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ZusammenfassungBilaterale Hornhauttrübungen können Ausdruck unterschiedlichster Systemerkrankungen sein. Insbesondere im Kindesalter ist hierbei differenzialdiagnostisch an diverse, aber meist sehr seltene Stoffwechselerkrankungen zu denken. Da die Hornhauttrübungen zu den ersten klinischen Symptomen dieser Erkrankungen zählen können, kommt dem Ophthalmologen eine entscheidende Rolle hinsichtlich der Einleitung einer frühen interdisziplinären diagnostischen Abklärung zu. Die frühe Diagnosestellung ist durch die daraus ggf. resultierende Therapieeinleitung für den weiteren klinischen Verlauf und die Prognose der betroffenen Patienten äußerst entscheidend.

Echography (also ultrasound) is a clinical, non-invasive imaging module that is used for the measurement of the axial length of the eye and for the investigation of pathologic entities of the chamber angle, the iris and the ciliary body. Furthermore, its role in the management of vitreoretinal pathologies and the differentiation of intraocular tumors is indisputable. Echography remains the first-choice imaging tool in case of insufficient visualization of the posterior segment due to opacity or obstruction of the optical media of the eye. In addition, it can contribute to a more precise diagnostic characterization of lesions in all eye segments. Patients with corneal opacities, abnormalities of the iris, the chamber angle and the ciliary body, as well as patients with dense cataract, vitreous hemorrhage or inflammatory opacities can be properly diagnosed via ultrasound and be treated accordingly.

Bilateral corneal opacities can be a leading symptom of different systemic diseases. Especially in childhood, various metabolic diseases, although very rare, should be considered as a possible diagnosis. Since corneal opacities can be among the first clinical symptoms of these diseases, the ophthalmologist plays a central role in initiating early interdisciplinary diagnostics. The early diagnosis is extremely important for further prognosis and the clinical outcome of the affected patients due to the early initiation of therapy.Bilaterale Hornhauttrübungen können Ausdruck unterschiedlichster Systemerkrankungen sein. Insbesondere im Kindesalter ist hierbei differenzialdiagnostisch an diverse, aber meist sehr seltene Stoffwechselerkrankungen zu denken. Da die Hornhauttrübungen zu den ersten klinischen Symptomen dieser Erkrankungen zählen können, kommt dem Ophthalmologen eine entscheidende Rolle hinsichtlich der Einleitung einer frühen interdisziplinären diagnostischen Abklärung zu. Die frühe Diagnosestellung ist durch die daraus ggf. resultierende Therapieeinleitung für den weiteren klinischen Verlauf und die Prognose der betroffenen Patienten äußerst entscheidend.

To report the visual outcomes and complications of automated anterior lamellar therapeutic keratoplasty (ALTK) in adults and children, and to examine these outcomes as a function of age and etiology.A consecutive series of cases undergoing automated ALTK procedures performed at the Jules-Gonin Eye Hospital Lausanne, Switzerland, between June 2003 and January 2015. Only patients with at least 3 months of follow-up were included.There were 53 eyes (24 right) of 51 patients (17 female, 16 juvenile), with a mean age of 34.8 years (range from 3 months to 88 years), analyzed. The mean follow-up was 35 (± 26) months. Diagnosis in the adult (n = 37) vs. juvenile (n = 16) eyes was different: opacity following surgical complication 8 vs. 0, congenital 1 vs. 1, dystrophy 5 vs. 2, infection 12 vs. 5, keratectasia 3 vs. 0, trauma 7 vs. 0, tumor 1 vs. 3, and allergy 0 vs. 5. Visual impairment as a consequence of corneal scarring was the principle indication for surgery in both adult (70%; 26) and juvenile eyes (63%; 10); other indications were choristoma, dermoid, other tumors, astigmatism, and congenital opacity. In adult vs. juvenile eyes, the mean visual acuity (spectacle and contact lenses) was, at last visit, 0.55 vs. 0.45 LogMAR (p = 0.78), with a range of 100% to hand movements. Failure occurred in 6 (16%) vs. 2 (13%) cases and complications were observed in 14 (38%) vs. 9 (56%) cases, however, more surgical revision was required in juvenile eyes, 4 (11%) vs. 7 (43%) (p = 0.01, Fisher test).This study shows that anterior lamellar keratoplasty in children retains good visual function when combined with adequate amblyopic therapy. However, the rate of complications is higher in juveniles and requires more intensive interdisciplinary follow-up.Der Visus und die Komplikationen nach automatisierter anteriorer lamellierender therapeutischer Keratoplastik (ALTK) bei Kindern und Erwachsenen wurden ausgewertet in Abhängigkeit von Alter und Ursache.Konsekutive Fallserie nach automatisierter ALTK, durchgeführt im Jules Gonin Augenspital Lausanne, Schweiz, zwischen 01.06.2003 und 01.01.2015. Nur Patienten mit einem Verlauf von mindestens 3 Monaten wurden eingeschlossen.53 Augen (24 rechte) von 51 Patienten (17 Frauen, 16 Kinder unter 16 Jahren) mit einem Durchschnittsalter von 34,8 Jahren (von 3 Monaten bis 88 Jahren) wurden analysiert. Das Follow-up betrug 35 (± 26) Monate. Die Diagnosen in adulten vs. juvenilen Augen waren verschieden: Trübungen infolge chirurgischer Komplikation 8 vs. 0, kongenital 1 vs. 1, Dystrophie 5 vs. 2, Infektion 12 vs. 5, Keratektasie 3 vs. 0, Trauma 7 vs. 0, Tumor 1 vs. 3 und Allergie 0 vs. 5. Visusminderung als Konsequenz einer Hornhautnarbe war die Hauptindikation für den Eingriff in beiden Gruppen, adulte (70%; 26) und juvenile Augen (63%; 10), andere Indikationen waren Choristom, Dermoid, andere Tumoren, Astigmatismus und andere kongenitale Trübungen. Der Durchschnittsvisus bei der letzten Konsultation in adulten vs. juvenilen Augen war 0,55 vs. 0,45 logMAR (p = 0,78), Bandbreite von 100% bis Handbewegungen, Versagen kam in 6 (16%) vs. 2 (13%) der Fälle vor und Komplikationen wurden in 14 (38%) vs. 9 (56%) der Fälle beobachtet, jedoch wurden mehr chirurgische Revisionen in juvenilen Augen benötigt (4 [11%] vs. 7 [43%]) (p = 0,01, Fisher-Test).Diese Studie zeigt, dass die anteriore lamellierende Keratoplastik zu einer guten Sehfunktion bei Kindern führt, falls sie kombiniert wird mit einer adäquaten Amblyopietherapie. Jedoch ist die Komplikationsrate höher als bei Erwachsenen und benötigt eine intensivere interdisziplinäre Behandlung.

