Concordance of disease progression in bilateral geographic atrophy due to AMD.

Purpose: To determine the degree of concordance for progression rate, size of atrophy, and visual acuity in patients with bilateral geographic atrophy (GA) due to age-related macular degeneration (AMD).

Methods: Analysis was performed in 156 eyes of 78 patients with bilateral GA. Best corrected visual acuity was determined with ETDRS charts.

Metamorphopsia in patients with macular telangiectasia type 2.

Purpose: To assess metamorphopsia in patients with macular telangiectasia (MacTel) type 2.

Methods: In a prospective observational cohort study, 40 eyes of 20 patients with MacTel type 2 were investigated by funduscopy, fluorescein angiography, optical coherence tomography and microperimetry. Metamorphopsia was assessed using Amsler grids following a standard protocol and standardized questionnaire.

Clinical evaluation of simultaneous confocal scanning laser ophthalmoscopy imaging combined with high-resolution, spectral-domain optical coherence tomography.

Purpose: To evaluate the clinical relevance of a new diagnostic modality, simultaneous confocal scanning laser ophthalmoscopy (cSLO) and high-speed, high-resolution, spectral-domain optical coherence tomography (OCT), for the visualization of macular pathologies.

Methods: OCT images and simultaneous recording of fluorescein angiography, indocyanine green (ICG) angiography, infrared, and blue reflectance ('red-free') or fundus autofluorecence (FAF) images were obtained with a novel imaging device (Spectralis HRA + OCT; Heidelberg Engineering, Heidelberg, Germany). An optically pumped solid-state laser generated the excitation wavelength (488 nm) required for blue reflectance, FAF and fluorescein angiography images.

Multimodal imaging including spectral domain OCT and confocal near infrared reflectance for characterization of outer retinal pathology in pseudoxanthoma elasticum.

Purpose: To investigate the value of multimodal confocal scanning laser ophthalmoscopy (cSLO) for phenotyping fundus lesions in patients with pseudoxanthoma elasticum (PXE) and to correlate these findings with morphologic alterations detected by spectral domain optical coherence tomography (SD-OCT).

Methods: Imaging was performed with a combined SD-OCT-cSLO system (Spectralis HRA-OCT; Heidelberg Engineering, Heidelberg, Germany). OCT scans were placed at locations of interest on near-infrared (NIR) reflectance, fundus autofluorescence (FAF), and fluorescein angiography (FA) images.

Characterisation of severe rod-cone dystrophy in a consanguineous family with a splice site mutation in the MERTK gene.

Aim: To characterise the ocular phenotype of a family segregating the splice site mutation c.2189+1G>T in the tyrosine kinase receptor gene MERTK.

Methods: Five affected children of a consanguineous Moroccan family were investigated by ophthalmic examinations, including fundus photography, autofluorescence (FAF) imaging, optical coherence tomography (OCT), psychophysical and electrophysiological methods.

In vivo imaging of foveal sparing in geographic atrophy secondary to age-related macular degeneration.

Purpose: To investigate morphologic alterations in geographic atrophy caused by age-related macular degeneration (AMD) in the presence of foveal sparing using high-resolution in vivo imaging.

Methods: Simultaneous spectral domain optical coherence tomography (SD-OCT, 870 nm, 40,000 A-scans/s) and confocal scanning laser ophthalmoscopy (cSLO; fundus autofluorescence; excitation, 488 nm; emission, 500-700 nm) were performed in 18 eyes with geographic atrophy and foveal sparing using a combined instrument. Anatomic layers were evaluated, and retinal thickness in the fovea and the peripheral macula were measured and compared with those in controls of similar age.

Pseudoxanthoma elasticum: genetics, clinical manifestations and therapeutic approaches.

Pseudoxanthoma elasticum (PXE) is an inherited disorder that is associated with accumulation of mineralized and fragmented elastic fibers in the skin, vascular walls, and Bruch's membrane in the eye. Clinically, patients exhibit characteristic lesions of the posterior segment of the eye including peau d'orange, angioid streaks, and choroidal neovascularisations, of the skin including soft, ivory-colored papules in a reticular pattern that predominantly affect the neck and large flexor surfaces, and of the cardiovascular system with peripheral and coronary arterial occlusive disease as well as gastrointestinal bleedings. There is yet no definitive therapy.

Quantification of reduced macular pigment optical density in the central retina in macular telangiectasia type 2.

Recently, a unique distribution, namely a reduction of macular pigment optical density (MPOD) within the central retina with a surrounding ring-like structure of preserved MPOD at about 6 degrees eccentricity was suggested to be a common finding in macular telangiectasia (MacTel) type 2. In order to quantify this reduced MPOD, 28 eyes of 14 patients with MacTel type 2 were investigated by fundus reflectometry and two wavelengths fundus autofluorescence (FAF; at 488 nm and 514 nm). Fundus reflectometry showed a reduced MPOD within the central 4 degrees eccentricity that was most absent temporal to the foveola.

Fundus autofluorescence and progression of age-related macular degeneration.

Fundus autofluorescence imaging is an imaging method that provides additional information compared to conventional imaging techniques. It permits to topographically map lipofuscin distribution of the retinal pigment epithelial cell monolayer. Excessive accumulation of lipofuscin granules in the lysosomal compartment of retinal pigment epithelium cells represents a common downstream pathogenetic pathway in various hereditary and complex retinal diseases including age-related macular degeneration (AMD).

