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The use of OCT to monitor intravitreal treatment varies in clinical practice and is not always mandatory. The ALBATROS data collection aimed to clarify the impact of routinely implemented OCT on clinical outcomes and its impact on vision-related quality of life (VRQoL). Methods: An observational cohort study included patients with retinal diseases starting an intravitreal anti-vascular endothelial growth factor treatment in Germany. Treatment followed clinical practice except mandatory OCT examination during the 12-month observation period. VRQoL was assessed by NEI VFQ-25 and compared with respect to OCT examinations and number of intravitreal injections in the different diseases (nAMD, DME, BRVO, CRVO). Results: 1478 patients (74.5 ± 10.9 years, 54.9% female) were included in the analysis. Patients had neovascular AMD (65.2%), DME (18.4%), BRVO (9.5%), or CRVO (6.9%). 8.8 ± 2.6 OCT examinations and 6.1 ± 3.2 intravitreal injections were performed within 12 months. VRQoL differed between indications at baseline, with substantially lower values for neovascular AMD and CRVO. After twelve months, an increase in visual acuity and visual functional scale was observed for nAMD, DME, and BRVO, while in DME only, there was an association between number of OCT examinations and VRQoL. Conclusion: Intravitreal treatment was able to maintain VRQoL over twelve months in a real-world setting. Regular OCT examinations were associated with higher gain in VRQoL in DME patients after 12 months. [ABSTRACT FROM AUTHOR]

Purpose: To determine prevalence of paraproteinemic keratopathy (PPK) among patients with monoclonal gammopathy (MG). To evaluate interrelation between corneal and hematological parameters in patients with PPK. Methods: Fifty-one patients with monoclonal gammopathy of undetermined significance (n = 19), smoldering multiple myeloma (n = 5) or multiple myeloma (n = 27) were prospectively included in this study. Best-corrected visual acuity, slit-lamp biomicroscopy, Scheimpflug tomography, in-vivo confocal laser scanning microscopy, optical coherence tomography and complete hematological workup were assessed. Results: We identified n = 19 patients with bilateral corneal opacities compatible with PPK. PPK was newly diagnosed in 13 (29%) of 45 patients with a primary hematological diagnosis and in n = 6 patients without previous hematological diagnosis. The most common form was a discreet stromal flake-like PPK (n = 14 of 19). The median level of M-protein (p = 0.59), IgA (p = 0.53), IgG (p = 0.79) and IgM (p = 0.59) did not differ significantly between the patients with and without PPK. The median level of the FLC κ in serum of patients with kappa-restricted plasma cell dyscrasia was 209 mg/l in patients with PPK compared to 38.1 mg/l in patients without PPK (p = 0.18). Median level of FLC lambda in serum of patients with lambda-restricted plasma cell dyscrasia was lower in patients with PPK compared to patients without PPK (p = 0.02). Conclusion: The PPK was mostly discreet, but its prevalence (29%) was higher than expected. Median level of the monoclonal paraprotein was not significantly higher in patients with PPK compared to patients without PPK. Our results suggest a lack of correlation between morphology and severity of the ocular findings and severity of the monoclonal gammopathy. Trial registration: German Clinical Trial Register: DRKS00023893. [ABSTRACT FROM AUTHOR]

Purpose: Retinal alterations in inherited metabolic diseases associated with neurodegeneration are poorly studied. The objective was to study retinal thickness, specifically the components of the ganglion cell complex (GCC)—nerve fiber layer (NFL), ganglion cell layer (GCL), and inner plexiform layer (IPL)—using spectral-domain optical coherence tomography (SD-OCT) in two different diseases with potential dopaminergic depletion, phenylketonuria (PKU) and Gaucher disease type 3 (GD3). Methods: Retinal layers in 19 patients with PKU, 15 patients with GD3, and 93 healthy individuals were measured using peripapillary ring scan and macular SD-OCT. Linear mixed models were computed including an adjustment for age, sex, and spherical equivalent. We calculated Spearman's rank correlations between retinal layer measurements and clinical and/or laboratory parameters. Results: Thinning of total retinal thickness was found in the macular inner ring (p = 0.002), and outer ring (p = 0.012), sparing the fovea (p = 0.12) in PKU, while in GD3, all subfields were thinned (fovea p < 0.001, inner ring p = 0.047, outer ring 0.07). In both conditions, thinning was most evident in the NFL, GCL, and IPL, while OPL (outer plexiform layer) was thickened. Peripapillary retinal nerve fiber layer measurements remained normal. GCL and IPL in PKU correlated with tyrosine serum concentration. Conclusion: Thinning of the NFL, GCL, and IPL, with thickened OPL, are both found in PKU and in GD3. Low dopamine concentrations in the retina might promote these effects. However, these data do not give evidence that retinal measurements can be used as a biomarker for disease severity in patients with GD3. [ABSTRACT FROM AUTHOR]

