thinning of both eyes with the formation of a descemetocele. Vitamin A substitution improved symptoms and ocular surface integrity., Conclusion: Vitamin A in combination with zinc is a crucial factor for maintaining conjunctival and corneal ocular surface integrity. Moreover, vitamin A regulates mucin production of the ocular surface epithelium, which is known to be an important part of the ocular surface defense. Several different primary diseases might cause vitamin A deficiency. Especially patients with rapid progressive corneal ulceration should be examined for vitamin A deficiency. Substitution of vitamin A should be initiated as early as possible to prevent serious disease progression.

Background: The Lowe oculo-cerebro-renal syndrome (OCRL1) is a rare X-linked disease which causes impairment of visual acuity. The situation may be further complicated by corneal alterations., Patients and Methods: In total seven patients from different families were clinically examined by slit-lamp examination, funduscopy, measurement of the intraocular pressure and ultrasound sonography. Molecular genetic analysis was performed in six patients by sequencing large PCR amplicons with a DNA sequencer and the ABI PRISM Sequence navigator software., Result: All affected boys were aphakic. Due to high intraocular pressure, iridectomy, goniotomy, cyclo-cryo treatment or trabeculectomy were performed. All patients showed opacity or pannus-like alterations of the cornea. Molecular genetic analysis revealed four novel and two known mutations., Conclusion: Reduced visual acuity was partly explained by morphological changes due to the underlying genetic defect and the development of cataract and glaucoma. Opacity of the cornea as well as alterations of the cornea seem to be a major problem in the course of the disease.

Background: Autologous rotational keratoplasty is a therapeutic option in patients with central corneal scars. Its advantage compared to allogeneic procedures is the absence of immunologic destruction. In this retrospective analysis, long-term results are presented., Patients: Since 1988, autologous rotational keratoplasty was performed in 19 patients. The trephine size varied between 7.0 mm and 9.0 mm. Mean follow-up was 7.1+/-4.6 years., Results: In 16 of the patients, the graft center remained clear during the entire follow-up period. Visual acuity improved from 0.25+/-0.16 preoperatively to 0.34+/-0.27 postoperatively with spectacles and to 0.43+/-0.3 with contact lenses. The mean postoperative astigmatism was 6.41+/-3.87 dpt with irregular components in six patients., Conclusion: Only a few postoperative graft complications were observed. Especially in patients with a markedly elevated risk for immune reactions (e.g., small infants), autologous rotational keratoplasty is a safe alternative to allogenic procedures.

The ocular surface consists of the lid margin, conjunctiva and cornea which together with the tear system represent a functional entity. The diagnosis of ocular surface disease can be very difficult due to the similarity of various disease entities. The classification should be made on the pathological and pathophysiological characteristics of ocular surface disease. The first part of the classification comprises diseases of the lid margin, the tear system as well as diseases of the conjunctiva. Both the clinical presentation as well as the underlying pathophysiological and pathological characteristics of the most important ocular surface diseases are reviewed.

Purpose: To describe several techniques for posterior lamellar keratoplasty through a scleral incision, for management of corneal endothelial disorders like pseudophacic bullous keratopathy and Fuchs' endothelial dystrophy, and to report the mid-term clinical results., Methods: Three techniques have been developed to perform a posterior lamellar keratoplasty procedure through a scleral incision, i.e. to replace the posterior corneal layers while leaving the anterior corneal surface intact and without the use of corneal sutures. In the first technique, a 9.0 mm scleral incision is made to accommodate an intracorneal trephine and spoon-shaped glide to insert a 7.5 mm donor posterior lamellar disc into the anterior chamber. In the second technique, the procedure is performed through a 5.0 mm scleral tunnel incision using microscissors and by folding a 8.5 mm donor posterior disc prior to insertion. In the third technique, a 4.0 mm tunnel incision is made to perform a descemetorhexis in the host cornea, i.e. Descemet's membrane is selectively excised from the recipient eye, and a 9.0 mm donor Descemet's membrane is inserted. In eyes with a minimal postoperative follow-up of 3-5 years ( n=16), we documented the best spectacle corrected visual acuity (BSCVA), keratometry readings, endothelial cell counts, and clinical events., Results: In all cases, the graft adhered to the recipient posterior cornea without suture fixation. In patients without concomitant ocular disease, BSCVA was 0.7-1.0 in all eyes. The astigmatism averaged 2.1+/-0.7 D, endothelial cell counts averaged 2126+/-529 cells/mm(2) at 6 months, 1839+/-473 cells/mm(2) at 12 months, 1418+/-434 cells/mm(2) at 24 months, and 1137+/-420 cells/mm(2) at 36 months. In two patients, an irido-corneal adhesiolysis was performed within days after the procedure. In one patient, residual visco-elastic adherence was present at the donor-to-recipient interface, and a penetrating keratoplasty was performed 1 month postoperation. One patient developed significant interface haze, requiring a penetrating keratoplasty 13 months after the first surgery., Conclusion: Posterior lamellar keratoplasty can be an effective surgical technique to manage corneal endothelial disorders. An improved visual acuity can be obtained within the first weeks after surgery, and the visual perfomance of the graft is stable up to 5 years postoperation.

