[What can anti-VEGF therapy achieve in clinical routine? : Effectiveness of anti-VEGF therapy in patients with macular diseases in clinical routine on 1492 eyes in Austria]. / Was bringt die Anti-VEGF-Therapie im Klinikalltag? : Wirksamkeit von Anti-VEGF-Therapie bei Patienten mit Makulaerkrankungen in der Klinikroutine an 1492 Augen in Österreich.

BACKGROUND: The aim of this user observational study was to illustrate the effectiveness of intravitreal operative medication administration (IVOM) in the clinical routine. DESIGN: A monocentric, single arm, prospective study. MATERIAL AND METHODS: Included in this study were all patients in whom a macular edema was diagnosed during an ophthalmological examination at the Barmherzigen Brüdern Hospital in Linz from November 2012 to August 2014 and who were treated as outpatients or day clinic patients with IVOM using anti-vascular endothelial growth factor (VEGF). The parameters measured during the clinical routine were as follows: best corrected visual acuity (BCVA) using the early treatment diabetic retinopathy study (ETDRS) chart, central optical coherence tomography (OCT) thickness, diagnosis and possible prior treatment with IVOM. Following diagnosis, the IVOM was administered in 6­week intervals and a final control with measurement of the named parameters (approximately 19 weeks after diagnosis). If edema was present a further 3­stage series (19 weeks) was initiated. In the case of a "dry" condition the further controls were performed by a private practitioner and the patient only presented again if macular edema reoccurred. A control of the patients with "dry" results at regular intervals was not planned. RESULTS: A total of 1492 eyes from 1184 patients with an average age of 75.6 ± 11.3 years were included in the study according to the abovenamed criteria. Choroidal neovascularization (CNV) with age-related macular degeneration (AMD) was diagnosed in 879 eyes and 314 eyes were treated for diabetic macular edema (DME). Of the eyes 122 (8.2%) were affected by branch vein thrombosis (AVT) and 63 (4.2%) from central vein thrombosis (CVT). In 47 (3.2%) eyes macular edema with myopic CNV (mCNV) was detected and 67 eyes (4.5%) were not further classified under "others". In all groups a gain in letters could be achieved in the EDTRS chart even after the first IVOM series: AMD + 3.4, DME + 1.3, AVT + 6.1, CVT + 10.1 and mCNV + 7.0. Patients who were treated with IVOM for the first time showed on average a better response than those previously treated with IVOM. CONCLUSION: As in many other studies our data also underline the necessity for intravitreal injections for treatment of macular edema. Even if a 6­week interval does not nowadays correspond to the current guidelines, we could achieve a stabilization of vision and do not shy away from comparison with large studies, such as the Marina study. In order to provide the increasing numbers of patients the best treatment in the future, an attempt at targeted implementation of resources must be made and if necessary symptoms which can be conservatively treated should be delegated to the private sector.

[Clarithromycin therapy of a B cell MALT lymphoma]. / Clarithromycin-Therapie eines B-Zell-MALT-Lymphoms.

Presentation of a 34-year-old patient with a conjunctival tumor of the left upper circumference of the eyeball clinically presenting as a salmon-pink colored conjunctival tumor. The incisional biopsy and immunohistochemical work-up revealed an extranodal conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma of the marginal zone B cell type. The therapy comprised a currently experimental therapy scheme with 500 mg clarithromycin 3 times per day for 21 days. This therapy achieved a significant tumor regression.

[Individualization of IOL constants for two hydrophobic intraocular lenses. SRK II, SRK/T, Hoffer-Q, Holladay 1 and Haigis formula]. / Individualisierung der IOL-Konstanten für 2 hydrophobe Intraokularlinsen. SRK II, SRK/T, Hoffer Q, Holladay 1 und Haigis-Formel.