Corneal topographs that measure the anterior and posterior corneal surface with optical methods need a clear cornea for precise measurements. Opacities cause artifacts in the corneal thickness (with measurements usually being too thin) and corneal curvatures. This is important to know as certain pathologies may repeatedly cause similar artifacts. This is highly relevant after a corneal cross-linking, Lasek or PRK, as these procedures cause typical artifacts that can easily be misinterpreted.

BACKGROUND: The aim of this study was to evaluate different methods of intraocular lens (IOL) implantation and posterior capsule management in the absence of vitrectomy with respect to visual axis opacification (VAO) in children. PATIENTS AND METHODS: Seventy-three eyes of African children undergoing cataract extraction and IOL implantation between 1998 and 2001 were evaluated. In all eyes, the IOL haptics were placed in the bag and the vitreous was preserved. The IOL optic and posterior capsule were managed in the following 3 ways: The IOL optic was captured behind the posterior capsule in conjunction with posterior capsulotomy (PC) in 47 eyes (group 1). The IOL optic was implanted in the bag with PC in 14 eyes (group 2) and without PC in 12 eyes (group 3). Cox proportional hazard analysis and Kaplan-Meier survival curves were performed to evaluate the incidence of Elschnig pearls (EP) and VAO. RESULTS: Elschnig pearls developed in 14.9 % of the patients in group 1, in 56.8 % in group 2 and in 91.7 % in group 3. The occurrence of EP depended significantly on optic capture (p < 0.001) and child’s age (p < 0.05), but not on PC (p = 0.084) and eye side (p = 0.1). The persistence of visual axis clarity depended significantly on optic capture (p < 0.001) but not on PC. CONCLUSIONS: In vitreous-sparing cataract surgery, posterior capsule opening does not effectively prevent VAO unless it is in conjunction with IOL optic capture.

Haze formation with loss of corneal transparency and surface irregularities and myopic regression are the major complications after corneal refractive surface surgery. The use of mitomycin C (MMC) with its antibiotic and antineoplastic properties is intended to inhibit wound healing mechanisms leading to subepithelial fibrosis. We report the use of MMC to achieve visual rehabilitation in the re-treatment of 3 eyes of 2 patients following refractive corneal surgery. According to the literature, the local use of MMC 0.02 % for 2 minutes is safe and enables one to treat and prevent stromal haze and myopic regression and allows a reduction of the postoperative topical pharmacotherapy. Results are still limited due to small case numbers and short follow-up periods.

The introduction of corneal cross-linking was associated with great expectations from patients as well as ophthalmologists. Previous results indicate that corneal cross-linking can slow down, stabilise or even reverse the progression of corneal ectasia in patients with keratoconus as well as in patients with ectasia after excimer laser ablations. We now describe our own experience with different protocols for corneal cross-linking and put these into perspective with the existing literature.We undertook a retrospective analysis of all of our corneal cross-linking treatments from January 2007 to July 2014 using different protocols. In addition, we provide an overview of the literature regarding complications of corneal cross-linking.In our patient cohort we observed sterile infiltrates, transient cloudy opacifications, diffuse lamellar keratitis and a mycotic infection leading to corneal scarring. Moreover, even after transepithelial corneal cross-linking with different protocols, we have observed pain perception and epithelial disruption. At present there are few reports about infections or endothelial damage in the literature, however, induction of neoplasia is described. The incidence of complications appears to be low.Corneal cross-linking appears to have a high success rate and a low complication rate. Endothelial damage seems to be a far smaller problem than initially suspected. However, prospective studies with long-term follow-up are still lacking and it remains to be seen whether more cases of neoplasia following cross-linking will be reported. In our literature research we found no reports on re-treatments. Similarly, we found no study other than some case reports on keratoplasty after cross-linking. In view of rapidly changing treatment protocols further trials are warranted to monitor the benefits and complications of these modifications.

Background A steroid-induced glaucoma may develop after bilateral laser in situ keratomileusis (LASIK) with normal intraocular pressure in applanation tonometry. Methods We present the case of a 32-year-old patient who underwent bilateral LASIK for myopia. Postoperatively, a steroid-induced glaucoma developed. After the steroid therapy was stopped applanation tonometry showed normal values. A slight corneal opacity was interpreted as a keratokonjunctivitis sicca because of occupational noxa. One year after LASIK, the patient presented with high intraocular pressure (IOP), maximally excavated optic nerve head and extensive visual fields defect in both eyes. Conclusion Elevated IOP after LASIK can lead to fluid accumulation in the interface. In this case applanation tonometry can underestimate the intraocular pressure. Even when steroid therapy is stopped, the elevated pressure can persist. This complication after LASIK is very rare and can cause severe damage if not diagnosed.

