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Both high myopia and glaucoma can have devastating effects on the visual function. Diagnostic tests and treatment can be challenging when both diseases are coexistent. This article discusses some of the typical problems in the management of glaucoma in highly myopic patients.

Numerous systemic diseases can be manifested at the cornea. Sometimes the corneal involvement is the first sign of the systemic process. It may be manifested in many different forms, such as an ulcer in the corneal periphery or a crystalline keratopathy. Therapy is interdisciplinary and primarily directed against the underlying disease.

Canaloplasty is a recently introduced non-penetrating surgical technique for glaucoma, which seeks to improve the natural outflow of aqueous humor through Schlemm's canal and the collector channels. Just as in all forms of glaucoma surgery there are a certain number of failures of the procedure and further surgery is often needed to reduce the intraocular pressure in these cases. This article describes some of the revision procedures after canaloplasty.

Little is known about how the expression of Ephrin type-A receptor 2 (EphA2) influences cell-cell adhesion, migration, angiogenesis, and the formation of vasculogenic mimicry (VM) channels in uveal melanomas or how this may be related to the rate of metastasis.Paraffin embedded sections of 50 histopathologically well characterised primary uveal melanomas (mean largest tumour diameter: 16.3 mm) were evaluated with respect to the expression of EphA2. Systemic metastasis was detected in 29 patients. The remaining 21 patients were followed for a mean of 10 years. Tumour angiogensis was analysed by endoglin expression (CD105), the activity of the mature vascular system (von Willebrand factor) and the presence of VM (CD31/PAS staining).All uveal melanomas expressed EphA2, with a mean of 95.93 % positive cells ± SD: 6.3 %. There was no significant association between EphA2 and the rate of metastases (p = 0.196), endoglin expression (p = 0.652), VM (p = 0.267) or with any other clinical or histopathological factors (p 0.05). However, there was significant up-regulation of EphA2 in the nucleus of the metastatic uveal melanoma subgroup, while cytoplasmatic localisation in the subgroup was associated with better prognosis (p = 0.006). There were low levels of EphA2 expression in the specific retinal layers, the ciliary and corneal epithelium, and the choroidal and corneal endothelium.Nuclear expression of EphA2 in this series of large tumours was significantly associated with an increased rate of metastasis. On the other hand, cytoplasmic localisation was associated with a better prognosis. As there was no correlation between EphA2 expression and angiogenesis, the mature vasculature or VM, EphA2 appears to become less important in the advanced stages of the disease.

This report describes the case of a 6-year-old girl who presented with painless swelling of the right orbit since 4 weeks and moderate proptosis. Tests revealed visual acuity RE 6/6, LE 6/6, normal intraocular pressure (IOP), anterior and posterior segments normal. Ultrasound examination showed multiple lesions in the spleen, normal liver, no abdominal mass and enlarged abdominal lymph nodes. Fine-needle aspirate results were not available at the time of clinical decision-making. In Malawi the treatment for all stages of Burkitt's lymphoma is intravenous cyclophosphamide (40 mg/kg on day 1 and oral cyclophosphamide 60 mg/kg on days 8, 18 and 28). Intrathecal hydrocortisone (12.5 mg) and methotrexate (12.5 mg) are given with each treatment cycle.

A 17-year-old asymptomatic girl presented with discrete bilateral deposits in the superficial corneal stroma. The diagnosis of very early and in this form as yet undescribed manifestation of Schnyder's corneal dystrophy, was only possible based on the family history.

An 81-year-old patient presented for cataract extraction of the right eye. In addition a bilateral disc-like corneal opacity was present which was classified as central cloudy corneal dystrophy of François. This rare and clinically unproblematic condition will be discussed.