AIM: The aim of the study was an assessment of refraction error after implantation of two types of hydrophobic acrylic intraocular lenses and derivation of customized IOL constants for the SRK II, SRK/T, Hoffer Q, Holladay 1 and Haigis formula. PURPOSE: The purpose of the present study was to analyze the refractive outcome of two hydrophobic acrylic intraocular lenses and to present a computerized calculation scheme for customization of lens-specific parameters provided by the lens manufacturers based on the refractive results of a surgeon or study center. METHODS: In this prospective monocentric study 100 consecutive cataract eyes were treated at the Eye Hospital of the Barmherzige Brüder (Compassionate Brothers) in Linz/Austria with a hydrophobic acrylic aspherical intraocular lens (Polytech Y10AS, n = 50) or a hydrophobic acrylic spherical intraocular lens (Hoya PC-60R, n = 50). The biometrical data were assessed preoperatively together with the refractive outcome 8-10 weeks after treatment in order to analyze the deviation of postoperative refraction (spherical equivalent) from target refraction (ΔREF) and to customize the lens constants for the SRK II, SRK/T, Hoffer Q, Holladay 1 and Haigis formula. RESULTS: Based on this data set it could be demonstrated that using the lens constant provided by the manufacturer (ΔREF) the Polytech lens showed a systematic trend to myopia in contrast to the Hoya lens which ranged around zero. This trend could be compensated by selecting appropriate lens specific constants. For the Polytech/Hoya lens median lens constants of: A = 118.0/118.6 (SRK II), A = 117.9/118.6 (SRK/T), pACD = 4.8/5.1 (Hoffer Q), SF = 1.1/1.5 (Holladay I) and d = 4.2/4.6 (Haigis) were extracted and using linear regression a lens constant triplet for the Haigis formula of a(0) = 4.39, a(1) = 0.29, a(2) = 0.11/a0 = 4.73, a(1) = 0.30, a(2) = -0.01 could be derived. CONCLUSIONS: Ophthalmic surgeons or surgical centers are encouraged to check (refractive) outcomes after cataract surgery permanently in terms of quality control. If a systematic trend in ΔREF could be extracted from the data set a customization of the lens-specific constants is recommended to eliminate systematic errors. Such customization is only valid for a dedicated environment (e.g. for one surgical center with standardized surgical techniques and measurement equipment) and cannot be generalized.

[Bilateral hyposphagma]. / Bilaterales Hyposphagma.

We present the case of an 85-year-old woman who presented with red eyes bilaterally. The right eye had been red for 2 months, and the left one for a shorter time. The ophthalmologic examination showed severely congested episcleral vessels in both eyes, reduction of visual acuity, hyperemia of iris vessels, and pigment epithelium detachment. Ocular tension was within normal range. Both eyes showed impaired ocular movement, and the left eye had an abducens paresis. Our strong suspicion of carotid-cavernous sinus fistula was confirmed through a computed tomographic perfusion study; the previously conducted magnetic resonance examination of the cerebrum was without result.

[Ocular toxoplasmosis and toxocariasis in childhood]. / Okuläre Toxoplasmose und Toxocariasis im Kindesalter.

Toxoplasmosis and toxocariasis are parasitic infections that are transmitted by cats and dogs, respectively, to humans, and which may induce posterior uveitis already in childhood. Toxoplasmosis presents as a congenitally or postnatally contracted infection whereas toxocariasis is always an acquired disease. The typical ocular sign of toxoplasmosis is retinochoroiditis, occurring as an active lesion, in most instances, associated with an inactive pigmented scar. In contrast, toxocariasis leads to a choroidal granuloma secondarily involving the retina or an endophthalmitis-like picture. Although toxoplasmosis represents the most common cause of posterior uveitis, there are uncertainties regarding the timing and specificity of the diagnosis, namely in atypical cases and those at risk of permanent severe loss of function. Antiparasitic treatment should be tailored to the severity of the inflammation and the risk of visual function loss. Concomitant steroids may be used to control the sequelae of unspecific inflammation, but should be used with caution and must be combined with an antimicrobial regimen. Because it is a rare disorder, one may not be familiar with the clinical presentation and suggested therapy for ocular toxocariasis. With this survey we, therefore, wish to provide a current, practice-oriented overview on the infection, ocular manifestations, diagnosis and treatment of ocular toxoplasmosis and toxocariasis in childhood.

[Evaluation of the residual refractive error of hydrophobic acrylic intraocular lenses]. / Evaluierung der refraktiven Treffsicherheithydrophober Acryllinsen.