Many systemic lysosomal storage disorders show basic corneal opacities already in childhood. The lysosome is a cell organelle, produced by Golgi's apparatus, that is surrounded by a membrane and contains hydrolytic enzymes that break down food molecules, especially proteins and other complex molecules. The ophthalmologist's precise diagnosis of corneal clouding at the slit-lamp may reveal the correct interpretation of the specific lysosomal storage disorder. It is very important to diagnose such diseases as soon as possible because today the development of systemic enzymatic therapies has broadened the therapeutic armamentarium for the current standard of care. The following corneal landmarks of systemic storage diseases and of the modern systemic therapy are presented: cornea verticillata in Fabry's disease, periodic infusion of alpha-galactosidase a; Kayser-Fleischer's ring in Wilson's disease, zinc, trienetin, low copper diet; multiple, punctiform crystals in cystinosis, cysteamine, Raptor RP 103(DR cysteamine) that reduces the cytotoxity in form of continous dissolving of cystine from lysosome, renal transplantation, haematopoietic stem cell transplantation; peripheral ring, but not true lipid arc, and moderate stromal haze in LCAT-deficiency, injection of recombinant enzyme or of encapsulated LCAT-secreting cells; diffuse stromal haze in mucopolysaccharidoses (MPS). Enzyme replacement therapy is currently indicated for MPS I, MPS II, and MPS VI, haematopoietic stem cell transplantation; painful, bilateral pseudo-dendritic opacities in tyrosinemia type II (eponym: Richner-Hanhart syndrome), low phenylalanine and tyrosine diet result in complete disappearance of corneal alterations with a consecutive painfree period. Strict diet during the whole life is necessary to prevent corneal recurrences and the occurrence of palmo-plantar keratoses. Such therapies can enable the patient to lead an otherwise normal life for decades.

Many forms of the 25 corneal dystrophies (CD) manifest already in childhood with bilateral corneal opacities without any visual impairment in most cases. Pain attacks due to recurrent erosion with red eye and epiphora can occur in combination with the first dystrophy-specific corneal opacities, e.g., nest-like lattice lines + corneal erosion in lattice CD. Often we can observe a joint occurrence. The parents accompany their child to consult the ophthalmologist concerning the recurrent and joint pain attacks. The ophthalmologist can also diagnose in such a situation subtle corneal opacities on the painless, contralateral eye, e.g., those of lattice, granular, macular or Reis-Bücklers CD. The correct interpretation of this combination does not lead the ophthalmologist to an inflammatory-, but to a dystrophy-induced interpretation of this entity. In a further group of CD, e.g., in Franceschetti CD, the typical history of this entity is a dominant disorder with recurrent epithelial erosions starting in the first decade, declining in frequency and severity at a later age, and associated with a central, disk-like haze of the subepithelial cornea from middle age. The recurrent erosions in childhood lasted 3-5 days and were followed by complete recovery. Congenital corneal clouding in the form of a milky ground-glass appearance can be observed in the rare endothelial CD, such as in congenital hereditary endothelial dystrophy 1 and 2 (CHED 1 and 2), X-linked endothelial CD (XECD), and in posterior polymorphous CD (PPCD). The differential diagnosis for congenital glaucoma is of essential importance. The parents of babies with congenital corneal opacification should be examined at the slit-lamp to in- or exclude the appearance of mooncrater-like endothelial changes.

BACKGROUND Toxic epidermal necrolysis (TEN) can lead to ocular surface scarring associated with pain, dry eye symptoms, and decreased visual acuity that often are difficult to treat. HISTORY AND SIGNS A 34-year-old woman was referred to our department two-years after TEN induced by lamotrigin (lamictal(R)). She was complaining of severe visual acuity loss, pain, and dry eye symptoms. Visual acuity was reduced to light perception in the right eye (RE) and to 0.2 in left eye (LE). Basal Schirmer test was 2 mm in RE and 3 mm in LE. With or without therapeutic contact lenses, the patient was experiencing severe discomfort requiring tear supplementation up to 50 - 80 times/day in both eyes. THERAPY AND OUTCOME A free autologous mucosal graft (3.5 x 2.0 cm) was transplanted from the lower lip into the upper RE fornix. Six months after surgery, with therapeutic contact lenses, the need for tear supplementation was markedly reduced to 3 - 4 times/day in RE while it remained unchanged in LE. In RE, slit-lamp examination revealed decreases in the corneal stromal edema and in the diameter of neo-vessels associated with an improvement of visual acuity (counting fingers at 30 cm). Basal Schirmer test values were unchanged. It has to be mentioned that the improvement observed after surgery was markedly dependent on wearing a therapeutic contact lens. CONCLUSIONS Free autologous buccal graft transplantation (with its presumably accessory salivary glands) in association with the use of a therapeutic contact lens can be an efficient approach to treat ocular complication following drug-induced TEN (Lyell's syndrome).

The aim of this study was to evaluate the incidence of posterior capsule opacification up to 50 months following 1.7-mm bimanual MICS-cataract surgery.Bimanual MICS cataract surgery was performed in 197 eyes (135 patients) via two 1.7-mm corneal incisions. Four MICS acrylic foldable IOLs were implanted: AcriSmart 48S-5, n = 54 (Acritec GmbH, Hennigsdorf, now AT.Smart 48S Carl-Zeiss-Meditec, AG, Jena, Germany), ThinLens UltraChoice 1.0, n = 53 (Technomed GmbH, Baesweiler, Germany), AcriFlex 46, n = 41 und AcriFlex 48 CSE, n = 7 (Acrimed GmbH, Berlin, now: Lentis L-303, Oculentis GmbH, Berlin, Germany) and CareFlex, n = 43 (w2o Medizintechnik AG, Bruchsal, Germany). Statistical analysis was performed using the Kaplan-Meier technique.High levels of completeness of follow-up rates were: ThinLens 96%, CareFlex 100%, AcriSmart 93%, AcriFlex 92%. The capsulotomy rate was 43.13% for ThinLens within a mean/max. follow-up period of 801/1131 days, 34.88% for CareFlex (565/872 days), 40% for AcriSmart (988/1506 days) and 15.91% for AcriFlex (728/975 days). By limiting the follow-up period to a comparable maximum of 850 days for all four IOLs, our capsulotomy rates were as follows: ThinLens 33.33%, CareFlex 32.56 %, AcriSmart 20.0% and AcriFlex 11.36%. MICS IOLs have higher capsulotomy rates than hydrophobic acrylic lenses and sharp-edged silicone lenses. In literature comparisons MICS-IOLs do not exceed the variance levels of capsulotomy rates of PMMA, hydrophilic acrylic and silicone lenses without sharp edges. Cases of decentration or luxation of MICS-IOLs following Neodym:YAG laser capsulotomy were not detected. Capsulotomy frequency with the CareFlex was statistically significantly higher in comparison to the AcriSmart (Log Rank Mantel Cox Test, p = 0.007) and AcriFlex (log rank Mantel Cox test, p = 0.002).Capsulotomy rates observed varied for the four MICS-IOL-types tested. The posterior capsule opacification frequency of the two best MICS-IOLs (AcriFlex, AcriSmart) did not exceed the higher variance levels of posterior capsule opacification rates of the round-edged "conventional" non-MICS IOLs of PMMA, silicone or hydrophilic acryl material. Sharp-edged silicone or hydrophobic acrylic "conventional" lenses have shown lower posterior capsule opacification rates.

