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BACKGROUND: Since 1982, the indirect binocular ophthalmoscope designed by Foerster has been in use in ophthalmology. The option to implement a new illumination technique using a light-emitting diode (LED) and a new power source should be evaluated in terms of technical feasibility and patient safety. METHODS: The cooling element was redesigned to accommodate the new LED electronics and their components, including an option for a variable brightness control. A more compact rechargeable battery was utilized with variable fixation at the headband or elsewhere. Photometric measurements of light intensity and the operating time were planned. Furthermore, a review of the new lighting technology in terms of EN ISO 15004-2 and EN ISO 10943 was necessary. RESULTS: Technical adjustments to accommodate the LED inside the cooling element could be realised. The power source was a modern rechargeable lithium-ion battery with variable fixation. The luminous intensity of the LED is superior to that of the halogen lamp and the operating time was increased to 520 minutes. The required limits according to DIN EN ISO 15004-2 for ophthalmic devices were met by our measurements. CONCLUSION: The optimisation of the indirect binocular ophthalmoscope brings improvements in illumination intensity and operating time. A conversion for models already in use is possible. A certified appraisal for compliance with the appropriate standards is the next step.

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OBJECTIVE: Uveal melanoma primarily metastasizes hematogenously with metastases often confined to the liver. The aim of this study was to investigate the presence of circulating tumor cells (CTC) in patients with metastatic disease as a marker for systemic disease and to determine their prognostic relevance. METHODS: Blood samples from 68 patients were collected at the time of initial treatment of metastases. mRNA expression of tyrosinase and MelanA/MART1 as a surrogate marker for the presence of CTC was analyzed by real-time RT-PCR and compared with patient characteristics. RESULTS: CTC were detected in 63% of all patients and in 67% of the 48 patients with only liver metastases. Univariate and multivariate analyses revealed PCR results and serum lactate dehydrogenase as independent prognostic factors for progression-free (hazard ratios 2.2/3.5) and overall survival (hazard ratios 4.0/6.5). Combination of PCR and lactate dehydrogenase divided the patient cohort into 3 groups with distinct prognosis. CONCLUSION: CTC as evidence for systemic disease can be found in the majority of patients with metastatic uveal melanoma, including patients with visible disease confined to the liver. Detection of CTC-specific mRNA transcripts for tyrosinase and MelanA/MART1 by PCR is a poor prognostic factor for progression-free and overall survival. Characterization of CTC could improve the understanding of their biology.

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BACKGROUND: The aim of this study was to characterise the results of a screening for von Hippel-Lindau disease (VHL), angiomatosis retinae (AR) and further VHL lesions in at-risk relatives of ophthalmological VHL index patients. METHODS: A retrospective analysis of 20 VHL index patients identified by the presence of angiomatosis retinae and a mutation of the VHL gene was carried out. A molecular genetic test for a VHL mutation and funduscopy was offered to all available at-risk relative. In the case of a positive test result, repeated screening for AR and further VHL lesions were suggested. RESULTS: Fifty-one out of 86 first- and second-degree relatives were screened, and 73 % showed a VHL mutation. At first presentation, asymptomatic AR was present in 55 %, at the end of the study in 72 % of gene carriers. In contrast to the index patients, angiomas were small and could be treated without functional loss. During the study 4 eyes of index patients developed blindness, whereas in the affected relatives no such event occurred. Affected relatives developed further VHL lesions to the same number and extent as the index patients. CONCLUSIONS: This study demonstrates the necessity of a screening of at-risk relatives of patients with AR and VHL. Molecular genetic screening allows an early identification of affected relatives. Early and regular screening enables the detection of small retinal angiomas and their treatment without functional loss.

