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Ciliary body melanoma have the worst prognosis among all uveal melanoma (UM). In this report we share our experience with CyberKnife and show that the outcome is not considerably worse than with UM located elsewhere.

Aims To describe results after neoadjuvant proton beam irradiation followed by transscleral resection of large uveal melanoma. Methods Retrospective interventional case series, including 106 consecutive patients. Local tumour control, enucleation and metastasis development were assessed with survival curves. Predictors of local recurrence and metastasis were investigated with log-rank testing. Results Mean follow-up was 3.2 years. Local recurrence occurred in five cases with an estimated risk of 4.2% and 10.4% at 3 and 5 years after treatment, respectively. Enucleation was performed in 10 cases with an estimated risk of 9.2% and 18.4% at 3 and 5 years, respectively. Significant risk factors for local recurrence were not evident. Metastasis was estimated to occur in 28.4% at 3 years and 40.3% at 5 years, correlating with patient9s age only (p=0.01). Seventy four patients (69.8%) underwent vitreoretinal surgery for complications after tumour resection. Median visual acuity (VA) was 20/50 at diagnosis and 20/400 in the third year after treatment. VA preservation of 20/200 or better was achieved in 33 patients (31.1%). Conclusion Neoadjuvant proton beam irradiation may help to prevent local recurrence after transscleral resection. Additional vitreoretinal surgery was frequently needed in the presented series. The majority of patients avoided enucleation and functional blindness.

Background Treatment modalities in iris melanoma include excision, plaque radiotherapy, photon or proton beam therapy and enucleation. In extensive tumours and diffuse seeding, radiotherapy remains as an alternative to enucleation. Methods This study is a retrospective, consecutive, interventional, single-institutional case series. 54 patients with a diffuse and non-resectable iris melanoma diagnosed from September 1998 to June 2012 were included. A 68-megaelectron volt proton beam was used to treat the anterior segment with a total dose of 4×12.5 cobalt grey equivalent. The cases were evaluated for local tumour control, eye retention, functional outcome and local complications after treatment. Results During a mean follow-up of 62.7 months (median 54.8 months, range 5.5–159.6 months), local tumour control was achieved in 96.3% of the patients. Cataract and glaucoma were the main complications developing after irradiation in 42.6% and 55.6%, respectively. In 34 of 44 patients (77.3%) who underwent cataract removal, a visual acuity of 20/40 or better following surgery was preserved. Enucleation was performed in three patients. The reason was suspected tumour recurrence in one and glaucoma in two. Hepatic metastasis occurred in one patient. Conclusions As an alternative to enucleation, whole anterior segment fractionated proton beam radiotherapy offered excellent local tumour control in diffuse iris melanoma. Given the limited alternatives, the rate of complications appears acceptable and visual function could be preserved in the majority of the patients during follow-up.

To delineate and discuss a not yet described possible ocular complication of selective intra-arterial chemotherapy (SIAC) for treatment of retinoblastoma.A 23-month-old girl with a large unilateral retinoblastoma was treated with repeated SIAC using 5 mg melphalan between July 2010 and January 2012. Clinical course of tumor and further ocular changes after therapy and histopathologic findings are described.In total, 5 SIAC were performed over a time period of 18 months. After the last SIAC, diffuse dense cataract prevented further funduscopy. In addition, anterior chamber seeding was obvious, leading to the decision to enucleate the eye. Histopathologically, nearly complete regression of the main tumor mass with prominent calcifications, but vital tumor seeding in the vitreous, on the lens surface, on the ciliary body, and in the anterior chamber, was observed. Peculiar vacuolation of the lens epithelial cells, liquefaction of the subepithelial lens fibers, and diffuse small vacuoles within the lens were striking.Repeated SIAC with melphalan may induce cataract formation, possibly as a toxic effect of the chemotherapeutic to the lens, maybe combined with radiation exposure during fluoroscopy. This ocular complication should be taken into consideration as a limitation of the number of feasible repeated treatments.

Bei einem choroidalen Osteom handelt es sich um einen benignen, zunehmend kalzifizierenden Tumor, der in der uberwiegenden Mehrzahl bei jungen Frauen in der zweiten oder dritten Lebensdekade diagnostiziert wird [1, 2]. Im fruhen Kindesalter kommen Osteome nur sehr selten vor. Klinisch imponiert ein choroidales Osteom meist als gut abgrenzbarer, gelb-orangener subretinaler Tumor mit geringer Prominenz [3], der typischerweise peripapillar liegt, in 25 % besteht eine bilaterale Manifestation. In der Literatur gibt es nur wenige Beschreibungen von Osteomen im Kindesalter (eine Falldarstellung eines 6-jahrigen [4] und eines 7-jahrigen Madchens [5]). Die Atiologie des choroidalen Osteoms ist unklar, es gibt hierzu 2 Theorien: Entweder ist das Auftreten eines Osteoms kongenital als ortsfremde Verknocherung – als sog. ossares Choristom – zu verstehen. Hierfur sprechen die belegten familiaren Haufungen. Einer zweiten Hypothese nach entwickelt sich das Osteom sekundar als Folge von Traumata oder Entzundungen, die Veranderungen im retinalen Pigmentepithel verursachen konnen und mit einer Latenz von Jahren zu einer Verknocherung fuhren [6]. In dem hier prasentierten Fall wurden wir eher von einer intraokularen ossaren Metaplasie sprechen.

Purpose External beam proton radiation therapy has been used since 1975 to treat choroidal melanoma. For tumor location determination during proton radiation treatment, surgical tantalum clips are registered with image data. This report introduces the intraoperative application of an opto-electronic navigation system to determine with high precision the position of the tantalum markers and their spatial relationship to the tumor and anatomical landmarks. The application of the technique in the first 4 patients is described. Methods and Materials A navigated reference base was attached noninvasively to the eye, and a navigated pointer device was used to record the spatial position of the tantalum markers, the tumor, and anatomical landmarks. Measurement accuracy was assessed on ex vivo porcine eye specimen by repetitive recording of the tantalum marker positions. The method was applied intraoperatively on 4 patients undergoing routine tantalum clip surgery. The spatial position information delivered by the navigation system was compared to the geometric data generated by the EYEPLAN software. Results In the ex vivo experiments, the maximum repetition error was 0.34 mm. For the intraoperative application, the root mean square error of paired-points matching of the marker positions from the navigation system and from the EYEPLAN software was 0.701–1.25 mm. Conclusions Navigation systems are a feasible tool for accurate localization of tantalum markers and anatomic landmarks. They can provide additional geometric information, and therefore have the potential to increase the reliability and accuracy of external beam proton radiation therapy for choroidal melanoma.