BACKGROUND: The postoperative refractive status after cataract surgery is mainly characterised by the preoperatively determined intraocular lens (IOL) power. Beyond this scope we evaluated the refractive error of hydrophobic acrylic IOLs. PATIENTS AND METHODS: Standard phacoemulsification with implantation of hydrophobic acrylic IOLs ("yellow" HOYA AF-1 YA60BB n = 63, Alcon MA 60AC n = 52, AMO AR 40e n = 131) was performed in 246 consecutive patients (first eyes). The "yellow" AF-1 YA60BB was mainly implanted in eyes with macular drusen, whereas the other two IOL types were selected statistically. Eyes with no guaranteed postoperative follow-up and eyes with previous surgical interventions were excluded. Beside a complete ophthalmological examination, biometry (IOLMaster, Zeiss) and objective refraction (Nidek) were assessed. IOL power was predicted according to the Holladay-I formula and with the IOL-specific constants provided by the manufacturer. The objective refraction was evaluated again four to six weeks after cataract surgery. The "predictive error" (PE) and the absolute value of PE (APE) were defined as the deviation of the postoperative objective refraction to the target refraction. RESULTS: Mean target refraction of the 246 eyes was - 0.54 +/- 0.68 D and PE was - 0.24 +/- 0.75 D (APE: 0.57 D). With the MA 60AC a PE/APE of - 0.24 +/- 0.71 D/0.59 D was achieved. With the AR 40e the PE/APE was - 0.10 +/- 0.73 D/0.51 D, and with the AF-1 YA60BB the PE/APE yielded - 0.54 +/- 0.73 D/0.69 D. With the AR 40e/MA 60AC/AF-1 YA60BB, altogether 77%/75%/62% ranged in three-quarters of a dioptre around the target refraction. CONCLUSIONS: In general, four to six weeks after cataract surgery the achieved spherical equivalent was more myopic than the target refraction. The AR 40e yielded the lowest PE/APE, whereas the AF-1 YA60BB resulted in the highest PE/APE. If this trend is manifested by other investigators with a larger sample size, the manufacturer should optimise the lens specific constants.

[Microsurgical quality assurance exemplified by cataract surgery]. / Mikrochirurgische Qualitätssicherung am Beispiel der Kataraktchirurgie.

BACKGROUND: The new German laws demand comprehensive quality assurance in all hospitals. The prerequisite for this in ophthalmic microsurgery is the complete documentation of all relevant surgical details including patient comorbidity. METHODS: Since 1989 we have documented all microsurgical procedures using a computer-based surgery recording system. To analyze the changing spectrum of cataract surgery in a university eye hospital, we evaluated 12,653 consecutive cataract extractions divided into 5 periods between 1990 and 1999. RESULTS: Within these periods the patients' age changed significantly (p < 0.001), whereas the gender showed no significant change (p = 0.34). Furthermore, there was an increase in the number of simultaneous surgical procedures (p < 0.01) as well as the frequency of the pseudoexfoliation syndrome (p < 0.001). In addition the mean duration of cataract procedures decreased (p < 0.001) and the occurrence of vitreous loss also decreased (p < 0.001). CONCLUSIONS: Long-term documentation of all relevant patient data enables valid evaluation of the patient spectrum. Furthermore, this monitoring helps to manage the challenge of quality assurance in ophthalmology.

Differential activation of TRAIL-R1 and -2 by soluble and membrane TRAIL allows selective surface antigen-directed activation of TRAIL-R2 by a soluble TRAIL derivative.

TNF-related apoptosis-inducing ligand (TRAIL) is a typical member of the tumor necrosis factor (TNF) ligand family that is expressed as a type II membrane protein (memTRAIL) and signals apoptosis via the death domain-containing receptors TRAIL-R1 and -2. Soluble recombinant derivatives of TRAIL (sTRAIL) are considered as novel tumors therapeutics because of their selective apoptosis inducing activity in a variety of human tumors but not in normal cells. Using antagonistic antigen-binding fragment (Fab) preparations of TRAIL-R1- and TRAIL-R2-specific antibodies, we demonstrate in this study that TRAIL-R1 becomes activated by both the soluble and the membrane-bound form of the ligand, whereas TRAIL-R2 becomes only activated by memTRAIL or soluble TRAIL secondarily cross-linked by antibodies. Furthermore, we show that the restricted signal capacity of sTRAIL can be readily converted into a fully signal competent memTRAIL-like molecule, i.e. a TRAIL-R2 stimulating ligand, by genetic fusion to an antibody derivative that allows antigen-dependent 'immobilization' of the fusion protein to cell surfaces. We conclude that antibody targeting-dependent activation can be used to design selective therapeutics derived of those ligands of the TNF family that are biologically inactive in their soluble form.

[Quality management according to DIN EN ISO 9001 at a university eye hospital]. / Qualitätsmanagement nach DIN EN ISO 9001 an einer Universitätsaugenklinik.

BACKGROUND: Quality assurance is an integral part of modern microsurgical ophthalmology. Health care laws also mandate overall quality management. MATERIALS AND METHODS: In recent years we have standardized the preexisting features of quality management according DIN EN ISO 9001 and have integrated previously missing features. RESULTS: Establishing quality management according to ISO 9001 is possible even at a university eye hospital and department of ophthalmology. Certification according to ISO 9001 specifications was granted in April 1999. The major difficulty was in translating industrial norms to the context of an eye hospital. It was also difficult to overcome skepticism towards quality-assurance measures that lie beyond ophthalmological quality control. CONCLUSION: It is useful and feasible to establish a quality management system at German university eye hospitals and departments of ophthalmology. Certification according to ISO 9001 is one possibility to make a quality management system transparent and evaluable both inside and outside the hospital.