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The treatment of large uveal melanomas poses a therapeutic challenge, due to the expected treatment-related side-effects. After sole radiotherapy the majority of patients are faced with radiogenic complications secondary to the large amount of tumour necrosis. Alternative treatment modalities addressing this issue are transscleral resection in arterial hypotension in anteriorly located tumours and endoresection via pars plana vitrectomy in posteriorly located tumours. A surgical resection treatment was applied in 292 patients with large uveal melanomas. In 150 patients the tumour was treated by transscleral resection and postoperative adjuvant (106)ruthenium brachytherapy and 142 patients were treated by primary proton beam irradiation and secondary endoresection. The mean follow-up time was 3.8 and 2.5 years, respectively. Local tumour control was achieved in 76 % and 98 %, respectively. The 5-year metastatic rates were 28 % and 21 % and eye retention was achieved in 82 % and 97 %, respectively. Surgical resection of uveal melanomas with adjuvant radiotherapy is the treatment of choice in cases of large tumours, avoiding enucleation in the vast majority of cases in the long term, without increasing the incidence of tumour-related risks.

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PURPOSE: To evaluate the association of HLA-B51 and ocular involvement in Adamantiades-Behçet's disease. METHODS: We retrospectively analysed all patients with Adamantiades-Behçet's disease examined in our Department of Ophthalmology since 1982. All patients fulfilled the criteria of the International Study Group for Behçet's disease. We included 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years. RESULTS: The mean age at the first manifestation was 23 years; full disease was noted at 32 years. The mean age at the time of eye involvement was 30 years. Most of the patients were of Turkish (n=73) or German (n=34) origin. A total of 56% patients developed eye involvement. Forty-nine out of 76 HLA-B51-positive patients (64.5%) and 26 out of 60 HLA-B51-negative patients (43.3%; P=0.014) developed ocular involvement. CONCLUSION: More than the half of the patients with Adamantiades-Behçet's disease evaluated in our department developed ocular involvement. There was a statistically significantly higher frequency of HLA-B51 in these patients.

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BACKGROUND: Because of the high local recurrence rates after excision of conjunctival melanomas, adjuvant local chemotherapy or irradiation is recommended. Strontium-90 brachytherapy is one radiotherapeutic option due to its low penetration depth. METHODS: 15 patients with conjunctival melanoma were treated with adjuvant strontium-90 brachytherapy after tumour excision. The treatment was fractionated into 9 irradiation sessions with 6 Gy each. The mean follow-up was 35 months (12-60 months). RESULTS: Seven patients (46%) had no recurrence during the follow-up. Three patients (20%) had a recurrence in the treated or adjacent area. Eight patients (53%) developed new tumours in non-treated areas. CONCLUSIONS: Strontium-90 brachytherapy is a useful adjuvant in the treatment of conjunctival melanomas. Regular ophthalmoscopic controls are necessary because of the high rate of new tumours in non-irradiated areas, especially in cases with primary acquired melanosis.

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The choice of primary vitrectomy as the first treatment method for rhegmatogenous retinal detachment has grown in popularity over recent years. The main reason behind this trend is the improved control of more complicated situations of retinal detachment. However, clinical trials comparing primary vitrectomy with scleral buckling had failed to demonstrate an advantage of this method regarding anatomical and functional results. The Scleral Buckling Versus Primary Vitrectomy in Rhegmatogenous Retinal Detachment Study (SPR study) is a prospective, randomised, multicentre study comparing primary vitrectomy with or without additional scleral buckling to scleral buckling alone. Overall, 681 patients with more complicated retinal detachments were recruited in 25 centres. In the phakic subgroup of patients, an advantage regarding the main endpoint (change in visual acuity) was found in the scleral buckling group. In the pseudophakic subgroup, no difference in functional outcome could be seen; however, better anatomical results with a lower rate of retina-affecting reoperations was observed in the vitrectomy group, with particularly good results in the subgroup of patients receiving vitrectomy and additional scleral buckling. Based on the available data, primary vitrectomy combined with a scleral buckle is the method of choice in more complicated types of retinal detachment in pseudophakic patients. In contrast, primary vitrectomy does not seem to offer an advantage over scleral buckling in phakic patients.