Hypoxia and its down-stream activated pathways are commonly involved in tumor progression. Genes involved in angiogenesis and glycolysis, i.e. vascular endothelial growth factor (VEGF) and lactase dehydrogenase A (LDHA), respectively, are transcriptionally controlled by the hypoxia inducible factors 1α and 2α (HIF1α and HIF2α). A series of 60 uveal melanomas were immunohistochemically assessed for the expression of VEGF and the phosphorylated/activated form of VEGF receptor 2 (pVEGFR2/KDR), after binding to VEGF. The expression of HIF1α, HIF2α and LDH5 was also investigated. Uveal melanomas overexpressing HIF2α (but not that of HIF1α) were significantly associated with high VEGF (P = 0.005), pVEGFR2/KDR (P < 0.0001) and LDH5 (P ≤ 0.0001). High LDH5 was linked with tumor necrosis (P = 0.01) and increased tumor size (P = 0.03). High VEGF was linked with phosphorylated pVEGFR2/KDR receptors. In univariate analysis high pVEGFR2/KDR receptor expression was significantly related with poor prognosis (P = 0.02). It is concluded that HIF2α plays an important role in the progression of uveal melanomas possibly by promoting the autocrine loop VEGF-pVEGFR2/KDR, and by enhancing the expression of LDHA gene, conferring thus a growth advantage. As pVEGFR2/KDR expression was significantly related with poor prognosis, inhibitors of this receptor may improve the clinical outcome of patients with pVEGFR2/KDR overexpressing uveal melanomas.

Autophagy is a self-degradation mechanism by which cells recycle their own cytoplasmic constituents. It has been claimed that, under certain conditions, such a process may be associated with tumor progression. In this study, the autophagic activity was investigated in a series of 99 uveal melanomas after immunohistochemical staining for the autophagy-associated proteins MAP1LC3A and BECN1, most commonly known as LC3A and Beclin 1, respectively. These were assessed in parallel with the hypoxia-inducible factor 1α (HIF1A) and its downstream protein lactate dehydrogenase 5 (composed by five LDHA subunits). Increased autophagic reactivity, detected by MAP1LC3A or BECN1, was associated with intense pigmentation and tumor hypoxia. Uveal melanomas with extensive overexpression of BECN1 or those with underexpression of this protein were associated with the worst prognosis, but the former manifested metastases much earlier than the latter; only 58% of patients with extensive BECN1 overexpression were alive at 4 years, compared with 80% of patients with underexpressed patterns. It is concluded that autophagy is commonly upregulated in uveal melanomas, and may be associated with hypoxia and intense pigmentation. There is a strong association between extensive BECN1 overexpression and early metastases/poor prognosis, and between underexpression of this protein and late metastases/better prognosis.

Endogenous endophthalmitis caused by filamentous fungi has been infrequently described and its prognosis in immunocompromised patients is largely unknown. Patients were identified through a single-centre database containing patients with endophthalmitis. Cases published since 2002 were reviewed. Clinical and treatment features as well as outcomes were analysed. Six patients were identified from the database. Underlying conditions were haematological malignancies (HM) and/or allogeneic haematopoietic stem cell transplantation (HSCT). Three patients underwent vitrectomy. None of the patients survived and the median time from first evidence of endophthalmitis until death was 33 days. The median time from first evidence of an invasive fungal infection to endophthalmitis was only 5 days. Fifty-six patients were identified from the literature. The majority of these patients underwent vitrectomy (27) or enucleation (10) and received intraocular antifungal therapy (28). Only 13 (23%) of 56 patients experienced an improved vision. The survival rate was 52% in all 56 patients but was significantly less in patients with HM or post-HSCT when compared with all others (26% vs. 70%, respectively; P = 0.003). Endogenous endophthalmitis caused by filamentous fungi is frequently associated with a permanent decrease or loss of vision. This type of fungal infection carries a particular poor prognosis in patients with profound immunosuppression, requiring improved treatment strategies.

Purpose. The black iris-lens diaphragm (ILD) can be used in the treatment of traumatic aniridia and aphakia. The aim of our study was to show postoperative functional and anatomic results and complications in a small case series. Methods. We retrospectively analyzed the files of 16 patients managed with a black ILD or a sole iris diaphragm in the period 1994–2007. Four of them were female and 12 were male. The mean age of the group was 50±17 years. At the time of the implantation, all of the eyes had already undergone primary surgical repair. Results. The preoperative best-corrected visual acuity in half of the patients was ≥0.1 and remained stable after the first postoperative year. During the follow-up years, one eye developed a subluxation of the implant and one eye an anterior chamber hemorrhage. At the same time, out of 8 eyes carrying a silicone tamponade in the vitreous cavity, silicone oil entered the anterior chamber in 3 cases. In one case, enucleation was undertaken due to phthisis. Conclusions. In the case of severely traumatized eyes with aniridia and aphakia, the implantation of a black ILD can have a positive effect on functional and anatomic stabilization.