Various publications especially from the field of dermatology have indicated in the recent years that the melanocyte is a "multitalent" with--besides UV-protection--(neuro-)humoral and immunological functions. Moreover, the melanocyte could play a role as a scavenger of free radicals or in pressure perception, so that it could even perhaps be part of the "intraocular pressure sensor". It is generally assumed that the cornea is devoid of melanocytes under physiological conditions. However, to the best of our knowledge a systematic investigation with a larger quantity of specimens has not been performed thus far.103 corneal specimens (whole eyes, corneal explants with different corneal diseases, corneoscleral donor buttons) and 13 pterygia (corneal part) were studied immunohistochemically using the monoclonal antibody Melan A which is specific for melanocytes.In healthy cornea melanocytes are found in the limbal area. In the corneal periphery, up to 1 mm distant from the limbus, the melanocytes disappear so that the mid-peripheral and the central epithelium of the cornea are devoid of melanocytes. Under pathological conditions (dystrophies, scars, ulcers) there is only exceptionally an invasion of melanocytes into the mid-peripheral corneal epithelium. The central epithelium almost always remains free of melanocytes even in various corneal diseases. In more than 50% of the pterygia melanocytes can be found in the epithelium.Under certain, pathological conditions melanocytes can settle in more central regions of the corneal epithelium. Thus, the very few "corneal melanomas" described in the literature could have theoretically developed within the cornea itself (and not within the limbus). Obviously, the cornea possesses mechanisms to inhibit centripetal migration of melanocytes perhaps via a (still hypothetic) "corneal melanocyte suppression factor" ("CoMeSuF"). To identify this factor will be the task for the coming years. If this factor is really existent it could possibly serve as a therapy for melanocytic proliferations (melanomas).

The aim of this study was to evaluate ocular changes in patients with xeroderma pigmentosum (XP) and Cockayne syndrome (CS). Both diseases belong to the progeroid syndromes caused by single gene mutations with premature aging. Both syndromes are very rare autosomal recessive diseases caused by a gene defect leading to deficient DNA repair.12 patients (4 female, 8 male) with XP between 3 and 67 years old and a 39-year-old female patient with CS were examined. The examination included visual acuity testing, slit lamp biomicroscopy, funduscopy and performance of a Schirmer test.Changes of the lids in the XP group included blepharitis in 7 patients, distichiasis in 2, and madarosis in 3 patients. 8 patients had multiple lentigines solares of the lids. One patient showed scars of the lower lids after excision of a squamous cell carcinoma and a basal cell carcinoma. Conjunctival lesions comprised a tumour of unknown origin of the conjunctiva in 1 patient, teleangiectasia of the conjunctiva in 5 patients, pterygia in 4, and pinguecula in 1 patient. Two patients had an intraepithelial melanosis of the conjunctiva, and one showed conjunctival nevi. Two patients had corneal scars and corneal vascularisation, another a significant conjunctivalisation of the cornea. A Schirmer test was feasible only in 3 patients. Tear production was markedly reduced in all these patients. Break-up time was shortened significantly in 6 patients examined. The patient with the CS showed atrophy of the pupillary rim and a subcapsular cataract. Funduscopically there were pigment epithelial changes.Patients with XP often exhibit ocular changes. Beside the light-dependent degenerative and inflammatory manifestations at the lids, the conjunctiva and the cornea patients with XP also develop malignancies early in life. The majority of patients suffer from dry eye symptoms.

We report about a 5-year-old boy who presented in our clinic with bilateral, slowly progressive solid tumors of the eyebrows. Histological examination of the excised tumors revealed the typical diversified picture of pilomatrixoma with basophilic and shadow cells. The bilateral or multiple manifestation of pilomatrixoma is uncommon and can be associated with myotonic dystrophy, sarcoidosis or Gardner's syndrome.