PURPOSE: We report the clinical, morphological, and ultrastructural findings of 13 consecutively explanted opacified Hydroview(R) (hydrogel) intraocular lenses (IOLs). Our purpose was to provide a comprehensive account on the possible factors involved in late postoperative opacification of these IOLs. PATIENTS AND METHODS: Thirteen consecutive opacified hydrogel IOLs (Hydroview H 60 M, Bausch ; Lomb) were explanted due to the significant visual impairment they caused. The IOLs underwent macroscopical examination, transmission electron microscopy (TEM), scanning electron microscopy (SEM), energy-dispersive X-ray spectroscopy (EDS), and electrophoresis for protein detection. Three unused control Hydroview IOLs served for comparison. RESULTS: Macroscopical examination showed a diffuse or localized grey-whitish opacification within the IOL optic. TEM confirmed the presence of lesions inside the optic in all the explanted IOLs and revealed 3 patterns of deep deposits: a) diffuse, thick, granular, electron-dense ones; b) small, thin, lattice-like ones, with prominent electron-lucent areas; and c) elongated electron-dense formations surrounded by electron-lucent halos. SEM showed surface deposits on four IOLs. EDS revealed oxygen and carbon in all IOLs and documented calcium, phosphorus, silicon and/or iron in the deposits. Two of the patients with iron in their IOLs had eye surgery prior to their phacoemulsification. Iron correlated well with the second TEM pattern of deep lesions, whereas calcium with the third TEM pattern. No protein bands were detected on electrophoresis. Control lenses did not show any ultrastructural or chemical abnormality. CONCLUSIONS: The present study supports the presence of chemical alterations inside the polymer of the optic in late postoperative opacification of Hydroview IOLs. This opacification does not follow a unique pathway but may present under different ultrastructular patterns depending on the responsible factors. Mechanical stress during surgery may initiate a sequence of events where ions such as calcium, phosphorus, silicon, and/or iron, participate in a biochemical cascade that leads to gradual alteration of the polymer network. Intraocular inflammation due to previous operation may be a factor inducing opacification through increase of iron-binding capacity in the aqueous humour. Calcification accounts only partially for the opacification noted in this type of IOL.

A 45-year-old man with Fabry's disease is presented. 27 relatives are examined. Among the different examinations (serum activity of alpha-galactosidase, urinary excretion of trihexosyl ceramide, renal function, ocular examination) ocular examination remains the easiest and cheapest test for detection of heterozygotes. The ocular manifestations of this enzymatic defect are reviewed.

An unusual bilateral ring-shaped corneal opacity has been observed in two patients. Biomicroscopical characteristics are minute greyish dots which are distributed within the outer stromal layers forming an arcus lipoides-like circular band. Besides of a typical arcus senilis no other corneal anomalies can be found. Up to date only 4 patients with similar corneal opacities have been described. Etiology remains unclear and according to the author who has first described this symptom we propose to name this corneal anomaly "Ascher-Ringe".

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.

Amiodarone, one of the most effective anti-arrhythmic drugs, is also known for its ability to accumulate lipid-pharmakon complexes in the lysosomes of different tissues. In the eye the lysosomal storage leads to typical side-effects. Whorl-like epithelial, reversible corneal inclusions occur in about 70 to 100 % of the patients on amiodarone therapy. Tiny lens opacities without visual impairment have been reported in 50 % of patients who had been treated with amiodarone. At present the most severe complication of amiodarone is optic neuropathy with an incidence of 1.3 to 1.8 %. The optic neuropathy, as the rule, is only reversible approximately in (1/2) of the patients after discontinuing the drug. The fundoscopic picture of amiodarone neuropathy is similar to classic AION. Retinal involvement has also been reported; however, a relationship with amiodarone has not been proven yet.

Haze formation with loss of corneal transparency and surface irregularities and myopic regression are the major complications after corneal refractive surface surgery. The use of mitomycin C (MMC) with its antibiotic and antineoplastic properties is intended to inhibit wound healing mechanisms leading to subepithelial fibrosis. We report the use of MMC to achieve visual rehabilitation in the re-treatment of 3 eyes of 2 patients following refractive corneal surgery. According to the literature, the local use of MMC 0.02 % for 2 minutes is safe and enables one to treat and prevent stromal haze and myopic regression and allows a reduction of the postoperative topical pharmacotherapy. Results are still limited due to small case numbers and short follow-up periods.