Background: Spectral domain optical coherence tomography (SD-OCT) is a widely applied non-invasive technique for evaluating optic nerve head parameters. The aim of this study was to evaluate the impact of biometric parameters such as the spherical equivalent (SE) and the anterior corneal curvature (ACC) on the peripapillary retinal nerve fiber layer (pRNFL), Bruch's membrane opening (BMO), and the minimum rim width (MRW) measurements performed by spectral domain optical coherence tomography (SD-OCT) in glaucomatous and healthy eyes.Methods: In this cross-sectional, case-control prospective pilot study, the glaucoma group consisted of 50 patients with previously diagnosed and treated glaucoma and one healthy group of 50 subjects. Two consecutive examinations of pRNFL, BMO, and MRW with SD-OCT for every patient were performed without ACC and objective refraction (imaging 1) and with them (imaging 2).Results: The interclass correlation coefficient (ICC) reflected high agreement between imaging 1 and imaging 2 in both groups. The ICC in the glaucoma and healthy groups for pRNFL (0.99 vs. 0.98), BMO (0.95 vs. 0.97), and MRW (1.0 vs. 1.0) was comparable.Conclusions: Our preliminary data from a small number of eyes showed that the measurements of pRNFL, MRW, and BMO reflected high agreement between both imaging techniques with ACC and objective refraction and without these parameters in subjects with a refractive error up to ± 6.0 diopters. Further studies with participants with higher refractive error are necessary to evaluate the impact of biometric parameters such as SE and ACC on measurements with SD-OCT. [ABSTRACT FROM AUTHOR]

Introduction: Niemann-Pick type C (NPC) is a lysosomal storage disease that is progressive and life-limiting, with an estimated incidence of 1:120,000 live births. In addition to systemic manifestation with (hepato-)splenomegaly, there are a number of neurological manifestations (ataxia, dysarthria, dementia, cataplexy, epileptic seizures, and psychiatric disorders). Characteristic is vertical supranuclear gaze palsy, which is often overlooked. Early diagnosis and start of therapy improve quality of life. This study aimed to characterize oculomotor dysfunction of NPC patients, and to provide ophthalmologic data including retinal imaging. Methods: Eighteen patients with biochemically or genetically diagnosed NPC completed oculomotor and ophthalmologic examination. Ten of them performed saccadometry by infrared based video-oculography. Saccadic parameters were compared to 100 healthy controls, and were correlated with clinical variables. Another subgroup of eight patients received optical coherence tomography (OCT) of the optic disc and the macula, of which the segmented layers were analysed using a crude linear mixed model, and one adjusted for age, sex, and spherical equivalent. Results: Saccadometry revealed slowed peak velocity compared to controls most evident vertically. Peak velocity correlated negatively with SARA-Score, but correlation with clinical assessment of saccades was not significant. Clinical features in the assessment of vertical saccades were intensive blinking and head movements to initiate gaze changes, and lateral trajectory of the eyes. Macular OCT revealed significant total retinal thinning in the fovea, specifically of the outer nuclear layer and outer retinal layer. Para- and perifoveal retinal thicknesses, as well as peripapillary retinal nerve fibre layer were normal. Conclusions: Foveal thinning was revealed in NPC. It remains to be shown, whether OCT will prove to be useful to monitor progression. Saccadic impairment reflects CNS involvement and therefore is a parameter to demonstrate the progression of NPC, and potentially also the efficacy of new therapies. Saccadometry, in contrast to clinical investigation, allows the precise evaluation of saccades. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the distribution of peripapillary retinal nerve fiber layer thickness as measured by optical coherence tomography and its associations with ocular parameters in a population-based setting.Methods: In the population-based Gutenberg Health Study, 1974 subjects from the Mainz-Bingen region were scheduled for ophthalmologic examinations, including visual acuity, refraction, tonometry, biometry, and measurements of peripapillary retinal nerve fiber layer thickness (pRNFL) by optical coherence tomography (SD-OCT, Spectralis®, Heidelberg Engineering, Germany). The associated factors in the global and sectoral pRNFL data were analyzed using multivariable linear mixed models related to age, sex, and ocular parameters.Results: Advanced age and longer axial length were associated with a thinner pRNFL, whereas pRNFL thickness was independent of sex, intraocular pressure (IOP), and central corneal thickness (CCT). Spherical equivalent was positively associated with pRNFL thickness, except in the temporal pRNFL sector.Conclusions: In this large, population-based study, pRNFL thickness decreased with age. Longer eyes had thinner pRNFL thickness. Sex, IOP, and CCT showed no significant association with pRNFL thickness. These data may improve the interpretation of pRNFL data. [ABSTRACT FROM AUTHOR]