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PURPOSE: This study was undertaken to identify and compare the prognostic value of gene expression, chromosomal, and clinico-pathological data for the prediction of subsequent metastases in patients with primary uveal melanoma. PATIENTS AND METHODS: For comparison of different sets of predictor variables diagonal linear discriminant analysis was used. Chromosomal events were assessed by comparative genomic hybridization and gene expression profiling by microarray. Twenty-eight patients with a median follow-up of 68 months were analyzed, of whom 12 had developed subsequent metastases. RESULTS: Diagonal linear discriminant analysis with crossvalidation of gene expression data detected 42 genes as differentially expressed in metastasizing vsnon-metastasizing uveal melanomas in all 28 cases. Comparing quantitative scores of discriminant analysis, grouping precision was significant better with gene expression profiling compared to comparative genomic hybridization (P=0.01) and to clinical data (P=0.001). Two published gene lists associated with monosomy 3 and metastatic tumor growth were used as classifier for discriminant analysis and yielded superior classification in patients with and without subsequent metastases than chromosomal or clinico-pathological data. CONCLUSION: In our patient cohort gene expression profiling of primary uveal melanoma tissue was superior to clinical-pathological and chromosomal analysis to assess for the risk of subsequent metastases.

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A case of juxtapapillary capillary retinal angioma associated with a vascularized epiretinal membrane of the macula in a 6-year-old girl is presented. Von-Hippel-Lindau-Syndrome was revealed by molecular genetic methods, and further family members were identified as gene carriers. The retinal angioma embedded in an epiretinal membrane was removed completely with the membrane by pars plana vitrectomy with a good functional result. Histopathology confirmed the diagnosis of capillary angioma.

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BACKGROUND: Several trials demonstrated efficacy of the gemcitabine/treosulfan (GeT) combination in metastatic uveal melamoma. This randomized phase II trial compared the GeT combination versus treosulfan alone (T) in this rare disease. PATIENTS AND METHODS: Chemotherapy-naive patients with proven metastatic uveal melanoma were randomly assigned to receive 1000 mg/m(2) of gemcitabine plus 3500 mg/m(2) of treosulfan (GeT) or 3500 mg/m(2) of T. Chemotherapy was administered on days 1 and 8 in both arms, cycles were repeated on day 29. Primary end point was rate of responses and disease stabilizations. RESULTS: Forty-eight patients were randomized. Seven confirmed stable diseases (SDs) and one partial remission (PR) were observed in 24 patients treated with the GeT regimen, whereas no PR and only three SDs were observed in the T arm (P = 0.08). Median progression-free survival (PFS) was 3 months (95% CI 1.1-4.9) and 2 months (95% CI 1.7-2.3) in the GeT and T arm (P = 0.008, log-rank). Six and 12 months PFS was 34.8% and 17.9% and 16.7% and 0% always favoring the GeT arm. CONCLUSIONS: This first randomized trial in metastatic uveal melanoma showed a superior PFS and a trend for a higher response/stabilization rate of the GeT combination over T.

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AIM: This retrospective study should examine and judge the surgical indications and the therapeutic possibilities as well as their complications in patients with ocular manifestations of Marfan syndrome (MFS) diagnosed according to the criteria of the Ghent nosology. PATIENTS AND METHODS: The study included 17 patients. Operative indications were increasing subluxation of the lens, retinal detachments and secondary glaucoma. The operative procedure depended on patient age and findings. Eleven MFS patients were operated in both eyes and six MFS patients in one eye. RESULTS: Stabilization or functional improvement of visual acuity could be achieved in all patients in whom no disorders limiting visual acuity or amblyopia were present preoperatively. In six eyes of five patients, lens insertion was accomplished via a pars plana approach. Lens removal without implantation of an intraocular lens was performed in 16 eyes of 10 patients. Pars plana vitrectomy was accomplished in 12 eyes. Complications were well controlled by pars plana vitrectomy. CONCLUSIONS: Difficult preoperative situations and postoperative complications are not rare in MFS patients. However, they can be controlled well by means of modern vitreous surgery.

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BACKGROUND: A retrohyaloidal haemorrhage can be caused by different underlying diseases. Usually dense haemorrhages are treated by early vitrectomy. We want to report the successful treatment of a retrohyaloidal haemorrhage by means of Nd:YAG laser membranotomy. MATERIALS AND METHODS: A 47-year-old man noticed two weeks before presentation a sudden visual loss in the left eye. Visual acuity on the first visit was c. c. 20/32 OD and c. c. 20/400 ex. OS. Dilated fundus examination of the left eye showed multiple intraretinal haemorrhages in the superior periphery and a dense retrohyaloidal haemorrhage over the macula. Anamnestic risk factors for ocular bleeding were systemic hypertension in connection with adiposity. RESULTS: A retinal branch vein occlusion with retrohyaloidal haemorrhage due to systemic hypertension was diagnosed. To drain the retrohyaloidal haemorrhage into the vitreous an Nd:YAG laser membranotomy of the anterior surface of the haemorrhage was performed. Visual acuity increased to 20/100 within 30 min. CONCLUSION: Possible therapeutic options to manage retrohyaloidal haemorrhages are observation, primary vitrectomy and Nd:YAG laser membranotomy. The presented case shows laser membranotomy to be a promising possibility for a rapid resolution of retrohyaloidal haemorrhage in selected patients.