It is a challenge to prevent irreversible amblyopia in infants suffering from Peters anomaly. In some cases of centrally located corneal opacifications an optical sector iridectomy can not lead to a clear optical axis. The homologous penetrating keratoplasty as early surgical procedure has shown an extremely poor outcome with a high risk of irreversible graft failure. We report on the autologous ipsilateral rotating penetrating keratoplasty in an eight-week-old infant suffering from Peters anomaly.An autologous ipsilateral rotating penetrating keratoplasty was performed in an eight-week-old infant suffering from Peters anomaly to prevent irreversible amblyopia.After a follow-up time of 8 months we saw a clear graft within the optical axis without any complications in wound healing. We removed the single sutures two months after keratoplasty. Postoperative astigmatism could be corrected first by fitting a special nursery contact lens and after reduction of astigmatism because of suture removal we fitted special nursery glasses. The intraocular pressure remained within the normal range during the follow-up period.The autologous ipsilateral rotating penetrating keratoplasty should be considered superior to homologous keratoplasty in infants with Peters anomaly if sector iridectomy is not advisable because of a central corneal opacification. Resulting high refractive errors can be successfully corrected by special contact lens fitting or by nursery glasses.

The authors report a case of a bilateral peripheral lipid keratopathy, rising differential diagnostic points as well. After several year-long follow-up period the peripheral corneal opacity left the center unaffected. The diagnosis was confirmed by tear examinations which disclosed elevated level of fatty and phosphorous content compared to healthy control eyes.

Bilateral stromal corneal opacifications are important to detect potentially associated systemic diseases. Differential diagnosis includes, besides inflammatory diseases mucopolysaccharidoses, HDL deficiencies (LCAT deficiency, Tangier disease and fish eye disease), Schnyder's crystalline corneal dystrophy, lipid keratopathy, cystinosis, gout and mucolipidoses.The cornea of a 60-year old male patient, 180 cm height, showed milky hazy diffuse stromal inclusions with a prominent lipoid arc (VA O. D. 20/30 and O. S. 20/40, contre-jour VA less than 20/150). Ten years earlier, lattice corneal dystrophy was diagnosed. The cornea was thickened (0.61 mm). Except for the cornea, the anterior and posterior segments of the eye were unremarkable. Serum HDL concentration was decreased pathologically (7 mg/dl), although the LCAT activity was combined with apo-AI deficiency. Due to membrane instability, the erythrocytes showed target cell configuration. Penetrating excimer-laser keratoplasty was performed O. S. The epithelial wound closed only on the 32nd postoperative day. Histologically, the corneal stroma showed multiple vacuoles and amyloid deposits. Genetic analysis revealed two mutations in chromosome 16.To our knowledge, this is the second description in the literature of a patient with LCAT deficiency and secondary amyloidosis of the cornea. Additionally, LCAT deficiency is associated with anaemia and proteinuria. After a penetrating keratoplasty, prolonged wound healing is possible. Because of the bilateral corneal clouding, the ophthalmologist may help to identify patients with LCAT deficiency. Thus, it is possible to start antiarteriosclerotic therapy.

Postoperative bacterial endophthalmitis are caused in 80 % of the cases by the patient's own flora. Most of the time, bacterial agents are Gram-positive ((2/3) of cases) and more rarely Gram-negative ((1/3) of cases). Usually, Pseudomonas sp, Proteus sp or Klebsiella sp are isolated, but very rarely Morganella morganii.We describe a case of a Morganella morganii endophthalmitis which occurred after a vitrectomy.Bacterial examinations disclosed the presence of Morganella morganii in the vitreous. An aggressive treatment (intravitreous [ceftazidim, vancomycin], topical [gentamycin, chloramphenicol] and intravenous [imipenem, ofloxacin] antibiotics) was introduced. In spite of this treatment, the outcome was not favorable.Post-vitrectomy endophthalmitis is very rare and the isolation of a Gram-negative bacteria, in this case Morganella morganii, is infrequent. The outcome of these infections is often poor despite the introduction of a rapid, specific and aggressive treatment.

A 73-year old man presented with a bilateral reduction of visual acuity which had slowly occurred over the last 20 years. Ophthalmologic examination revealed the presence of stromal opacifications which were denser in the central cornea associated with cataractous changes. His general history was remarkable for a status post polio infection in the early childhood and a medically treated arterial hypertension. We performed a triple procedure and histologic examination of the corneal button revealed the presence of a lipid keratopathy.Lipid keratopathy is mostly caused by corneal vascularization or chronic inflammation or is a consequence of systemic disorders of lipid metabolisms. Our patient did not exhibit these underlying disorders and therefore we made the diagnosis of a primary lipid keratopathy. The underlying corneal pathomechanisms are incompletely understood but they should be limited to the corneal keratocytes.

Bilateral stromal corneal opacifications are a differential diagnostical challenge to identify associated systemic diseases.A 47-year old civil engineer (height 167 cm) with bilateral stromal corneal clouding presented with visual loss for the last 27 years: VA 20/100 OD and 20/50 OS (following penetrating keratoplasty OS). The cornea showed milky-whitish, cloudy, diffuse stromal deposits without a separate lipoid arc. The posterior segment showed tapetoretinal degeneration. Scotopic ERG was decreased. A suspicious stiffness of interphalangeal joints on both hands was observed. There was an aortic and mitral insufficiency grade I. Serum levels of LDL, HDL and triglycerides were normal. The biomicroscopical diagnosis of Scheie's syndrome (mucopolysaccharidosis I-S) was confirmed by a deficiency of alpha-L-iduronidase in leukocytes (0.02 nmol/min/mg protein, normal range: 0.3 - 1.5).The differential diagnosis of bilateral corneal stromal opacification includes in addition to the mucopolysaccharidoses HDL-deficiency diseases (LCAT deficiency, Tangier disease, Fish eye disease), Schnyder's crystalline stromal dystrophy, cystinosis, gout and mucolipidoses. MPS I-S may easily be detected by alpha-L-iduronidase deficiency in leukocytes and increased mucopolysaccharides in the urine. Furthermore, patients with MPS I-S need general medical care because of cardiovascular abnormalities, joint stiffness and myopathies.