The number of available glaucoma drainage implants has increased in recent years and now offers additional therapeutic opportunities in refractory glaucoma patients.In drainage surgery two different functional principles are practised. The most commonly used implants (Ahmed, Krupin, Schocket, Molteno, Baerveldt) drain ocular fluid via a silicone tube into an episcleral reservoir. Multiple studies have compared the operative results of tubes, which differ in insertion, valve, material and surface characterstics. In analogy a miniature stainless steel implant (Ex-PRESS) is implanted at the limbus into the anterior chamber and drains subconjunctivally. These implants shunt ocular fluid under a scleral flap or directly under the conjunctiva. Intrascleral systems (Seton, SLOX, Silicone tubes) drain ocular fluid from the anterior chamber into the suprachoroidal space to regulate intraocular pressure (IOP).In the literature the postoperative success rates in tube shunts range between 22 % and 100 % depending on glaucoma form and implant. Most of the studies were retrospective. Miniature stainless steel devices (Ex-PRESS) placed directly under the conjunctiva may cause erosion of the conjunctiva and hypotony. Implantation under a scleral flap decreases these complications. Compared with trabeculectomy the implantation of this shunt under a scleral flap reduces the early postoperative rate of hypotony and choroidal effusion. Microimplants of Schlemm's canal ab externo or interno should reduce the intraocular pressure by connecting the anterior chamber and Schlemm's canal. Suprachoroidal shunts are an option in patients with scarred conjunctiva. Though, complications of fibrous encapsulation and dislocation are described.The development of drainage implants allows the reduction or avoidance of complications which are typical of traditional glaucoma filtering surgery. However, in the absence of long-term follow-ups, the procedures with their new side effects have to be further evaluated.

An extremely rare type of injury is presented: unintentional traumatic self-enucleation.

Traumatic wound dehiscence is a rare complication after penetrating keratoplasty. Limbo-keratoplasty seems to alter the pattern of rupture after blunt trauma.We report on one patient with dehiscence of a penetrating limbo-keratoplasty after blunt trauma.The dehiscence occurred over the entire extent of the clear corneal scar, whereas the sclero-corneal interface remained adapted.This case suggests a different tensile strength of these two scar types, possibly associated with their different grades of vascularization or epithelialization.

Cutis marmorata telangiectatica congenita is a rare, cutaneous and vascular anomaly characterised by congenital cutis marmorata, telangiectasis, and phlebectasia. Systemic abnormalities are frequently associated with this condition, although they may be highly variable. Ocular abnormalities are quite rare and include retinal detachment and congenital glaucoma. If glaucoma is present it is unilateral in most cases.A case of bilateral congenital glaucoma associated with cutis marmorata telangiectatica congenita in a 4-month-old girl is presented. Follow-up and treatment over a four-year period are reported.Congenital glaucoma is a rare ophthalmic condition encountered in patients with cutis marmorata telangiectatica congenita. If glaucoma is present it is most likely unilateral, although cases of bilateral glaucoma in these patients occur. Regular ophthalmological examinations are recommended for children with cutis marmorata telangiectatica congenita--especially if characteristic skin changes are present in the face.

Protein kinase C (PKC) plays a key role in cell metabolism. Three subgroups and 12 isoforms have been isolated so far, catalysing specific functions in cell metabolism. The demonstration of PKC subtypes in corneal tissue has been inconsistent. The aim of this study was to verify the expression of several PKC subgroups and isoforms in human and bovine corneal epithelial and endothelial cells.PKC subgroups and isoforms were studied using polyclonal antibodies.Antibodies to PKC-alpha, -delta, -epsilon and -zeta, representing all three PKC subgroups, bound in human and bovine corneal epithelium and endothelium. No binding was found for antibodies to PKC-beta2.For the first time the presence of all three PKC subgroups was demonstrated in human and bovine corneal epithelial and endothelial cells. Further studies are needed to show the role of these subgroups in cellular functions such as cell proliferation and differentiation.