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PURPOSE: The aim of this study was to analyse the results of retinopathy of prematurity (ROP) screening, stage of disease and time of treatment from one tertiary care centre. PATIENTS AND METHODS: Between 1991 and 2001, 666 infants with a gestational age (GA) < 33 weeks or birth weight (BW) less than < 1501 g were examined. Coagulation treatment was performed in accordance with the Cryo-ROP study, after reaching the threshold. All infants were followed-up until a stable retinal situation was reached. RESULTS: Mean GA of the 666 infants was 28.5 +/- 2.3 weeks. Mean BW was 1180 +/- 372 g. During the years of screening, the GA and BW significantly decreased, but no significant change in the yearly incidence of ROP was detected. 460 (69.1 %) of the infants did not develop any stage of ROP. Maximum stage of ROP in the worse eye was stage one in 51 (7.6 %) infants, stage two in 59 (8.9 %) infants and stage three below threshold in 62 (9.3 %) infants. 34 (5.1 %) infants were treated with coagulation treatment. Mean time of treatment was 36.5 weeks postmenstrual age, or 10.7 weeks postnatal age. Postnatal age showed a better correlation to treatment time than postmenstrual age. No infant was treated before seven weeks of life and not before 32 weeks postmenstrual age. CONCLUSION: In spite of the increasing survival of children with lower GA and BW the incidence of ROP has not increased, and the incidence of ROP in our centre appears to be comparable to other international studies. In contrast to the Cryo-ROP study, treatment time correlates better to postnatal age than to postmenstrual age. All infants who underwent treatment were detected at an appropriate time using German screening guidelines.

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BACKGROUND: Photodynamic therapy (PDT) with verteporfin has been successfully introduced to treat choroidal neovascularisations (CNV) that have more than 50% classic CNV components due to exudative age-dependent macular degeneration (AMD) and other diagnoses. However, what is still unclear is if patients with recurrent subfoveal CNV who have had prior laser coagulation can also profit from PDT for CNV. METHODS: The course of visual acuity (ETDRS) and the fluorescein angiographic findings were retrospectively analysed after standard PDT of recurrent subfoveal CNV after prior argon laser coagulation of an extrafoveal or juxtafoveal CNV in AMD or other diagnoses. RESULTS: A total of 20 consecutive eyes from 19 AMD patients were evaluated. After 12 months in 14 of the 20 eyes (70%) deterioration in visual acuity of 3 or more ETDRS lines could be avoided and likewise in 7 of 16 eyes (44%) after 24 months. After the 1st year, 5 of the 7 eyes (71%) with recurrent subfoveal CNV after laser coagulation of a myopic, postinflammatory or idiopathic CNV had deterioration of visual acuity of less than three lines or an improvement in visual acuity. CONCLUSIONS: Despite the low number of patients examined, we could see an indication for PDT in recurrent subfoveal CNV after prior argon laser coagulation of extrafoveal or juxtafoveal CNV. When the patient presented with a CNV cause other than AMD, a better prognosis for visual acuity was achieved.

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BACKGROUND: The aim of this study was to evaluate whether photodynamic therapy (PDT) with verteporfin is able to induce tumor cell necrosis in human uveal melanomas. METHODS: On four eyes with an uveal melanoma, PDT with verteporfin was performed on the tumor 2-3 days before planned enucleation. The eyes were evaluated histologically. RESULTS: In two melanomas with only mild pigmentation some effects after PDT were detected on tumor tissue in a depth up to 2.5 mm at light doses > or =100 J/cm(2). Histologically, vascular occlusion and thrombosis in tumor vessels were observed. In the heavily pigmented melanoma no tumor necrosis was induced with the above-mentioned parameters. CONCLUSION: Depending on treatment parameters and tumor pigmentation, PDT with verteporfin is able to induce tumor necrosis in human uveal melanomas. Based on these results it is possible that PDT can become an adjuvant treatment method for uveal melanoma.