Background: Possible improvements of best corrected visual acuity (BCVA) and the stability of the endothelium in deep lamellar keratoplasty (DLKP) assisted by Excimer laser polishing were evaluated., Patients and Methods: A 59-year-old male patient was treated 1 year after DLKP with the intention of improving is BCVA. Excimer laser treatment was performed on the deep layers of the graft and on the posterior lamellae of the recipient's cornea 100 micrometer over the endothelium., Results: BCVA improved from 20/100 to 20/22 within 9 months. Refraction remained identical pre- and post-laser treatment at +1.25 sph=-2.0 cyl/17 degrees The endothelium remained unchanged with regard to its cell count of 2,100 cells/mm(2) and the normal morphology., Conclusion: Smoothing of the interface by Excimer scanning laser treatment may be recommended in such cases of DLKP in which postoperatively the resulting visual acuity does not correspond to the retinal resolution. Use of scanning or flying-spot lasers are the prerequisite for the maintenance of the endothelium.

Background and Aims: Patients with recurrent corneal erosion syndrome can suffer both psychologically and socially due to excessive pain. After the failure of conservative treatment, conventional surgical interventions, such as anterior stromal puncture have been suggested. The purpose of this study was to assess the morphological and functional results of minimally invasive excimer laser phototherapeutic keratectomy (e-PTK) for treatment of recurrent corneal erosion syndrome and in particular, to evaluate the cumulative recurrence rate., Patients and Methods: Between July 1990 and January 2001, 116 e-PTKs have been performed mostly in the acute stage of the disease in this single center study. In 15 eyes an unsuccessful PTK had previously been performed elsewhere. Reasons for the erosion included trauma ( n=80), Cogan's epithelial dystrophy ( n=14), bullous keratopathy ( n=2), and in 20 cases no cause could be detected. A manually guided spot profile was applied in 99 cases (pulse energy 10 mJ, repetition rate 2/s or 3/s, 66-330 pulses). In 17 cases a scanning slit procedure was applied (intended ablation per scan 1 microm, repetition rate 20/s, 447-1,017 pulses). The broad deepithelialisation of Bowman's layer was treated with defocussed overlapping laser pulses., Results: Complete epithelial closure was achieved after an average of 2.3+/-1.3 (median 2) days, the mean follow-up was 2.2+/-1.6 years with a maximum of 5.6 years. Best corrected visual acuity increased from 0.6+/-0.4 preoperatively to 0.9+/-0.3 postoperatively. The keratometric central power remained constant (preoperative 43.0+/-2.2 D (diopters), postoperative 43.3+/-1.9 D). The median keratometric astigmatism remained constant at 1.0 D. Only in 2 patients was an iatrogenic, not completely reversible irregular astigmatism of more than 2 D induced during the learning curve. The spherical equivalent did not change significantly (-1.0+/-3.3 D preop., -1.1+/-3.4 D postop.). In 9 eyes (7.8%) a recurrent epithelial defect occurred after 2-24 (average 8+/-6) months. The cumulative 1-year recurrence rate was 6.5%, the 2-year recurrence rate was 11.5%, the 3-, 4-, and 5-year recurrence rates were all 13.6%., Conclusions: For recurrent corneal erosion syndrome, e-PTK performed with low pulse energy and low number of pulses can be considered an effective treatment modality to achieve a fast and mostly durable epithelial closure. Even after broad removal of the loose epithelium, change of refraction or induction of an irregular astigmatism with visual loss seems to be the exception.

