Discrete arcs of increased fundus autofluorescence in retinal dystrophies and functional correlate on microperimetry.

PURPOSE: To describe the occurrence of discrete arcs of increased fundus autofluorescence (FAF) associated with various retinal dystrophies and to assess their functional significance by fundus-controlled microperimetry. METHODS: Seven patients, three with pigmented paravenous retinochoroidal atrophy (PPRCA), one with sector retinitis pigmentosa (RP), one with typical RP, and two with macular dystrophy were assessed by retinal imaging including FAF imaging. Serial images were obtained within a review period of 6 and 10 years in a patient with PPRCA and macular dystrophy, respectively. Fundus-controlled microperimetry was performed in eight eyes of five patients to determine light increment sensitivity. RESULTS: A discrete arched line of increased FAF was observed without obvious correlate on fundus biomicroscopy. The orientation of this line differed from ring shape in RP and macular dystrophy, a semi-circle structure in sector RP to crescent shape with tiplike extensions towards branching retinal veins in PPRCA. Longitudinal investigation revealed slight migration of the arc in PPRCA and peripheral extension of the ring diameter in macular dystrophy. Microperimetry revealed that the arc of increased FAF sharply delineated areas of severely impaired retinal sensitivity. CONCLUSIONS: The findings indicate that arcs of increased FAF in PPRCA and other retinal dystrophies demarcate areas of impaired retinal function and may migrate over time. FAF imaging may therefore reveal the exact extent of neurosensory dysfunction that may exceed the dimensions anticipated by conventional examinations.

[Monitoring of AMD patients on anti-vascular endothelial growth factor (VEGF) treatment. Practical notes on functional and anatomical examination parameters from drug approval studies, specialist information and case series]. / Monitoring von AMD-Patienten unter Anti-VEGF-Therapie. Praktische Hinweise zu funktionellen und anatomischen Untersuchungsparametern aus Zulassungsstudien, Fachinformationen und Fallserien.

Age-related macular degeneration (AMD) is one of the most common causes of blindness in western industrialised nations. Most AMD patients suffer from the dry early form of AMD; however, wet AMD with choroidal neovascularization (CNV) is the main cause of blindness in all AMD patients. New prospects have been developed in AMD treatment using pharmacological methods available for treating all subtypes of exudative AMD. A number of inhibiting and inducing growth factors, such as vascular endothelial growth factor (VEGF), are particularly important in the pathophysiology of wet AMD. The secreted VEGF appears to play a crucial role in the pathogenesis of CNV and macular edemas as a result of its angiogenetic and permeability-enhancing effect. This recognition led to the treatment approach now used, i.e., competitive VEGF blocking through intravitreal adminsitration of anti-VEGF drugs. The anti-VEGF durgs lead to a rapid decrease in retinal thickness. Optical coherence tomography (OCT) is a valuable monitoring tool, but may only be used to assist in decision-making. Clinical follow-up of patients and further treatment recommendations must always be guided by the overall clinical picture. Visual acuity is regarded as the decisive criterion for repeat treatment.

[Unilateral pigmented paravenous retinochoroidal atrophy]. / Unilaterale pigmentierte paravenöse retinochorioidale Atrophie.

We report the case of an asymptomatic unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) in a 43-year-old patient. The right eye showed chorioretinal atrophy with bone-spicule-like pigmentations along the retinal veins. Visual acuity was 20 / 20 and perimetry revealed scotomas correlating to the chorioretinal atrophy. Electrophysiological examination showed decreased signals in ERG and EOG. Fundus autofluorescence and angiography findings are presented. Pathogenetically, a classification as hereditary retinal dystrophy (as in retinitis pigmentosa) as well as a post-inflammatory residuum are discussed.

[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.

[Modern pharmacotherapy of age-related macular degeneration]. / Moderne Arzneimitteltherapie der altersabhängigen Makuladegeneration.

Age-related macular degeneration (AMD) is now the most common cause for blind registration in all developed countries. Epidemiologic data indicate that there are 4.5 millions affected in Germany with constant increase in incidence and prevalence with subsequent considerable health economic implications. Late manifestations of the disease result in the inability to read and to perform daily tasks. Therefore, there is an urgent need for efficacious prophylactic and therapeutic measures to prevent irreversible loss of central vision. Based on a better understanding of the underlying molecular mechanisms new therapeutic approaches have been brought forward and expand previous approaches such as thermal laser surgery or photodynamic therapy. Repeated intravitreal injection of anti-VEGF (vascular endothelial growth factor) agents as well as corticosteroids have a beneficial effect on growth and permeability of neovascular membranes. The risk for progression from early to late stages of AMD can be reduced with certain antioxidative preparations (AREDS medication) in presence of defined funduscopic signs. Early diagnosis is key for all currently available interventions since a beneficial effect can only be achieved in early stages of the disease process.

Lower limits of fluorescein and indocyanine green dye for digital cSLO fluorescence angiography.

BACKGROUND: With the advent of digital confocal scanning laser ophthalmoscopy it is possible to detect low levels of fluorescence. Here we used a novel confocal scanning laser ophthalmoscope (cSLO) to determine lower limits of dye required for fluorescein (FL) and indocyanine green (ICG) angiography. METHODS: A cSLO (Heidelberg retina angiograph 2, Heidelberg Engineering, Dossenheim, Germany) with an optically pumped solid state laser (488 nm) for FL and a diode laser (790 nm) for ICG angiography (FL/ICG-A) was used. 62 FL-As were performed in 53 patients and 45 ICG-As were performed in 39 patients with neovascular age related macular degeneration. The volume and overall dye content of bolus injections was gradually tapered (FL: 500 mg, 250 mg, 200 mg, 166 mg, 100 mg; ICG: 25 mg, 20 mg, 15 mg, 10 mg, 5 mg, 2.5 mg), while dye concentrations were kept constant at 100 mg/ml for FL and at 5 mg/ml for ICG. Images were obtained 1, 5, 15, and 30 minutes after dye injection. Image quality was evaluated by two independent readers using standardised criteria. RESULTS: For amounts down to 166 mg for FL and to 5 mg for ICG, sufficient image quality was achieved during all phases following injection. Only late phase images showed less contrast compared to typically used dye amounts, which was irrelevant for interpretation and clinical management. CONCLUSIONS: With the increased sensitivity of this novel cSLO system, amounts of injected dye during FL-A can be reduced to one third for FL and to one fifth for ICG without relevant loss of image quality or information compared to conventionally used dye levels. These amounts can be used for routine angiography and allow relevant savings for units performing FL-A.