[The Susac syndrome--a retinocochleocerebral angiopathy]. / Das Susac-Syndrom--eine retinokochleozerebrale Angiopathie. Ein Beitrag zur Differenzialdiagnose der Multiplen Sklerose.

INTRODUCTION: There has been a series of case reports of otherwise healthy patients suffering from microangiopathy of the brain, retina and cochlea. Most patients were young women presenting clinically with a subacute encephalopathy, branch retina artery occlusions, and hearing loss. In 1994 the name "Susac syndrome" has been proposed for this disease entity. METHOD: Case report and review of 64 published cases, identified through MEDLINE are given. CASE REPORT: We describe a 32-year-old otherwise healthy woman presenting with a subacute encephalopathy, multiple branch retinal artery occlusions and bilateral hearing loss. MRI of the brain revealed multiple small white and grey matter lesions without contrast enhancement. CSF protein was elevated, oligoclonal bands were negative. Immunological laboratory parameters, microbiology, virology, coagulation studies, SEP, AEP, VEP and cerebral DSA were normal. REVIEW OF THE LITERATURE: Of 64 identified patients 58 were women. The mean age of the patients was 30 years. 60 patients (94%) had arterial occlusions, which were bilateral in 39%. 48 patients reported hearing loss, 37 patients (58%) had a global encephalopathy, but other neurologic manifestations were common. CONCLUSION: This rare syndrome has a strong young female preponderance. MRI of the brain often shows lesions suggestive of multiple sclerosis. Fluorescein angiography may show arteriolar wall hyperfluorescence. Patients can be identified at an early stage with a careful history and physical examination. Early treatment with corticosteroids is often associated with a good prognosis. Cyclophosphamide and antiplatelet drugs may be added in complicated cases.

[Adamantiadis-Behçet syndrome: fluorescein angiography and choroid ischemia]. / Das Adamantiadis-Behçet-Syndrom: Fluoreszenzangiographie und choroidale Minderperfusion.

BACKGROUND: Adamantiadis-Behçet's disease is a chronically progressing multisystemic disorder. The underlying disease mechanism is an obliterative vasculitis of unknown etiology. Main clinical symptoms are oral and genital aphthous ulcers and intraocular inflammations. Additionally, cutaneous, rheumatoid, neural, gastrointestinal, or cardiovascular manifestations may be observed. Diagnosis is based on clinical features since currently no specific laboratory tests or pathognomonic histopathological features are available. Ocular changes may provide important diagnostic clues to the systemic disease. PATIENTS AND METHODS: In a retrospective fashion 196 patients of the Department of Ophthalmology of the University of Erlangen-Nürnberg between 1988 and 1998 with retinal vasculitis seen by fluorescence angiography were evaluated according to the diagnostic criteria of the "Behçet's Disease Research Committee of Japan". RESULTS: Among 196 patients there were 12 patients with Adamantiadis-Behçet's disease. Apart from retinal vasculitis, angiographic features included capillary dropout in 64% (9/14), swelling of the optic disc in 79% (11/14) and irregularly delayed areolar filling of choriocapillaris in the early phase of fluorescence angiography in 43% (6/14) of eyes. There was no statistically significant relationship between severity of the systemic disorder and the activity of the ocular disease. CONCLUSION: Apart from retinal vasculitis, 43% of eyes in patients with Adamantiadis-Behçet's disease presented a delayed choroidal filling in fluorescence angiography as a sign of choroidal involvement of occlusive vasculitis. We observed leakage of fluorescein from the optic disc, which could be due to a secondary inflammation of the ciliary circulation. Inflammatory involvement predominantly of choroidal vessels, as visualized in fluorescence angiography, may be a diagnostic lead in Adamantiadis-Behçet's disease.

[Laser tyndallometry and monitoring of treatment in 20 children with juvenile uveitis]. / Laser-Tyndallometrie und Therapie-Monitoring bei juveniler Uveitis--eine retrospektive Untersuchung bei 20 Kindern.