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PURPOSE: To analyze and compare the anatomical and functional outcome following cryotherapy or laser therapy for ROP. METHODS: Between April 1991 and February 2002, 127 eyes with ROP (65 patients) were treated after threshold ROP was reached. Ten additional eyes with zone I disease were treated with prethreshold. All patients were followed up until a stable retinal situation occurred. RESULTS: An "unfavorable outcome," as described in the Cryo ROP study, occurred in 1 of 91 (1%) eyes with laser treatment and in 3 of 46 (6.5%) eyes with cryotherapy. Temporal dragging of vessels was noticed in 6 of 91 eyes (6.6%) with laser treatment vs 7 of 46 eyes (15.2%) with cryotherapy, respectively. Visual acuity > or =20/25 was achieved in 39.2% in eyes with laser therapy and in 17.6% with cryotherapy (p<0.05). CONCLUSION: A strict screening protocol and treatment at onset of threshold disease results in an excellent anatomical and functional outcome. The results of laser therapy were superior over cryotherapy, indicating that laser treatment is the therapy of choice.

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PURPOSE: This study compares clinical and molecular genetic findings in patients with congenital cone dysfunction. METHODS: In this study 28 patients underwent a basic ophthalmologic examination. Except for a 1-year-old boy, color vision, perimetry, and full-field ERG (ISCEV standard) were evaluated in all patients. Blood samples were taken for molecular genetic analysis of the CNGA3, CNGB3, or GNAT2 genes. RESULTS: Two patient groups could be distinguished: patients without and with residual cone function in the ERG. In 14 of 17 patients without cone function, mutations in one of the three genes were detected, and except for one patient mutations in both alleles could be determined. In these patients, visual acuity was reduced to 20/400 and color discrimination was absent. In 2 of 11 patients with residual cone function, mutations in one allele of the CNGB3 gene were detected. It is of interest that 6 of 16 patients with mutations perceived their disease as progressive; in three of them we could determine a progression. Only in 4 of 16 patients was the ocular fundus normal. The other patients with mutations presented with central pigment irregularities, attenuated vessels, or pale optic disk. CONCLUSION: In patients with congenital cone dysfunction without cone function in the ERG, an analysis of the CNGA3, CNGB3, or GNAT2 gene is advisable. In contrast, patients with residual cone function did not show clear association with mutations in one of the three genes. In patients with mutations, retinal alterations and nystagmus are frequent. In contrast to the designation of these disorders as stationary, in some patients with mutations in the CNGA3 and CNGB3 gene slow progression was observed.

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PURPOSE: Large uveal melanomas located close to the optic nerve and/ or to the fovea have an unfavourable prognosis with regard to visual preservation and eye retention, due to the high incidence of radiation and tumour necrosis induced complications. Endoresection of the tumour after proton beam irradiation, is an alternative approach, aiming to reduce the incidence of ocular morbidity caused by tumour necrosis after sole radiotherapy. PATIENTS AND METHODS: 32 patients with large uveal melanomas (mean tumour thickness: 9.1 mm, mean tumour volume: 0.77 cc), received a primary proton beam irradiation (60 CGE) and underwent subsequent endoresection via a 3-port pars plana vitrectomy. The median pretreatment visual acuity was 0.2. The mean follow-up was 13.9 months. RESULTS: The postoperative visual acuity after 12 months was 0.12 (median, mean visual acuity loss 0.08). The probability of developing radiation retinopathy or papillopathy within the first year after treatment was 35% and 28% respectively and the probability of enucleation was 9% within the first postoperative year. No tumour recurrences were observed and 2 patients developed liver metastases. CONCLUSIONS: Endoresection following irradiation of large uveal melanomas located close to the optic nerve and/ or the fovea seems to be a useful and safe alternative to the traditional irradiation or enucleation. The incidence of complications following our approach seems to be lower when compared to radiation alone, where tumour necrosis is a substantial problem.

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