The liver is the predominant site of metastases in the majority of patients with uveal melanoma, suggesting the evaluation of regional treatment approaches. Here we report our experience with transarterial chemoembolization (TACE) in uveal melanoma patients with pretreated liver metastases. Twenty-five patients were treated with fotemustine-based or cisplatin-based TACE after treatment failure of systemic therapy between 2003 and 2008 at our institution. Grade III toxicity consisted of gastric ulcer (n=1), fever (n=3), splenic infarction (n=1), and thrombocytopenia (n=1). No grade IV toxicity or catheter-associated complications were observed. Fourteen of 25 patients (56%) had stable disease for at least 2 months and four had partial remission. The median progression-free survival (PFS) was 3 months (95% confidence interval: 2-4 months) and the median overall survival (OS) was 6 months (95% confidence interval: 5-7 months), with 15% of patients alive at 1 year. Both PFS and OS were significantly longer, when pretreatment lactate dehydrogenase was below the two-fold upper limit of normal (n=11): PFS 5 versus 2 months (P

Die funktionellen Ergebnisse der Behandlung von Aderhautmelanomen ist abhangig von deren Grose und Lage innerhalb des Auges. Grose Tumoren haben eine erhohte okulare Morbiditat wegen der zu erwartenden radiogenen Nebenwirkungen. Hinzu kommt die okulare Belastung durch die vermehrte Anhaufung von Tumorzerfallprodukten durch den Tumorabbau und die Tumornekrose. Um diese Problematik zu adressieren sind kombinierte strahlentherapeutisch-chirurgische Verfahren entwickelt worden, die sowohl eine sehr gute Tumorkontrolle als auch eine reduzierte okulare Morbiditat zur Folge haben. In dieser Ubersichtsarbeit werden 292 Patienten zusammengefasst, bei denen entweder eine transsklerale Resektion in arterielle Hypotonie mit anschliesender Ruthenium-106 Brachytherapie oder eine Protonentherapie mit anschliesender Endoresektion durchgefuhrt wurde. Die 5-Jahres Uberlebenswahrscheinlichkeit der Patienten schwankte zwischen 21 und 28%. Die Enukleationsraten betrugen 3 und 18% und die lokale Rezidivraten 2 und 24% bei ab interno Endoresektion und ab externo Resektion entsprechend. Bei Patienten deren Tumor ab externo transskleral reseziert wurde fand sich eine Korrelation zwischen erhohter Rezidivrate und Mortalitat. Die Rezidivrate konnte mit einer praoperativen Protonenbestrahlung auf ein Minimum (2%) reduziert werden. Ein kombiniertes strahlentherapeutisch-chirurgisches Vorgehen scheint unter dem Lichte dieser Daten unabdingbar zu sein.

Die Protonenstrahlentherapie ist eine Hochprazisionsbestrahlung, die fur umschriebene Tumoren geeignet ist, die sich in der Nahe von kritischen Strukturen befinden und von der Bestrahlung geschont werden mussen. Aderhautmelanome des hinteren Augenpols erfullen durch ihre Nahe zu Makula und Sehnerv diese Kriterien. Durch den sehr steilen distalen und lateralen Dosisabfall des Protonenstrahls, konnen diese Strukturen optimal aus dem Bestrahlungsfeld herausgehalten werden, so dass optimale funktionelle Ergebnisse erzielt werden konnen. Zudem fand sich bei den 336 Patienten eine lokale Tumorkontrollrate von 97,3% und ein Augenerhalt von 94,5% in 3 Jahren. Die Strahlenretinopathie und die strahleninduzierte Opticusneuropathie betrugen 53% respektive 54% nach 3 Jahren. Der mittlere Visus fiel von ursprunglich 0,5 auf 0,27 ab. Zusammenfassend bietet die Strahlentherapie mit Protonen bei Aderhautmelanomen des hinteren Augenabschnittes hervorragende lokale Tumorkontrollraten und funktionelle Ergebnisse, so dass der etwas erhohte medizinische Aufwand allenfalls gerechtfertigt ist.

BACKGROUND: Because of high local recurrence rates after excision of conjunctival melanoma adjuvant local chemotherapy employing mitomycin C (MMC) or irradiation is recommended. Brachytherapy is possible with ruthenium-106-plaques ( 106 Ru) or with the strontium-90-plaques ( 90 Sr). PATIENTS AND METHODS: Fifty-six patients received an excision and adjuvant radiotherapy of conjunctival melanoma between 1992 and 2007. The mean follow-up was 42 months (12 - 151 months). Mean age was 62 (28- 86) years. As an adjuvant radiotherapy 15 patients received X-ray irradiation, 12 patients received 106 Ru-brachytherapy, 4 patients received proton beam therapy and 16 patients with conjunctival melanoma were treated with adjuvant strontium-90 brachytherapy after tumour excision. Four patients received proton beam irradiation and in 13 patients an exenteratio was performed. RESULTS: Twelve patients (21 %) developed tumour recurrences in or adjacent to the irradiated area. Thirteen patients (22 %) showed a recurrence distant from the primary site. Ten patients (18 %) developed systemic metastasis during follow-up. Seven patients (46 %) had no recurrence during the follow-up. Three patients (20 %) had a recurrence in the treated or adjacent areas. Eight patients (53 %) developed new tumours in non-treated areas. CONCLUSIONS: Adjuvant radiotherapy allows an acceptable local tumour control rate after excision of conjunctival melanoma. No obvious differences regarding tumour control or systemic metastasis could be seen between the different modes of radiotherapy used.

Hintergrund: Charakterisierung der Ergebnisse systematischer Screeninguntersuchungen nach einem von Hippel-Lindau Syndrom (VHL), einer Angiomatosis retinae (AR) und anderer Organläsionen eines VHL bei Verwandten von ophthalmologischen VHL- Indexpatienten. Methode: Retrospektive Auswertung einer[for full text, please go to the a.m. URL], 22. Jahrestagung der Retinologischen Gesellschaft