BACKGROUND: Treatment of children with juvenile uveitis requires adequate control of inflammation while minimizing systemic or ocular side effects. The study was performed to evaluate the potential use of the laser flare-cell meter in monitoring and adjustment of therapy in juvenile uveitis. PATIENTS AND METHODS: Retrospectively, we monitored 20 children (11 girls and 9 boys) with an age range from 3 to 15 years presenting with juvenile iridocyclitis (10/20), intermediate (5/20) or posterior (5/20) uveitis. During the follow-up period (median 25.2 months, range: 2 to 83 months) multiple clinical controls were performed. We recorded clinical data, present therapy, and measurements using the laser flare-cell meter (Kowa FC-1000). RESULTS: Laser flare-cell meter measurements were easily obtained and highly reliable even in these young patients. During follow-up, 36 recurrences of intraocular inflammation were detected in 19 eyes. Relapses of inflammation as well as a response to treatment were seen at an early stage. Semiquantitative observations of cells and aqueous flare in the anterior chamber, or visual acuity of patients were less reliable in predicting recurrences. CONCLUSIONS: Laser tyndallometry offers a reliable, examiner-independent method to assess intraocular inflammation in children with juvenile uveitis and to adjust the necessary treatment.

Age-related macular degeneration and optic disk morphology.

PURPOSE: To assess the relationship between age-related macular degeneration and appearance of the optic disk. METHOD: By morphometric evaluation of wide-angle color fundus photographs, 143 patients with age-related macular degeneration were compared with 33 normal subjects and with 83 diabetic patients. RESULTS: Size and shape of the optic disk and size of parapapillary atrophy did not vary significantly (P > .12) between the study groups. CONCLUSIONS: Age-related macular degeneration is not associated with special features in the appearance of the optic disk. Parapapillary atrophy, large or small optic disk, and abnormal disk shape are neither risk factors nor protective factors of age-related macular degeneration.

Recurrent optic nerve head infarctions associated with combined factor V Leiden- and factor II:G20210A-mutation.

PURPOSE: To demonstrate the association of bilateral recurrent optic nerve head infarctions with thrombophilia due to combined factor V:R506Q- and factor II:G20210A-mutation. METHODS: Case report. We examined a 55-year-old man with a two-year history of three segmental optic nerve head infarctions. Visual acuity was 20/80 on the left and 20/25 on the right eye. RESULTS: Ophthalmologic, cardiologic, radiologic, neurologic and hematologic-immunologic examinations were unremarkable except for increased APC-resistance (APC ratio: 1.4; normal value >2) due to heterozygous factor V:R506Q-mutation and high factor II-levels due to factor II:G20210A-mutation. Therapy with coumarin was instituted at INR 2.0-3.0 and no relapse has occurred over the past 1-year period. CONCLUSION: Combined occurrence of thrombogenic factor II:G20210A-mutation and factor V:R506Q-mutation may be causally linked to recurrent optic nerve head infarctions.

[Outcomes after penetrating keratoplasty in congenital hereditary corneal endothelial dystrophy (CHED). Report on 13 eyes]. / Ergebnisse nach perforierender Keratoplastik bei kongenitaler hereditärer Hornhaut-Endotheldystrophie (CHED). Bericht über 13 Augen.

BACKGROUND: Congenital hereditary endothelial dystrophy (CHED) is a rare bilateral corneal disease. The stromal opacity is supposed to result from terminal misdifferentiation of the endothelial cells. In this study we present the morphological and functional results after penetrating keratoplasty in children with CHED who were operated in our department between 1981 and 1997. PATIENTS AND METHODS: In a retrospective clinical cross-sectional study we looked up case histories of 13 eyes from 8 children (7 female, 1 male) with a mean age of 6.0 +/- 3.1 years (ranged from 3 to 14 years). In all children penetrating keratoplasty was performed by one surgeon (GOHN), in 3 eyes using nonmechanical excimer laser trephination. The graft-diameter was in 7 eyes 7.0/7.1 mm, in 2 eyes 7.0/7.2 mm, in 2 eyes 6.5/6.6 mm (resp. 6.8 mm), in 2 eyes 6.0/6.1 mm (resp. 6.2 mm). Fixation of grafts was achieved in 2 eyes by single running suture, in 8 eyes by double running suture and in 3 eyes by multiple interrupted sutures. RESULTS: During a mean follow-up of 4.0 +/- 2.4 years visual acuity increased in all patients (from light perception to 6/20 preoperatively to 2/200 to 14/20 postoperatively). In one patient corneal endothelial-epithelial-decompensation occured (both eyes unterwent previous antiglaucomatous surgery elsewhere), and in 1 patient loosening of one suture happened after 10 month. No immunological graft reaction occurred during follow-up. After excimer laser trephination (3 eyes from 2 patients) visual acuity and corneal astigmatism after surgery was favorable in comparison to all other patients. CONCLUSION: In children with CHED penetrating keratoplasty results not only in a clear cornea but also in a satisfactory functional outcome. Postoperatively periodical morphological controls and assessment of refraction as well as means to prevent amblyopia are indispensable before age 7.