[Progressive maculopathy despite discontinuation of chloroquine treatment-multimodal imaging and review of the literature]. / Progressive Makulopathie trotz Absetzen der Chloroquin-Therapie ­ Multimodale Bildgebung und Review der Literatur.

This case report presents a pictorially well-documented case with multimodal images of a progression of maculopathy despite discontinuation of chloroquine treatment in a long-term follow-up over 6 years. Additionally, the current detection methods in screening for early recognition of maculopathy and their prognostic significance are reviewed.

Intravitreal bevacizumab for choroidal neovascularisation associated with pseudoxanthoma elasticum.

PURPOSE: To investigate the efficacy of intravitreal bevacizumab injections for treating choroidal neovascularisation (CNV) secondary to pseudoxanthoma elasticum (PXE). METHODS: Patients with active CNV due to PXE received intravitreal bevacizumab (1.5 mg) and were reviewed at monthly intervals. Further treatments were administered depending on disease activity (visual loss of 5 letters or one line, persistent leakage, persistent macular oedema). Baseline and 1-3 monthly follow-up examinations included best corrected visual acuity (BCVA), biomicroscopy, optical coherence tomography (OCT), fluorescein and indocyanine green angiography, fundus autofluorescence and digital fundus photography. RESULTS: 15 patients (16 eyes) with CNV and PXE were treated. Mean (SD) age was 53 (12.3) years (range 24-72). Mean BCVA at baseline was 20/100 (mean (SD) LogMAR 0.68 (0.51)), improved to 20/63 after the first injection (LogMAR 0.49 (0.45); p = 0.028), and was 20/63 (LogMAR 0.48 (0.48); p = 0.126) at the last follow-up. The mean follow-up time was 8 months. Central retinal thickness decreased significantly from 252 mum at baseline to 214 mum at the last follow-up (p = 0.004) as measured by OCT. Eyes were injected an average of 2.4 times. Categorising patients into two groups (group 1 with only mild changes and group 2 with evident morphological changes in the central macula) revealed that group 1 improved significantly more (LogMAR range 0.41-0.06) than group 2 (LogMAR range 0.80-0.66) (p = 0.014). CONCLUSIONS: The results indicate that intravitreal bevacizumab is effective both functionally and morphologically in treating CNV due to PXE. Best recovery can be achieved in eyes with disease that has not progressed too far and if treatment is initiated at the earliest point possible.

[Ocular manifestations and surgical results in patients with Marfan syndrome]. / Okuläre Manifestationen und chirurgische Ergebnisse beim Marfan-Syndrom.

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.

[Bevacizumab for treatment of macular edema secondary to retinal vein occlusion]. / Bevacizumab zur Therapie des sekundären Makulaödems nach venösen Gefässverschlüssen.

Application of VEGF inhibitors represents a treatment option for macular edema secondary to retinal vein occlusion that targets the disease at the causal molecular level. First reports on intravitreal injections of bevacizumab show promising morphological and functional effects and demonstrate that bevacizumab is a potent antiedematous agent in this context. A significant reduction of the central retinal thickness followed by a rapid improvement of visual acuity may be achieved within days. In a pilot study with a review period of 3 months, we found a significant improvement of one or more lines in 93% and four or more lines in 27% of eyes. This was associated with a concomitant significant reduction in central retinal thickness, which, however, was not sustained by a single injection (64% reduction after 1 month and 28% after 3 months). No relevant adverse events were noted. The duration of action after intravitreal bevacizumab administration is currently unknown. Reinjections will be necessary to maintain a lasting beneficial effect. Prospective, controlled long-term studies are mandatory to develop standardized treatment protocols that allow a safe and effective application of this off-label therapy.

[Intravitreal bevacizumab for neovascular age-related macular degeneration]. / Intravitreales Bevacizumab bei der neovaskulären altersabhängigen Makuladegeneration.

The efficacy and safety of the therapeutic anti-VEGF concept has already been demonstrated for pegaptanib and ranibizumab. Bevacizumab acts as an antibody against all VEGF-A isoforms and has been developed for oncological indications with intravenous application. Initial reports on intravitreal administration in patients with neovascular age-related macular disease (AMD) have shown beneficial morphological and functional effects. In the meantime, bevacizumab has been used off-label in thousands of patients with AMD. However, data from prospective, controlled, randomized trials on both safety and efficacy are lacking. Herein recent experiences with bevacizumab are summarized and discussed. Furthermore, a web-based platform for online data registration and pooled analyses is presented.

[Pseudoxanthoma elasticum]. / Pseudoxanthoma elasticum: Genetische Grundlagen, Manifestationen und therapeutische Ansätze.

Pseudoxanthoma elasticum (PXE) is an inherited disorder that is associated with accumulation of mineralized and fragmented elastic fibers in the skin, vessel walls, and Bruch's membrane. Clinically, patients exhibit characteristic lesions of the skin (soft, ivory-colored papules in a reticular pattern that predominantly affect the neck), the posterior segment of the eye (peau d'orange, angioid streaks, choroidal neovascularizations), and the cardiovascular system (peripheral arterial occlusive disease, coronary occlusion, gastrointestinal bleeding). There is no causal therapy. Recent studies suggest that PXE is inherited almost exclusively as an autosomal recessive trait. Its prevalence has been estimated to be 1:25,000-100,000. The ABCC6 gene on chromosome 16p13.1 is associated with the disease. Mutations within the ABCC6 gene cause reduced or absent transmembraneous transport that leads to accumulation of substrate and calcification of elastic fibers. Although based on clinical features the diagnosis appears readily possible, variability in phenotypic expressions and the low prevalence may be responsible that the disease is underdiagnosed. This review covers current knowledge of PXE and presents therapeutic approaches.

Prostaglandin E1 infusion therapy in dry age-related macular degeneration.

OBJECTIVE: The pilot study is intended to show whether prostaglandin E1 (PGE1) infusions are able to stop the gradual vision loss in dry age-related macular degeneration (AMD) and, further, to stabilize or improve visual acuity. METHODS: With PGE1 infusions 11 patients with different forms of dry AMD were treated and compared with a control group of 10 untreated patients with dry AMD. The target parameter was the visual acuity, as determined with the ETDRS logMAR charts. Other examinations performed during the study were tests of contrast vision, colour vision and central visual fields, as well as autofluorescence and fluorescein angiography and multifocal electroretinography. RESULTS: On termination of the infusions, six patients showed an increase in visual acuity by at least one line, an improvement that was seen in eight patients 2 months after the end of the infusion therapy. After 6 months, one patient exhibited an improvement of visual acuity by three lines and three patients an improvement by one line. Five patients were found to show no change of their baseline acuity values after 6 months, while two patients exhibited an impairment by one line. The visual acuity in the dry AMD control group without PGE1 treatment had decreased by 0.8 lines on the average after 6 months. Contrast vision, central visual fields and the multifocal electroretinogram showed improvements on the termination of infusions and up to 2 months later; no substantial change of these parameters, as compared with the baseline findings, was seen 6 months after the termination of infusions. SUMMARY: This pilot study suggests that PGE1 infusions have a stabilizing or improving effect on the visual acuity of patients with dry AMD. Owing to the limitations of a pilot study, these results should, however, be validated in a larger, randomized and blinded study.