Confocal scanning laser ophthalmoscopy findings in chronic solar retinopathy.

A 68-year-old man with solar retinopathy had a lamellar foveal lesion on ophthalmoscopy and optical coherence tomography in one eye while the fellow eye appeared unremarkable. However, macular imaging by means of confocal scanning laser ophthalmoscopy (reflectance imaging and autofluorescence) revealed alterations in both eyes. Visual deterioration due to solar retinopathy may be a diagnostic challenge and the patient's history may be incomplete.

Reading performance is reduced by parafoveal scotomas in patients with macular telangiectasia type 2.

Purpose: Macular telangiectasia (MacTel) type 2 typically exhibits sharply demarcated parafoveal scotomas. In an investigation of their significance for reading performance, reading acuity and speed were measured and correlated with parafoveal sensitivity and fixation stability.

Methods: In this prospective controlled cross-sectional observational study, 49 eyes of 26 patients with MacTel type 2 were investigated.

[Neovascular age-related macular degeneration in Germany. Encroachment on the quality of life and the financial implications].

Background: Approximately 35,000 cases of neovascular age-related macular degeneration (AMD) occur annually in Germany. The neovascular form of AMD (NV-AMD) is responsible for severe vision loss associated with the disease in 90% of the cases. This study was conducted to assess the humanistic and economic burden of NV-AMD in the German population.

[Patient reported outcomes. Relevance and application in ophthalmology].

The use of patient-reported outcomes (PROs) - standardized assessments by the patient of the impact of illness or therapy - has been met with increasing interest over the last years. Evidence generated from PROs is used not only to support claims in the drug regulatory process but is also used in political decision making and activities by the pharmaceutical industry. PROs can reflect the benefits of a treatment very well because the patient's quality of life is a central variable, and its preservation or improvement is certainly the main objective of any therapeutic intervention.

Systemic complement activation in age-related macular degeneration.

Dysregulation of the alternative pathway (AP) of complement cascade has been implicated in the pathogenesis of age-related macular degeneration (AMD), the leading cause of blindness in the elderly. To further test the hypothesis that defective control of complement activation underlies AMD, parameters of complement activation in blood plasma were determined together with disease-associated genetic markers in AMD patients. Plasma concentrations of activation products C3d, Ba, C3a, C5a, SC5b-9, substrate proteins C3, C4, factor B and regulators factor H and factor D were quantified in patients (n = 112) and controls (n = 67).

Eighteen-month follow-up of intravitreal bevacizumab in type 2 idiopathic macular telangiectasia.

Aim: To evaluate the effects of intravitreal bevacizumab for non-proliferative type 2 idiopathic macular telangiectasia (type 2 IMT) within a mean follow-up period of 18 months.

Methods: The authors retrospectively studied six eyes of five patients with type 2 IMT who received two doses of intravitreal bevacizumab (1.5 mg) at a 4-week interval, followed by further applications depending on disease activity.

Abnormal macular pigment distribution in type 2 idiopathic macular telangiectasia.

Purpose: To determine the distribution of macular pigment in type 2 idiopathic macular telangiectasia (IMT).

Methods: Twenty-two eyes of 12 patients with type 2 IMT were examined by means of best-corrected visual acuity testing, fundus biomicroscopy, fundus photography, fluorescein angiography, and optical coherence tomography. Macular pigment optical density (MPOD) was assessed using a modified confocal scanning laser ophthalmoscope whereby MPOD was calculated from fundus autofluorescence images acquired at two different excitation wavelengths (488 and 514 nm).

High-resolution spectral domain-OCT imaging in geographic atrophy associated with age-related macular degeneration.

Purpose: To describe morphologic variations in outer retinal layers in eyes with atrophic age-related macular degeneration (AMD) using high-resolution, spectral-domain optical coherence tomography (SD-OCT).

Methods: SD-OCT scans were obtained with a combined confocal scanning laser ophthalmoscope (cSLO) and SD-OCT for simultaneous tomographic and topographic in vivo imaging. A total of 81 eyes of 56 patients (mean age, 77.

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.

Quality of life in age-related macular degeneration: a review of available vision-specific psychometric tools.

Purpose: Age-related macular degeneration (AMD) has a considerable impact on older adults' independence and autonomy. Recently, patient reported outcomes (PROs) such as QoL have been met with increasing interest by the scientific community, healthcare payers and planners. Against this background, the multitude of psychometric tools used to measure QoL in AMD was reviewed.

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.

Confocal blue reflectance imaging in type 2 idiopathic macular telangiectasia.

Purpose: To report the characteristics of confocal blue reflectance imaging in type 2 idiopathic macular telangiectasia (type 2 IMT).

Methods: In a prospective observational cross-sectional study, both eyes of 33 patients with type 2 IMT were examined by means of fundus biomicroscopy, fundus photography, fluorescein angiography, and optical coherence tomography (OCT). Confocal blue reflectance (CBR) imaging was performed using a confocal scanning laser ophthalmoscope (HRA2; Heidelberg Engineering, Heidelberg, Germany).