Corneal haze is a severe complication after excimer laser PRK and may lead to a compromised visual performance and regression. Currently, corticosteroids are topically applied to inhibit the appearance of haze. However, this therapy is not always successful in preventing haze. Various animal studies have shown that topical applied mitomycin C reduces keratocyte activity and collagen synthesis and subsequently haze.We have treated 5 patients (5 eyes) with severe haze and regression after PRK. In all eyes we have mechanically debrided the haze and 0.02 % mitomycin C was applied to the area of previous ablation for 1 minute. In 2 eyes we have only performed the debridement, whereas in 3 eyes we also have performed a re-PRK after the laser ablation the mitomycin was applied. The postop follow-up was 9 months.In all eyes the surgery and the postop time was without any complications. The haze could be removed in all eyes completely and in the entire postop period recurrent haze was not more than trace. Regression could be eliminated in the three eyes with a re-PRK, whereas the 2 eyes without re-PRK still remained myop. We could not observe any mitomycin C associated complications on the conjunctiva or cornea.Topical applied mitomycin C seems to be a promising therapy to prevent recurrent haze after a second PRK procedure. As mitomycin C can be associated with a variety of serious complications, it should only be used in patients with high risk of haze.

A 63-year-old man without complaints presented with a bilateral symmetric opacity of the cornea between the mid and the outer periphery which was classified as Ascher's ring.Ascher's ring is a very rare entity. Characteristic slit lamp features allow its differentiation from other anular corneal opacities. Corneal or systemic alterations of the lipid metabolism can be discussed. Heredity can be suspected but could not be proved as yet. The visual prognosis is excellent. Therapy is not necessary.

We report the case of a 14 year old girl who obtained simultaneous penetrating keratoplasty for corneal opacity with functional loss as a consequence of a mucopolysaccharidosis typ Hurler/Scheie. Postoperatively, the host cornea displayed partial clearing. We discuss mechanisms possibly involved.

Central corneal pathologies can lead to an irreversible decrease of best corrected visual acuity if not diagnosed and treated appropriately. This article reviews the differential diagnosis of central corneal opacities in the newborn, of central infectious corneal ulcers, and the therapy of sterile, central keratolysis.Authors' personal experience and review of the literature.Flow charts for diagnosis and treatment strategy have been elaborated.Corneal opacities in newborns create an emergency situation. In order to treat successfully and avoid or diminish amblyopia, it is imperative to rule out congenital glaucoma. The aetiology of central corneal ulcers should always be confirmed by positive cultures to be able to treat specifically. When the standard topic therapy fails, one has to consider rare bacteria, parasites, virus, or patients' compliance. The treatment of central sterile keratolysis in rheumatoid arthritis must be intensive and immunosuppression has to be performed early enough in the course of prevent the formation of a descemetocoele or spontaneous corneal perforation.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Ocular involvement has been reported in about 25% of patients (1). Band keratopathy is rare in sarcoidosis.A 31 year-old clerk presented to our clinic with a 10 months' history of foreign body sensation OU. Visual acuity was OD1.2, OS 1.0. Intraocular pressure was 10 mm Hg OU. We found a moderate band keratopathy OU (Fig. 1a, 1b). The rest of the ocular exam was unremarkable. 10 months before, the patient had experienced symmetrical swelling of his large joints and his creatinine and calcium levels had been elevated at that time. Therefore, we referred the patient to the internist to rule out primary hyperparathyroidism (2). The differential diagnosis included myeloma, paraneoplasia and sarcoidosis (3). The general medical examination was unremarkable. Calcium (2.5 mmol/l), creatinine (2.0 mg/dl) and ACE-levels (177 U/ml) were elevated, parathormone (1.4 pg/ml) was low. The chest x-ray showed reticular, small-nodular changes of both lungs. Pulmonary function tests and the bronchial mucosa were normal. The CD4/CD8-ratio was markedly elevated. Ultrasonography of the kidneys suggested nephrocalcinosis. This was confirmed by kidney biopsy. Histologic findings were consistent with the diagnosis of sarcoidosis. After initial symptomatic reduction of calcium levels by forced diuresis, calcium and creatinine levels were lowered by administration of 60 mg prednisolone daily. Six months after presenting to our clinic the patient is well on low-dose steroids and his calcium levels are normal.Primary hyperthyroidism was ruled out by low PTH-levels. Because of clinical, serologic and radiologic findings suggestive of sarcoidosis, bronchoscopy with bronchoalveolar lavage was performed. Since the CD 4/CD 8-ratio was increased, a kidney biopsy was performed which confirmed the diagnosis.

It was the purpose of this prospective study to evaluate the effect of topical Ciclosporin 2% on epithelial and subepithelial opacities in Thygeson's superficial punctate keratitis.We administered Ciclosporin A 2% eye drops to 31 eyes of 17 patients with Thygeson's superficial punctate keratitis. The initial dose was 3 drops daily during the first month. It was reduced according to the following scheme: 2 drops in the second month, 1 drop in the third month, 1 drop every second day in the fourth to sixth months. Thereafter therapy was stopped.The opacities were suppressed completely in 21/31 eyes mostly after 4 weeks of therapy and incompletely in 8/31 eyes. Of the 21 eyes with complete suppression, 6/21 showed recurrent opacities when therapy was stopped after six months. 7/21 eyes had no recurrences after cessation of therapy. Follow-up of 8 eyes is currently less than 6 months and these eyes still receive Ciclosporin A 2% eye drops. All patients complained of a slight burning caused by the drops.In the majority of patients with Thygeson's superficial punctate keratitis topical Ciclosporin A 2% suppresses epithelial and subepithelial opacities as long as this non-steroid therapy is administered at a low dose. In a considerable percentage therapy seems to be curative.