BACKGROUND: Today photodynamic therapy (PDT) with Verteporfin is the standard treatment option for symptomatic choroidal haemangiomas. Nevertheless, peripapillary or large haemangiomas pose a therapeutic dilemma, since severe adverse events have been demonstrated in cases where the optic disc was included in the treatment spot. In addition due to the size and the shape of the laser spot, peripapillary and large haemangiomas can only be undertreated or overtreated (with overlapping multiple spots). With the presented new ”paint-brush technique” it is possible to treat peripapillary and large choroidal haemangiomas completly without danger for the optic disc and without overtreatment. MATERIAL UND METHODEN: With the new technique the application of the laser spot is performed in a ”paint-brush fashion” by moving he spot with constant speed excentrically around the laesion’s centre over the entire tumour surface. This allows under visual control a complete and confluent PDT of the whole tumour surface without overlapping and/or missing areas or treating the optic disc. In total, 13 eyes of 13 patients (6 with peripapillary and 7 with large choroidal haemangiomas) have been treated. The mean follow-up time was 25.6, respectively, 14.5 months. RESULTS: The PDT with the ”paint-brush technique” allows an effective treatment of the leakage in both groups. The mean increase of visual acuity was 1, respectively, 1.4 ETDRS lines 3 months after therapy, which could be maintained over the whole follow-up period. At the last examination visual acuity was unchanged or better in 12 / 13 (92 %) of eyes. After 3 months the tumour height was significantly (p = 0.031 and 0.018) reduced to 1.7 mm (52 %), respectively, 1.3 mm (30 %), which was stable during the whole follow-up. No significant side effects could be detected. CONCLUSION: The use of the PDT ”paint-brush technique” allows complete treatment of choroidal haemangiomas under visual control. Sensitive structures like the optic disc could be excluded from treatment without double- or undertreatment. The functional and anatomic results are good. The technique is a safe and effective amendment in the treatment of symptomatic choroidal haemangiomas.

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.

Objective: The aim of the study was to assess the functional results and long-term visual prognosis in patients treated for acute endophthalmitis (AE). Design: Observational clinical series. Participants: Evaluation of 120 eyes of 114 patients with AE treated between 1991 and 2004. Methods: This retrospective institutional analysis included 120 eyes of 114 patients (52 male, 68 female) with AE and a minimum follow-up of half a year. The patients had a mean follow-up of 23 months (6–135 months) and a mean age of 61 years (8 months-94 years) at the time of treatment. Treatment included intraocular injection of antibiotics alone ( n = 18, 15%) and vitrectomy combined with intraocular and topical postoperative antibiotics ( n = 85, 70%). Results: The most frequent complications were vitreous or retinal hemorrhages ( n = 17, 14%), retinal detachment ( n = 17, 14%), choroidal detachment ( n = 3, 3%), secondary glaucoma ( n = 7, 6%), and recurrent endophthalmitis ( n = 3, 3%). Four eyes had to be enucleated because of recurrent and uncontrollable infection. Positive microbiological results were achieved in 67 of 88 specimens (76%). The most common isolate was Staphylococcus. At the end of follow-up, visual acuity was reduced (more than 2 lines) in 18 eyes (15%), stable in 22 (18%), and improved (more than 2 lines) in 72 eyes (60%). Conclusions: AE is a rare but severe disease with a potentially deleterious outcome in affected eyes. In our series, 78% of all eyes had stable or improved postoperative visual acuity following various treatment regimens, depending on the severity of each case.

Purpose: To determine the clinical characteristics of symptomatic choroidal metastasis (CM) resulting from metastatic lung cancer. Methods: Twenty-two consecutive patients with symptomatic CM resulting from lung cancer were retrospectively reviewed for ocular findings, medical history and systemic disease. All patients underwent a complete screening for further organ metastasis by computed tomography (CT) and bone scintigraphy. Annual frequency of CM was determined and compared with the incidence predicted from ocular screening studies. Results: In eight of 22 (36%; 95% confidence interval [CI] 17–59) patients, lung cancer had been diagnosed before occurrence of CM, with a median interval of 13 months. In 14 patients lung cancer was detected after diagnosis of CM, with a median interval of 1 month. Choroidal metastasis was unilateral, solitary and located close to or at the posterior pole in the majority of patients. Further organ metastasis with a median number of three affected organ systems was present in 19 (86%; 95% CI 65–97) patients. Median survival after diagnosis of symptomatic CM was 13 months, by contrast with 2 months in lung cancer patients with CM identified in an ocular screening study. The mean number of patients in Berlin diagnosed with symptomatic CM was 1.4 per year, which was two orders of magnitude less than predicted from screening studies. Conclusions: Symptomatic choroidal lung cancer metastasis in the majority of patients presents as a solitary tumour before diagnosis of lung cancer in patients with multiple organ systems affected by metastatic disease. Contrary to predictions from ocular screening studies, it is a rare clinical entity.

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.

To evaluate the association of HLA-B51 and ocular involvement in Adamantiades–Behcet's disease. We retrospectively analysed all patients with Adamantiades–Behcet's disease examined in our Department of Ophthalmology since 1982. All patients fulfilled the criteria of the International Study Group for Behcet's disease. We included 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years. 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. More than the half of the patients with Adamantiades–Behcet's disease evaluated in our department developed ocular involvement. There was a statistically significantly higher frequency of HLA-B51 in these patients.

Background The implantation of scleral fixated sutured posterior chamber lenses enables lens implantation in the absence of adequate lens capsule support. Anterior chamber lenses and their side effects can also be avoided, particularly in cases of unilateral aphakia and contact lens incompatibility. Methods This study is a retrospective analysis of 119 scleral fixated sutured posterior chamber lenses implanted in 115 patients. Seventy-nine cases of damaged zonula or damaged lens capsule and 17 cases of corneal decompensation secondary to anterior chamber lenses were the main indications for the suture fixation. The mean follow-up was 23 months (6–83 months). Results In 50% of all cases, improved visual acuity was achieved. Twenty-four percent of patients showed unchanged visual acuity, while 26% had poorer vision postoperatively, mainly because of corneal decompensation following anterior chamber lens explantation. The main postoperative complication was transient rise of intraocular pressure, occurring in 44% of the cases. Conclusions In most cases improved or comparable postoperative visual acuity was achieved. The main complication observed was postoperative glaucoma, especially in patients with pre-existing glaucoma.