Objective: Laser in situ keratomileusis (LASIK) has become the leading refractive option to correct myopia, hyperopia and astigmatism. In addition to the relative rapid visual recovery and the lack of postoperative pain, new flap-related complications are being added. The formation of striae can lead to a significant loss of corrected visual acuity if the central pupil zone is affected. Awareness of possible complications is important not only for the surgeon, but also for the ophthalmologist in practice for a rapid diagnosis and prompt management., Clinical Course and Therapy: Striae have a variety of appearances and are caused by misalignment/displacement of the corneal flap after replacement or are results of a slippage effect of the flap over the stromal bed after LASIK. Symptomatic striae ideally are treated on the first or second day after surgery by refloating the flap, irrigation with balanced salt solution or hypotonic saline solution, stroking and stretching the surface with moist and dry sponges. In more severe cases scraping away the epithelium and antitorque or interrupted sutures can be performed to treat persistent striae., Conclusion: Symptomatic deep striae should be diagnosed early and managed promptly within the first days. Any later intervention decreases the chance of successful elimination and visual outcome. Hints to prevent the development of striae formation are provided.

Background: A reduced number of endothelial cells increases the risk of corneal decompensation after cataract surgery. It is difficult to quantify the number of endothelial cells using slit-lamp microscopy since normal corneas may also show a reduced number of endothelial cells., Materials and Methods: We compared the number of endothelial cells pre- and postoperatively in a group of 500 consecutive patients., Results: Corneas diagnosed preoperatively with corneae guttata by slit-lamp microscopy may reveal more than 1800 endothelial cells/mm2. These corneas may decompensate after surgery., Conclusion: We consider the routine use of endothelial microscopy to be a helpful diagnostic tool prior to cataract surgery.

Introduction: We report a patient suffering from lattice corneal dystrophy and with a corneal granuloma 8 months after phototherapeutic keratectomy (PTK)., Case Report: PTK was performed in a 33-year-old man with hereditary lattice corneal dystrophy. An intracorneal tumor was found in the right eye 8 months after treatment. There was no indication of previous foreign body injury. Detailed clinical examinations yielded no evidence of the cause or nature of the tumor. For this reason an excision was performed., Results: Histological examination revealed a dense inflammatory infiltration of the deeper corneal stroma with epithelioid cells, histiocytes, lymphocytes, eosinophilic leukocytes, and giant cells of the Touton type. There was no indication of a generalized granulomatous inflammation., Conclusion: A monosymptomatic, intracorneal, granulomatous infiltration is very rare and has not previously been described in a case of lattice corneal dystrophy. This may have been a case of xanthogranuloma or sarcoidosis. Strangely, the granuloma occurred only several months after PTK; nevertheless, a pathogenetic connection seems unlikely.

Introduction: This study evaluated the clinical use of optical coherence tomography (OCT) for two-dimensional representation of the cornea., Patients and Methods: Noncontact slitlamp-adapted OCT was used in selected cases to evaluate pathologically altered corneas and to measure the central corneal thickness and curvature., Results: OCT provided correlation between differences in reflection and morphological changes. Scar tissue resulted in hyper-reflective light scattering, whereas cystic lesions were hyporeflective. Precise biomorphometry also allowed representation of intrastromal and retrocorneal changes. Central corneal thickness measured by OCT yielded reproducible values and corneal curvature could be calculated from the optical signals of the corneal surface., Conclusions: OCT provides high-resolution representation of the cornea and exact evaluation of its morphology, thickness, and curvature. Due to the noncontact, simple, and rapid examination using the slitlamp the corneal OCT method is a promising additional diagnostic modality.