The penetrating keratoplasty as an early surgical procedure to present deep irreversible amblyopia in patients suffering from Peters' anomaly has shown an extremely high risk for transplantat failure.From 1980 to 1992 20 sectoriridectomies (6 bilateral, 1 twice because of progressive corneal opacification in the area of sectoriridectomy) were performed in 13 patients with Peters' anomaly (diameter of corneal opacification greater than half of the corneal diameter). The mean age at surgery was 1 year and 9 months, the mean follow-up was 3 years and 6 months. Preoperatively the visual function was uncertain light perception in all eyes with marked horizontal nystagm. Except for two eyes (24 mm Hg) the intraocular pressure (IOP) was normal.In 9 (47%) eyes the visual acuity was 20/500 to 20/200. One eye showed certain light fixation, one eye hand movement. In one eye the visual function remained uncertain light perception "after combined lens aspiration." One eye developed a phthisis after combined anterior vitrectomy and lens aspiration for PHPV. In three eyes, the visual outcome could not be determined certainly, in three eyes, the follow-up failed. In two eyes the IOP was regulated under topical antiglaucomatous eye drops, in the other eyes the IOP was normal.In contrast to penetrating keratoplasty sectoriridectomy seems not to be followed by secondary glaucoma postoperatively. The visual outcome is comparable to that after early keratoplasty. We recommend "optical" sectoriridectomy as an alternative surgical approach to early penetrating keratoplasty in patients suffering from Peters' anomaly.

In recent years TPA (tissue-plasminogen activator) has been increasingly and successfully used for the treatment of severe, postoperative fibrin reaction in the anterior chamber. So far no serious side effects of this treatment have been reported.Altogether, 32 patients received 0.2 ml solution with 20 micrograms TPA intracamerally. In 2 cases a dense corneal opacity was observed 12-24 hours after the injection of TPA which was resistant to treatment with local dexamethasone and lubricants. Therefore it was removed by superficial keratectomy. In one case the keratectomy specimen could be examined by light- and electronmicroscopy.In the keratectomy specimen a selective, fine-granular calcification of Bowman's membrane could be demonstrated.The intracameral TPA treatment for postoperative fibrin reaction can cause a rapid band keratopathy. Therefore the application of TPA should be restricted to severe therapy-resistant cases of intracameral fibrin reaction. In cases with the development of a band keratopathy EDTA-treatment is recommended.

In patients with corneal opacity caused by diseases like okular pemphigoid, Stevens-Johnson-Syndrome or burns, visual rehabilitation can only be achieved with keratoplasty or keratoprosthesis. The first has generally a poor prognosis in these circumstances, and the latter bears several problems in the postoperative course as well. A biological, corneal replacement could possibly combine the advantages offered by keratoplasty (no interaction between host tissue and plastic) and keratoprosthesis (more permanent transparency). Theoretical considerations and practical experience suggest that the best candidate would be hyaline cartilage.Bovine sterni were used to obtain circular cartilage slices which were approximately 60 microns thick, having a diameter of approximately 6 mm. These slices were implanted in one eye each of 4 rabbits instead of the natural cornea. The eyes were enucleated after 8 to 29 days.The cartilage remained so transparent that the iris and to a certain extent the fundus blood vessels could be visualized. Connective tissue served to bind the host cornea to the cartilage, but did not infiltrate the cartilage. Perforation was observed in 3 animals. The extent of epithelialization of the cartilage slices ranged form ca. 30% to 95%. None of the animals exhibited an intraocular inflammation. The cartilage did not proliferate and remained avascular. Secondary intraocular changes, with the exception of anterior synechiae, could not be detected.A tissue for biological corneal replacement has to fulfill many requirements which to a certain extent can be achieved with the use of hyaline cartilage. Despite numerous, unsolved problems, a biological corneal replacement as an alternative to keratoplasty and keratoprosthesis in cases of corneal blindness does not seem out of imagination.

Sclerocornea is a congenital, uni- or bilateral, non-progressive, non-inflammatory malformation characterized by histological changes of the cornea resembling scleral tissue.We report the case of a 2-month-old child with bilateral dysplasia of the anterior segment including: 1. extreme corneal ectasia; 2. vascularized, opacified microcornea; 3. presence of rudimental limbus; 4. anterior scleral dysplasia. The axial length measured by means of ultrasonography (A-scan) was about 24 mm in both eyes. A penetrating keratoplasty was first performed in the right eye and one week later in the left eye. Intraoperatively an advanced iris atrophy with extensive anterior synechiae, as well as a localized anterior subcapsular cataract were seen bilaterally. A pale optic disk with a maximally enlarged optic cup could be seen only in the left eye.Although the histologic examination was compatible with the diagnosis of sclerocornea, the presence of atypical clinical findings previously unreported does not allow a definite classification of the case reported herein.

The reasons for corneal graft failure during the first postoperative year were examined.The functional and morphological parameters were recorded prospectively on 210 eyes with penetrating keratoplasty postoperatively after 1, 4 and 12 months.1 month postoperatively one graft, 4 months after surgery 5 grafts, and 12 months after keratoplasty 15 grafts had failed. The reasons were: 3 cases of irreversible rejection, 4 retrocorneal membranes, 4 persisting corneal erosions with ulceration, and 4 stromal infections (3 Herpes, 1 Akanthamoeba). During the first postoperative year, in our patients corneal graft rejection was a rare event accounting for 3 of 15 graft failures with an incidence of 1.4% in all grafts studied.

We want to present a 47-years-old patient suffering of a histologically and biochemically confirmed M. Fabry. Besides the ocular findings, we list the systemic findings and discuss the diagnostic and therapeutic possibilities. We especially want to point out the interdisciplinary collaboration in dealing with this condition.