Die primare Vitrektomie als Ersteingriff in der Behandlung der rhegmatogenen Netzhautablosung hat in den letzten Jahren weltweit eine immer weitere Verbreitung gewonnen. Der wesentliche Grund fur diesen Trend ist in der Erleichterung der Operation von komplizierten Ausgangsbefunden zu sehen. Die bisher durchgefuhrten vergleichenden Studien konnten jedoch keine Vorteile der anatomischen und funktionellen Erfolgsraten gegenuber der Buckelchirurgie zeigen. Die „Scleral Buckling versus Primary Vitrectomy in Rhegmatogenous Retinal Detachment Study“ (SPR-Studie) ist eine prospektive randomisierte multizentrische Studie, bei der die primare Vitrektomie mit oder ohne Zusatzbuckel bei 681 Patienten mit Netzhautablosungen mittleren Schweregrads an 25 Zentren mit der reinen Buckelchirurgie verglichen wurde. In der phaken Patientengruppe ergab sich ein Unterschied im Hauptzielkriterium (Visusanderung) mit Vorteilen fur die Buckelchirurgie bei vergleichbaren anatomischen Ergebnissen. In der pseudophaken Patientengruppe zeigten sich keine Unterschiede der funktionellen Ergebnisse und des Hauptzielkriteriums, allerdings konnte in der Gruppe der primaren Vitrektomie bessere anatomische Ergebnisse mit signifikant weniger netzhautrelevanten Reoperationen beobachtet werden. Besonders gute anatomsiche Ergebnisse wurden bei pseudophaken Patienten mit der Kombination der primaren Vitrektomie und einem Zusatzbuckel erzielt. Auf der Basis der bisher vorliegenden Studienergebnisse ist bei pseudophaken Patienten mit komplizierteren Ausgangssituationen die primare Vitrektomie mit Zusatzbuckel als Therapierverfahren vorzuziehen. Bei phaken Patienten bietet die primare Vitrektomie keine Vorteile gegenuber der Buckelchirurgie.

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.

Background: The aim of this study was to evaluate patients with solitary juxtapapillary capillary retinal angioma for the presence of von Hippel—Lindau disease (VHL). Methods: A retrospective case series of 11 patients, each presenting with a solitary juxtapapillary capillary retinal angioma, was examined. Patients were evaluated for type of angioma, presence of other VHL lesions, and mutations of the VHL gene. Results: Juxtapapillary angioma was exophytic in 7 patients and endophytic in 4 patients. VHL could be diagnosed in 7 patients (64%). Four patients were affected by VHL-related lesions as distinct from ocular angioma. A mutation of the VHL gene could be detected in 6 patients; in 1 of these patients, this mutation of the VHL gene was the only evidence of VHL.There was no difference in the age at manifestation or the type of juxtapapillary angioma in VHL patients compared with non-VHL patients. Interpretation: A solitary juxtapapillary angioma may indicate the presence ofVHL in a majority of patients, irrespective of the growth pattern of the tumour. Molecular genetic diagnostics is the most effective method of detecting VHL. Because of the high risk of the presence of other VHL lesions,thorough screening for VHL is mandatory for patients presenting with a solitary juxtapapillary angioma.

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.

Objective To characterize the clinical course of retinal angiomatosis (RA) in von Hippel–Lindau (VHL) disease. Design Retrospective observational case series from a tertiary referral center. Participants Fifty-seven consecutive VHL disease patients with RA with a mean follow-up of 7.3 years. Methods A retrospective chart review was performed to characterize the clinical course and functional outcome of RA to substantiate ophthalmic screening recommendations for VHL disease patients. Main Outcome Measures Age and visual acuity (VA) at diagnosis, angioma number, size, fundus position and growth behavior, functional outcome, and risk factors for adverse visual outcome (VA ≤20/1000) were evaluated. Results The onset of RA was observed to occur between the ages of 5.5 and 62.5 years. Ocular disease was unilateral in 58% of patients at diagnosis; prevalence of bilateral RA as calculated by Kaplan–Meier analysis was 100% at age 56.4 years. Twenty-seven eyes showed an adverse visual outcome, occurring at a mean age of 23.2 years. Risk factors included large angiomas at presentation, first manifestation at a younger age, and symptomatic RA. In most eyes, development of new angiomas was slow and only small angiomas were detected on annual follow-up. Eyes harboring multiple angiomas or RA complicated by retinal detachment were at risk of developing large angiomas after short follow-up intervals. Formation of new angiomas was largely independent of patient age. Conclusions Retinal angiomatosis in VHL disease bears a high risk of severe vision loss at a young age. In uncomplicated RA, annual ocular screening for presymptomatic angiomas is sufficient. Because RA can occur at any age, lifelong ocular screening is recommended in VHL disease gene carriers starting at preschool age.

Proton therapy for uveal melanoma provides high-conformal dose application to the target volume and, thus, an optimal saving of the organs at risk nearby. Treatment planning is done with the model-based treatment-planning system EYEPLAN. Tumor reconstruction is based only on a fundus composite, which often leads to an overestimation of the clinical target volume (CTV). The purpose was to exploit MRI on trial in a proton therapy-planning system by using the novel image-based treatment-planning system OCTOPUS. Ten patients with uveal melanomas received both a high-resolution planning CT and MRI of the eye. MR examinations were made with an eye coil. EYEPLAN requires eye geometry data for modeling, and tantalum marker clips for submillimeter positioning and additional information from ultrasound and 3-D imaging. By contrast, OCTOPUS provides the full integration of 3-D imaging (e. g., CT, MRI). CTVs were delineated in each slice. For all patients, CTVs (EYEPLAN vs. OCTOPUS) were compared intraindividually. OCTOPUS planning led to a mean reduction of the target volume by a factor of 1.7 (T1-weighted [T1w]) and 2.2 (T2w) without compromising safety. The corresponding field size could be scaled down on average by a factor of 1.2 (T1w) and 1.4 (T2w), respectively. Compared with the conventional EYEPLAN, MRI-based treatment planning of ocular tumors with OCTOPUS could be a powerful tool for reducing the CTV and, consequently, the treatment volume and the field size. This might be translated into a better patient compliance during treatment and a decreased late toxicity.