The measurement of corneal thickness plays an increasing role in glaucoma screening and diagnosis. The influence of a variety of drugs on corneal thickness is well established. Especially for antiglaucomateous drugs this effect seems to be important. However, little is known about the influence of beta receptor antagonists on corneal thickness. The aim of this study was to provide evidence of the effect of timolol on central corneal thickness and endothelial cell density.Ten healthy volunteers (five women and five men) with a mean age of 29 years (range 25 to 56 years) were examined in a double-blind, prospective and randomised pilot study. Intraocular pressure, corneal thickness and endothelial cell density was estimated before as well as fifteen minutes, 24, 48, 72 and 96 hours after application of timolol 0.5 % eye drops twice daily. The partner eye received sodium hyaluronate eye drops twice daily and served as a control.The application of timolol showed a decrease of intraocular pressure from initially 12 mmHg to 9 mmHg after four days (p = 0,0188) as well as an increase of corneal thickness from 537 microm to 557 microm after four days (p = 0,0659). There was no change of intraocular pressure (p = 0,9935) or corneal thickness (p = 0,9998) in the control eyes. There was also no effect of timolol (p = 0,2782) or sodium hyaluronate (p = 0,1940) on endothelial cell density.The study provides evidence of the influence of beta receptor antagonists on corneal thickness. This effect may be caused by receptor mediated influences on corneal ion and fluid transport. Further studies are needed to show if the increase of corneal thickness after application of topical timolol has clinical importance.

In spite of a huge amount of morphological and biochemical findings the aetiology of keratoconus (KC) is still unclear. While the primary changes are seen and found mainly within the anterior corneal stroma, some authors believe that the corneal epithelium plays the major role in KC formation. Consequently, the epithelial basement membrane (BM) has become a matter of interest in KC research.The study included 55 consecutive patients who had a perforating keratoplasty performed because of KC. The corneal explants were processed for light microscopy in the usual manner. Slides were stained with PAS. The whole explant was investigated although the focus of interest was put on the epithelial BM. 10 normal corneas of eyes which had to be enucleated because of a choroidal melanoma served as a control.In 3 patients (5.5 %) there was a significant linear thickening of the BM (thickness of the BM more than 5 % but less than 30 % of the thickness of Bowman's layer). Only in one patient (1.8 %) there was an excessive BM thickening (thickness of the BM more than 30 % of that of Bowman's layer) with focal (central) BM warts directed against the epithelium ("inverse cornea guttata"). In general, thickening of the BM was most pronounced paracentrally. None of the control corneas had a significantly thickened BM.A massively thickened epithelial BM can be occasionally observed in KC but is rare on the whole. It is most likely an unspecific secondary phenomenon and not a prerequisite for KC development. The "inverse cornea guttata" was a unique finding.

Lisch corneal dystrophy is an epithelial corneal dystrophy where diffuse gray corneal opacifications are seen in direct illumination that appear in different patterns. Examination in retroillumination shows that these gray changes do consist of multiple densely crowded clear microcysts according to the opacity units. We wanted to investigate if one single case of Lisch corneal dystrophy can be differentiated easily from the other epithelial corneal dystrophies by exact analysis of the opacity units.We examined three non-related patients with Lisch corneal dystrophy at the slit-lamp. The epithelial corneal opacities were documented photographically with direct slit-lamp illumination and retroillumination.All three patients showed the characteristic intraepithelial densely crowded clear microcysts in retroillumination. Diffuse gray radial opacities were noted in both corneas of a 15-year-old female patient. A club-shaped opacity was noted in one cornea of a 35-year-old female patient and a feathery gray opacity pattern was found in one cornea of a 49-year-old male patient. All three patients showed the characteristic densely crowded clear microcysts of Lisch corneal dystrophy in retroillumination.Taking the previous literature into account, five different opacity patterns can be summarised: radial, band, flame/feathery, whorled and club-shaped gray epithelial corneal opacities. A single case of Lisch corneal dystrophy can be differentiated from other epithelial corneal dystrophies by means of an exact analysis of the opacity pattern and units by slit-lamp examination.

About 800,000 people in Germany suffer from glaucoma. As the population ages, the prevalence of glaucoma will rise and the socio-economic impact of the disease increase. The costs of glaucoma include those of diagnosis, medical, laser and surgical treatment, and costs of blindness as a result of glaucoma. So far the costs of diagnosis and screening programs are not well known but there is substantial knowledge of the therapeutic costs. Daily therapy costs are about 0.50 to 1.00 euro per patient. Every year the German statutory health insurance system pays about 1,000 euro per glaucoma patient. In addition, blindness due to glaucoma costs the German government over euro 150 million annually in Social Security benefits, lost income tax revenues, and health care expenditures. Demographic changes and scientific progress will even aggravate the socio-economic burden of this disease.