Recurrences of granular corneal dystrophy (Groenouw type I dystrophy) after penetrating keratoplasty have been often reported in the literature. Up to now the therapy of these recurrences consists in performing a lamellar or penetrating re-keratoplasty. As those recurrent opacities at first are located in the area of Bowman's membrane and the superficial corneal stroma, a phototherapeutic keratectomy (PTK) with an excimer laser offers a therapeutic alternative to re-keratoplasty.We describe three eyes of two patients with recurrent opacities on the transplant after successful keratoplasty because of granular corneal dystrophy. In all eyes we performed an excimer-laser PTK (Keratom of Schwind Company, Kleinostheim/Germany, wave length 193 nm) after abrasion of the epithelium with a hockey-knife. The depth of ablation depended on the location of the opacities and was between 7 microns and 35 microns. During the excimer-laser treatment several biomicroscopical slit-lamp controls were performed to make sure whether a sufficient depth of ablation was obtained.The opacities could be totally removed in all eyes by excimer-laser PTK. Postoperatively the visual acuity increased and the patient's photophobia decreased. At the same time a slight progression of hyperopia developed depending on the depth of ablation. During the follow-up period of 5 to 12 months no recurrent opacities could be found until now.Phototherapeutic keratectomy with the excimer laser seems to be a good alternative to re-keratoplasty in superficially located recurrences of granular corneal dystrophy in a graft after keratoplasty. A slight progression of hyperopia depending on the depth of ablation has to be accepted. The future will show whether there will develops recurrent opacities of the dystrophy after an excimer laser treatment.

Vitamin A, the fat soluble vitamin, must be supplied orally before resorption in the mucosa of the small intestine and storage in the liver. Vitamin A deficiency can cause alterations in the anterior segment of the eye, from Bitot spots, which are reversible, to irreversible keratomalacia.5 patients suffering from manifestations at the cornea underwent ophthalmological, general and dermatological investigation and measurements of the vitamin A, retinol-binding protein and, in one patient, zinc were performed. 4 patients suffered from cornea manifestations, reaching from almost a reactive ulceration to spontaneous perforation. One patient had relapsing episcleritis. All patients were alcoholics, had hepatopathies and dermatological diseases. Vitamin A and retinol-binding protein were decreased and in one patient a highly decreased zinc was measured. In one patient a systemical and local substitution of vitamin A increased the clinical findings significantly. Two eyes had to undergo a keratoplasty à chaud, one evisceration had to be done.Vitamin A deficiency can be the reason for bilateral painless cornea manifestations. An interdisciplinary cooperation is essential for the elaboration of the diagnosis and the treatment.

Myopic PRK (Photorefractive Keratectomy) has been performed on 116 eyes (myopia of between -3.0 D and -25.5 D) in the eye clinic of Lucerne, since october 1990. 25 patients had both eyes treated, 7 eyes were retreated.Results are satisfactory for myopia of up to -10 D (-15 D); treatment of the higher myopia is problematic.Regression was the biggest problem in all patients, being the more pronounced the higher the ablation. Based on this experience recommendations for treatment are given.

The results of penetrating keratoplasty in patients with systemic mucopolysaccharidosis (MPS) type VI-A are controversial. Clouding of the transplant was often observed and was thought to be related to storage of glycosaminoglycans also in the donor button.We performed penetrating keratoplasties successfully in three children with MPS VI-A (severe type) at the age of 7-11 years.The transplants remained clear during the follow-up of 2.5-5 years and the longterm visual acuity was encouraging. In two patients we could observe a partial, circular clearing of the host's cornea adjacent to the transplant. The related pathomechanism and the clinical, histopathological and ultrastructural findings of the cornea will be discussed.As the intellectual development is normal, decrease of vision impairs the patient's life extremely. Thus, the indication of penetrating keratoplasty should be made early to improve the patient's quality of living, who have a reduced life-span.

The potential benefits of anterior segment surgery to achieve visual rehabilitation in severely compromised eyes subsequent to infectious complications in the anterior segment remain a subject of controversy.Anterior segment reconstructive surgery was performed on twelve patients (12 eyes) with infectious adherent leukoma and extensively damaged intraocular structures. Nine patients (nine eyes) were followed up over a period of two years (20-26 months) and three over a period of nine months (8-11 months).Simultaneous and consecutive surgical interventions including corneal grafting, anterior chamber reconstruction and posterior chamber lens implantation combined with antimicrobial and antiviral prophylaxis as well as corticosteroid treatment resulted in visual recovery in nine eyes, and in four in a visual acuity of 0.4 or better (reading VA).Surgery for optic reasons due to infectious adherent leukoma does not seem to be completely unsuccessful with respect to the low complication rate despite proven retrocorneal membranes in this study.

The time for recovery of recognition of an optotype presented at a distance of 40 m subsequent to exposure to a flash gun (Eso company, Tettnang; flash energy 200 Ws, duration 1/1000 s; distance 10 m) was studied on 97 subjects aged 20 to 83 years. A Landolt-ring (contrast 1:5, diameter 29 cm), mounted on a disk (diameter 58 cm) which could be rotated in steps of 45 degrees served as a test target. The flash gun could be dimmed down by a red filter (Schott RG 665). The tests were performed at night without additional illumination except the low beam of the car. The flash gun was foveally fixated. After a white flash, a period of 93.1 s +/- 48.6 s (MEAN +/- SD) passed before the optotype could be recognized correctly again. By adding the red filter in front of the flash gun the recovery time could be shortened significantly (p0.0001; Wilcoxon signed-rank test) to 4.9 s +/- 1.7 s. "Time for re-adaptation" after dazzling by red flash is comparatively short and therefore strongly influenced by many variables (e.g. time for recognition and for verbalization). Because of that a final evaluation of dazzling by red flash seems to be impossible with this method. Subjects of advanced age and opacities of the ocular media showed an increase in the "time for re-adaptation" for the white as well as the red flash. In conclusion, a speed control using white flash guns at night can be considered as critical.

The authors report on the Phototherapeutic 193nm-Excimer Laser Keratectomy (PTK) for the treatment of superficial corneal opacities.In two cases with corneal opacities after contact lens wearing and after therapy resistant keratoconjunctivitis epidemica respectively the treatment strategy is presented. The epithelium and the corneal opacities are removed mechanically as far as possible. Then the Excimer laser smoothing with masking fluids of different viscosity is performed.In both patients the visual acuity improved from 1/50 and 0.16 respectively to 0.7. Both eyes had an hyperopic shift. The patients were without pain within a follow-up period of 8 months after treatment.The phototherapeutical use of the 193nm Excimer laser to treat superficial corneal opacities can be regarded as a real alternative to conventional surgical techniques.