Das Aderhautmelanom unterscheidet sich in der Tumorbiologie und im Metastasierungsverhalten vom kutanen malignen Melanom. Das metastasierte Aderhautmelanom ist eine seltene Erkrankung mit schlechter Prognose. Zu den therapeutischen Moglichkeiten zahlen operative Verfahren, die nur fur sehr wenige Patienten sinnvoll sind, die systemische Chemotherapie und lokale Behandlungsverfahren wie die Chemoembolisation und die lokoregionare Chemotherapie uber die A. hepatica. Die wenigen vorliegenden klinischen Studien haben nur eine geringe Zahl von Patienten eingeschlossen. Sie belegen eine Wirksamkeit der Chemoembolisation und der lokoregionaren Chemotherapie bei Patienten mit alleiniger Metastasierung in die Leber. Bei Patienten mit Metastasierung in mehrere Organe werden zurzeit Zytostatikakombinationen aus Gemcitabin und Treosulfan in klinischen Studien untersucht. Fortemustin, eine moderne Nitroseharnstoffverbindung, stellt ebenfalls eine therapeutische Option dar.

Proton and stereotactic radiotherapy with photons (SRT) are both used to treat choroidal melanomas in proximity to optic disk and fovea centralis, a situation where plaque therapy is prone to complications. A comparative treatment– planning study was done to assess the capability of both modalities to preserve vision. In ten patients treated with 68–MeV protons, SRT with 6–MV photons was planned. Structures most important for visual acuity (fovea and optic disk, optic nerve) were contoured identically for both therapies. Safety margins of 1.5 mm for proton therapy were reduced to 1.0 mm for SRT. Proton–beam therapy was superior in eight of ten situations, and this result did not differ significantly by changes in the weighting of the different parameters analyzed. When dose deposition to those structures most important for the preservation of vision is taken into account, under the conditions examined proton therapy offers an advantage in the majority of the patients evaluated.

The purpose of this retrospective study was to determine the relevance of both visual-evoked potentials (VEP) and multifocal electroretinography (mfERG) to evaluate unexplained visual loss. Seventy-two consecutive patients (1996-2002) with visual disturbances of unknown origin underwent both VEP and mfERG (ISCEV standard). The mean age was 42.4 years (11.8-74.5) and median visual acuity 0.5 (no light perception - 1.0). Symptoms reported included visual acuity loss (n=69), visual field defects (n=11), disturbances of colour vision, light or dark adaptation (n=10). VEP and mfERG were normal in 43% (n=31). Both VEP and mfERG were pathological in 24% (n=17). In a further 18% (n=13) only the mfERG was pathological and in 15% (n=11) only the VEP was pathological. Macular dysfunction as detected with mfERG was present in 73% of 41 patients with at least one pathological test. Neuroimaging (MRI, CCT) and/or neurological examination was performed in 27/72 patients (38%), to account for unexplained visual loss, prior to the electrophysiological tests; these were normal in all patients. Electrophysiological tests revealed disturbances of the post-retinal visual pathway in only 3/27 patients. In 12/27 patients, mfERG revealed a macular disorder; in a further 12/27 patients VEP and mfERG were normal. The combined evaluation of VEP and mfERG is useful both to establish the area of dysfunction and the normality of the visual system. Electrophysiological testing prior to neuroimaging is recommended for patients where clear clinical signs of cerebral disorders are not evident. This reduces the frequency of unnecessary neuroimaging and associated radiation exposure.

Primary pars plana vitrectomy (PPPV) has gained widespread popularity in the treatment of rhegmatogenous retinal detachments (RRD). However, the surgical procedure is still flawed by a significant rate of anatomical and functional failures. The study was conducted to analyse the risk factors for a dissatisfying postoperative outcome.We carried out a retrospective study of 512 cases of PPPV with a minimum follow-up of 3 months from one institution over a 9-year period. Preoperatively, 24.8% of patients (127 out of 512) were pseudophakic, 16.4% (84 out of 512) highly myopic, 19.9% (102 out of 512) had preoperative proliferative vitreoretinopathy (PVR) and 14.6% (75 out of 512) had vitreous haemorrhage.The follow-up period ranged from 3 to 108 months (median 14.8). Retinal reattachment was achieved with one operation in 70.7% (362 out of 512) and after one or more operations in 97.5% of cases (499 out of 512). The major reasons for redetachments were new retinal breaks, followed by a combination of new breaks and PVR, and PVR without apparent breaks. Postoperative visual acuities ofor =0.1 andor =0.4 were achieved in 82.8% (424 out of 512) and 48.2% (247 out of 512) respectively. Out of 376 phakic patients at study entry, 66.4% (250 out of 376) underwent cataract surgery either in combination with PPPV or during the postoperative course. Factors that were significantly associated with either anatomical or functional failure included duration of symptoms, low preoperative visual acuity, myopia, amblyopia, hypotony, macular detachment, preoperative PVR, extent of detachment, involvement of inferior quadrants, no detectable breaks, large breaks, breaks posterior to the equator, surgeon, level of surgical training, endocryotherapy, and combined scleral buckling surgery.Primary pars plana vitrectomy is still flawed by a relatively high primary redetachment rate following the initial procedure. The advantages of the technique are a high final reattachment rate and relatively good functional results in a subset of patients with more complicated types of RRD. The risk factors for postoperative failures following PPPV for RRD match to a large extent those following scleral buckling surgery (SBS). Future improvements of the technique will have to focus on modifiable risk factors, such as details of the surgical procedures, surgical training and case selection, to distinguish it from SBS.

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.