Shortly after birth, a massive enlargement of the right eye was observed in an otherwise healthy male child. The cornea of the affected eye was vascularized and completely cloudy without a sharp border between cornea and sclera. The diagnosis of a congenital glaucoma was made but an operation was not undertaken because of the difficult anatomical situation and the lost function. When the child was almost 3 years old enucleation was performed to prevent complications due to corneal exposure, and to improve the cosmetic aspect.The morphological investigations of the enucleated eye disclosed findings typical of what is called in the literature "congenital anterior staphyloma" or "congenital corneal staphyloma", namely a massively staphylomatous cornea with superficial neovascularization, destruction of Bowman's layer, and absence of Descemet's layer as well as corneal endothelium. Angle structures were completely absent, and the corneal back-side was lined by a pigment epithelial layer and focally by an additional inner layer of non-pigmented epithelium. There was no anterior chamber. The lens was markedly diminished in size (microphakia) and partly embedded in the corneal stroma. Pars muscularis and pars ciliaris of the ciliary body were separated. Elongated, thin ciliary processes were extended towards the small lens while the pars muscularis was fully covered by the retina.This rare, complex malformation syndrome which can be easily distinguished from primary congenital glaucoma should not be reduced conceptually to the corneal staphyloma because this staphyloma constitutes only a part of the whole. Taking the leading morphological aberrations into consideration we would rather propose the new term " corneal staphyloma- anterior chamber agenesia- microphakia syndrome (CSAMS). We hypothesize that CSAMS may be due to a pathological fusion of the early anterior optic cup. As the posterior eye segment is often normal in CSAMS, a staphyloma excision along with a sclero-keratoplasty might be an alternative therapeutic option to avoid enucleation and restore ambulatory vision.

With few exceptions all intraocular tumours can principally induce elevation of intraocular pressure (IOP). The frequency of IOP-elevation is mainly dependent on the biologic behaviour of the neoplasm (benign / malignant), its dimensions, localization, and its ability to provoke secondary changes like retinal detachment, hemorrhages, inflammation, and necroses. There are many and diverse pathomechanisms which may cause a "neoplastic glaucoma". The leading mechanisms are direct invasion of the chamber angle by tumour cells, rubeosis iridis, and forward displacement of the iris-lens-diaphragm with (lens induced) pupillary block. As intraocular tumours may have pressure-lowering effects too, eyes bearing a tumour may also be hypotonic or normotonic inspite of a "glaucomatous chamber angle morphology". The therapy of the tumour-induced glaucomas is hardly validated and generally follows the treatment of the other secondary glaucomas. However, filtration procedures are contraindicated when a malignant tumour is diagnosed or suspected. Because of the progress of tumour therapy with salvation of eyes which had to be enucleated in former times and prolongation of survival in some tumour entities (like Non Hodgkin's lymphoma and metastases) the significance of tumour-induced glaucomas will probably further increase in the future.

A sixty year old woman presented with a tumor of the caruncle that had persisted for one year. Ophthalmologic history was unremarkable. Physical examination disclosed an indolent, tight elastic, highly vascularizated tumor. The lesion measured five millimeter in diameter. Histopathology revealed tall columnar cells with small round nuclei. The cytoplasm stained positive with hematoxylin and eosin. The typical histopathologic features were consistent with an oncocytoma.Tumors of the caruncle are very heterogenous because the caruncle contains tissues of the conjunctiva and the epidermis. Therefore to exclude a malignant process an excisional biopsy should always be performed even so in general tumors of the caruncle are benign. The most frequent tumors of the caruncle are papilloma, nevi, pyogenic granuloma, epithelial inclusion cyst and chronic inflammation. In only 4 % of the cases oncocytomas are found even the oncocytoma is considered to be a typical tumor of the caruncle.