Purpose Optical coherence tomography ( OCT) of the anterior segment allows quantitative analysis of the geometry of the iris. We performed spectral domain OCT examinations in healthy emmetropic, hyperopic and myopic subjects to investigate iris curvature and its associations. Methods In a cross-sectional study, out of 4617 eyes (2309 subjects) those with refractive errors of <−4 or >+3 dioptres were identified by objective refraction. The iris was examined using the anterior segment mode of a spectral domain 3D OCT-2000 (Topcon Inc., Japan) in the temporal meridian, and OCT scans were investigated with respect to presence and amount of convex and concave iris configuration. Ninety-three eyes of 50 subjects served as emmetropic group (−0.5 ≤ x ≤+0.5 dioptres). Previous ocular surgery was exclusion criterion. Results Six hundred and sixty-eight eyes of 398 persons [292 male (76%); age range; 18-66 years] were included in the study. In the myopic group, 105 eyes had a concave iris configuration (26%), while in the hyperopic group, no eye had this configuration (0%) and in the emmetropic group five eyes (5%). Convex iris configuration was found in 96% of hyperopic, in 85% of the emmetropic and in 67% of the myopic eyes. There was an association between concave iris configuration and myopia, younger age and male gender, and with anterior chamber angle width. Conclusion Spectral domain OCT images can be used for analysis of the iris structure and geometry. Our results are limited to the properties of the study population having an age range from 18 to 66 years and consisting mainly of men. [ABSTRACT FROM AUTHOR]

Background: Phakic anterior chamber iris-fixated IOL (PIOL) are established for the correction of high myopia. These patients require attention to macular pathologies. This study was to evaluate the quality of imaging through the PIOLs.Methods: 54 eyes of 31 myopic and hyperopic patients with Verisyse, Verisyse toric, and Veriflex phakic iris-fixated IOL were included. Mean preoperative refraction was -11.0 D and +5.7 D, respectively; mean follow-up time was 7.4 years (5-11 years). A matched control group was formed without PIOL implantation. OCT scans were performed and evaluated qualitatively and quantitatively.Results: In 52 eyes of 31 patients (96%), a 3D-OCT image and fundus photo could be obtained in good quality. Foveal thickness was significantly thicker in the myopic patients, whereas all other sectors and total volume had no significant difference.Conclusions: It proved possible to obtain high-resolution scans and digital photos of the same quality as without additional phakic IOL. [ABSTRACT FROM AUTHOR]

PURPOSE: To examine choroidal thickness in nonarteritic anterior ischemic optic neuropathy (AION). DESIGN: Retrospective case control study. METHODS: In the eye clinic of the University Medical Center in Mannheim, Germany, we studied a group that consisted of patients with nonarteritic AION and a control group that consisted of individuals with normal fundus. Choroidal thickness was measured by the enhanced-depth imaging of spectral-domain optical coherence tomography. The main outcome measure was choroidal thickness. RESULTS: The study group consisted of 20 patients: 11 patients with acute nonarteritic AION and an unaffected contralateral eye and 9 patients with acute unilateral nonarteritic AION and previously nonarteritic AION in the contralateral eye. The control group consisted of 58 patients (58 eyes). In multivariate analysis, thinner subfoveal choroidal thickness was associated with the diagnosis of nonarteritic AION (P = 0.001; regression coefficient B, --55.1), after adjusting for age (P < 0.001) and refractive error (P = 0.20). Similarly, unaffected eyes contralateral to eyes with acute nonarteritic AION as compared to control eyes showed thinner subfoveal choroidal thickness (P = 0.037) after adjusting for age (P = 0.001) and refractive error (P = 0.06). In a reverse manner, nonarteritic AION was associated with thinner subfoveal choroidal thickness (P = 0.007) after adjusting for age, optic disc diameter, gender, and refractive error. CONCLUSIONS: Eyes affected by nonarteritic AION and unaffected contralateral eyes showed significantly thinner macular choroids than eyes of a control group after adjusting ocular and systemic parameters. A thin choroid may be added to the diagnostic features of nonarteritic AION. Future studies may examine the pathophysiologic meaning of the finding. [ABSTRACT FROM AUTHOR]