Choroidal neovascularizations (CNV) is the major cause of significant visual loss in patients with angioid streaks. We evaluated the functional and morphological outcome of Verteporfin photodynamic therapy (PDT) in the treatment of these patients.This was a retrospective study in two tertiary referral centres over a 3-year period. Examinations included visual acuity assessment with ETDRS charts, binocular fundoscopy and fluorescein angiography. PDT was performed with standard parameters; earlier retreatments were feasible in active CNV.Fifteen eyes from 12 patients (9 male, 3 female) with a follow-up of 12-50 months (mean 26.1, median 19 months) were included. Five lesions were extra-or juxtafoveal and ten were subfoveal. Baseline visual acuity was between 20/63 and 20/16 (mean 20/32, median 20/32). Eyes were treated with two to eight treatments of PDT (mean 4.2, median 4). Treatment intervals were between 5.6 and 72 weeks (mean 12.1, median 9.2 weeks). At the 1-year follow-up, visual acuity was below 20/200 in 27% (4/15), 20/200 or better in 73% (11/15) and 20/63 or better in 47% (7/15) with an improvement of3 lines in 13% (2/15), no change in 27% (4/15) and a decrease of3 lines in 60% (9/15). At the final follow-up examination, all lesions were located subfoveally. Visual acuity was below 20/200 in 47% (7/15), 20/200 or better in 53% (8/15) and 20/63 or better in 13% (2/15) with a change in visual acuity between +2 and -18 lines (mean -9 lines, median -8 lines). No change was noted in 7% (1/15) and a decrease of3 lines in 93% (14/15) of eyes. The maximum measured greatest linear dimension of the lesion during the follow-up varied between 2400 microm and 6200 microm (mean 3680 microm, median 3600 microm) with an increase in the lesion size compared with baseline values between +/-0 microm and +3700 microm (mean+1420 microm, median+1500 microm).PDT for CNV associated with angioid streaks seemed to slow down but not prevent the progression of the disease and associated visual loss. Further modifications of the treatments parameters or a combination with other therapeutical options seem warranted for a more effective treatment of these lesions.

Nordic ophthalmologists and vision scientists are active in many fields of eye research. This is most evident at the biannual Nordic Congress of Ophthalmology, most recently held in Malmo in June 2004. The authors here review some of the research in vision and ophthalmology presented at this meeting or published recently by Nordic scientists. This paper does not represent a comprehensive review of all Nordic research in the field, but attempts to give an overview of some of the activities underway in eye research in this part of the world.

Hintergrund Die photodynamische Therapie (PDT) mit Verteporfin wurde erfolgreich zur Behandlung von ≥50% der klassischen choroidalen Neovaskularisationsmembranen (CNV) bei exsudativer altersabhangiger Makuladegeneration (AMD) und anderer Genese eingefuhrt. Unklar ist z. Z. jedoch, ob Patienten mit subfovealen CNV-Rezidiven nach vorangegangener Laserkoagulation einer CNV ebenfalls von der PDT profitieren.

PURPOSE Enucleation is the main form of treatment for advanced retinoblastoma. The major complication of this procedure is orbital implant exposure. Different implants and wrapping materials are currently in use. The aim of the current study was to analyze the complications associated with the use of polyester-urethane, an artificial dura substitute, as a wrapping material for enucleation in advanced retinoblastoma. METHODS A retrospective review of 32 cases (28 patients), who were treated with enucleation for advanced retinoblastoma, was performed. The age of the patients ranged between 3 months and 6.7 years (median, 19 months). Additional chemotherapy was administered in 12 cases. The removed eyeball was replaced either with a silicone implant and polyester-urethane wrapping (13 cases) or hydroxyapatite, silicone-hydroxyapatite, or polyethylene implants without additional wrappings (19 cases). The follow-up period ranged from 7 months to 5.9 years (median, 22.4 months). Statistical analysis was performed using the Kaplan-Meier method. RESULTS Single or multiple exposures occurred in 22% of cases (7/32). There were 6 exposures (46%, 6/13) in the group with polyester-urethane wrapping compared with only 1 exposure (5%, 1/19) in the implants without wrapping. This difference was statistically significant (p=0.0236). None of the other analyzed factors (additional chemotherapy, surgeon, age of the patient, or size of the implant) demonstrated a significant correlation to implant exposures. CONCLUSIONS Wrapping of orbital implants with polyester-urethane resulted in a high rate of implant exposures after enucleation for advanced retinoblastoma. In this series, the best results were achieved with integrated implants without additional wrapping.

Ziel dieser Studie ist es, zu untersuchen, ob mittels einer photodynamischen Therapie (PDT) mit Verteporfin eine Tumorzellnekrose beim humanen Aderhautmelanom induziert werden kann. Bei 4 Augen mit einem nicht bulbuserhaltend therapierbaren malignen Melanom der Aderhaut wurde 2–3 Tage vor der geplanten Enukleation das Melanom mit einer PDT behandelt. Die Bulbi wurden histologisch untersucht. Bei einer Lichtdosis ≥100 J/cm2 konnten bei 2 weniger stark pigmentierten Tumoren nach der PDT Effekte im Tumorgewebe bis 2,5 mm Tiefe gefunden werden. Histologisch zeigten sich Gefasverschlusse und Thrombosen in den Tumorgefasen. Bei starker Pigmentierung konnte keine Tumorzellnekrose induziert werden. In Abhangigkeit von den Behandlungsparametern und dem Pigmentierungsgrad des Tumors lasst sich mit der PDT mit Verteporfin eine Nekrose im humanen Aderhautmelanom erzeugen. Evtl. kann die PDT mit angepassten Parametern als adjuvante Therapie bei der Behandlung von wenig pigmentierten okularen Malignomen eingesetzt werden.

To clarify the potential role of endothelin-1 (ET-1) in the pathogenesis of glaucoma, the endothelin receptors expressed in bovine trabecular meshwork (TM) and ciliary muscle (CM) were identified. TM and CM strips were subjected to ET-1 as well as to specific endothelin receptor antagonists. In both tissues BQ123, a specific ET-A receptor antagonist, substantially inhibited ET-1-induced contraction. BQ788, a specific ET-B receptor antagonist, showed only moderate effects. Both ET receptor types were detected in bovine TM and CM using Western blot analysis. ET-1 produced an increase in intracellular calcium in cultured TM cells. This effect was inhibited by BQ123, but not by BQ788. Thus, although both receptors are present, the ET-A receptor appears to play the predominant role in mediating contraction in both the TM and CM, while the ET-B receptor seems to contribute little to the overall ET-1 effect.

Vergleich der anatomischen und funktionellen Ergebnisse nach einer Laser- oder Kryokoagulationsbehandlung aufgrund einer Fruhgeborenenretinopathie. Zwischen April 1991 und Februar 2002 wurden 127 Augen (65 Sauglinge) nach Erreichen der Schwellenkriterien zur Behandlung entweder kryo- (46 Augen bis Marz 1993) oder laserkoaguliert (81 Augen). Zusatzlich wurden 10 Augen (5 Sauglinge) mit einer Vaskularisationsgrenze in der Zone I bereits zum Zeitpunkt von „prethreshold“ laserkoaguliert. Ein „unfavorable outcome“, entsprechend der Cryo-ROP-Studie, bestand bei 1/91 (1%) laser- und 3/46 (6,5%) kryokoagulierten Augen. Eine Gefaswinkelverengung bzw. eine Makulaverlagerung wurde bei 6/91 (6,6%) der laser- und in 7/46 (15,2%) der kryokoagulierten Augen beobachtet. Ein Visus von mindestens 0,8 wurde bei den laserkoagulierten Augen signifikant haufiger erreicht (39,2% in der Lasergruppe, 17,6% in der Kryogruppe). Durch eine rechtzeitige Koagulation nach Erreichen der Schwellenkriterien zur Behandlung kann ein sehr gutes anatomisches und funktionelles Ergebnis erzielt werden. Die Resultate nach Laserkoagulation sind denen nach Kryokoagulation uberlegen, sodass die Laserkoagulation die Therapie der Wahl ist.

The aim of this study was to analyse 1- and 2-year outcomes after photodynamic therapy (PDT) in clinical routine outside of the TAP [treatment of age-related macular degeneration (AMD) with PDT] study. We analysed the functional results, possible influencing factors and the rate of side effects.We analysed the medical records of 210 consecutive patients between 50 and 93 years of age (73+/-9 years) who had been treated with PDT for activeor =50% classic CNV resulting from AMD. Only patients with a minimum follow-up of 1 year (127) were included; 52 patients completed 2 years of follow-up. Juxta- and extrafoveal CNV were also analysed. Treatment was given in accordance with TAP parameters and regular follow-up examinations were performed with standardised ETDRS visual acuity (VA) measurements and fluorescein angiography.In the subfoveal group, in 63.6% (70/110) a loss of VAor =3 lines could be prevented after 1 year, and in 51.1% (23/45) after 2 years. An improvement ofor =1 line was found in 31.8% (1 year) and in 22.2% of eyes (2 years). Severe VA loss ofor =6 lines occurred in 10.9% of cases after 1 year and in 15.6% after 2 years. The mean change of VA was -1.7+/-3.4 lines (1 year) and -2.5+/-3.9 lines (2 years). For the group of CNV with juxta-/extrafoveal localisation, the mean change of VA was +0.8+/-2.5 lines after 1 year and +1.0+/-4.2 lines after 2 years. With regard to different CNV localisations, the results for juxta-/extrafoveal CNV are statistical significantly better (p=0.005 and p=0.035 after 1 and 2 years, respectively). A mean of 2.6 treatments were performed in the first year and 0.5 in the second year.The results obtained in a single institution compare favourably with the results of the TAP study. The results regarding functional visual outcome could be obtained with a lower number of treatments in clinical practice. Juxta- and extrafoveal CNV showed significantly better results than a subfoveal localisation of the CNV. In this subgroup a mean improvement of VA could be obtained after 1 or 2 years.

Purpose: Detailed morphologic and functional evaluation of adult vitelliform macular dystrophy (AVMD). Methods: The records of 61 consecutive AVMD patients (inclusion criterion: vitelliform lesion smaller than one disk diameter at least in one eye) were evaluated retrospectively regarding visual acuity, color vision, perimetry, retinal pigment epithelium (RPE) autofluorescence, fluorescein angiography, electro-oculography, full-field and multifocal electroretinography, and molecular genetic evaluation of the VMD2 and RDS/peripherin genes. Results: The mean age of subjects was 54.6 years. Visual loss was variable (median, 0.6; range, 1.25–0.05). Color vision and visual field were normal in about half of the patients but presented defects with high variability in the remaining patients. Autofluorescence findings showed increased fluorescence within the foveal yellow lesion in 76%. In the majority of eyes, the amplitude of the 30 Hz flicker response of the full-field electroretinogram (72%) and the central P1 amplitude of the multifocal electroretinogram (63%) were reduced. Mutational analyses revealed a potentially disease-associated mutation in the RDS/peripherin gene in one patient. Conclusion: AVMD is characterized by late onset, slow progression, good prognosis, and high variability of morphologic and functional abnormalities resulting frequently in misdiagnosis. Autofluorescence findings indicate lipofuscin accumulation in the yellow lesion. Electroretinography revealed a generalized cone system dysfunction with increasing severity toward the fovea.

In June 1998, proton-beam therapy of ocular tumors started at the Hahn-Meitner Institute Berlin, Germany. The purpose of the present study is to evaluate treatment outcome for uveal melanomas. 245 consecutive patients with primary melanoma of the uvea were treated from June 1998 to April 2003 with a 68-MeV proton beam. In 96.2% of all patients, a uniform fractionation scheme was applied: single dose 15 CGE (cobalt gray equivalent), total dose 60 CGE on 4 consecutive days. Follow-up is available in 229 patients. At the time of median follow-up (18.4 months), local control is 96.4% and 95.5% at 3 years. Eye retention rate is 92.6% at 20 months (median follow-up) and 87.5% at 3 years. Proton-beam irradiation of uveal melanomas at the Hahn-Meitner Institute after the first 5 years of its initiation reveals local tumor control and eye retention rates in the range of other centers with larger experience. Delivering high treatment quality in hadron therapy from the beginning has been achieved.

Hintergrund Ziel dieser Studie ist die Evaluation der photodynamischen Therapie (PDT) hinsichtlich ihrer Sicherheit und Effektivitat bei der Behandlung idiopathischer chorioidaler Neovaskularisationen (CNV).

Hintergrund Grose uveale Melanome, die sich in der Nahe der Papille und/ oder der Fovea befinden, haben wegen der zu erwartenden Spatkomplikationen eine ungunstige Prognose bezuglich des Visus- und Bulbuserhaltes. Die Endoresektion dieser Tumoren ist eine neue Behandlungsmodalitat, die moglicherweise Vorteile in der Therapie dieser komplizierten Falle bieten wird.