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The aim of the study is to evaluate the progression of visual field (VF) defects over 16 years of observation and to assess abnormalities in vessels and retinal nerve fibre layer (RNFL) thickness in patients with optic disc drusen (ODD). Both static automated perimetry (SAP) and semi-automated kinetic perimetry (SKP) were performed in 16 eyes of 8 patients (mean age 54 years) with ODD among 26 eyes of 13 patients examined 16 years before. The area of I2e, I4e, III4e, and V4e isopters was measured in deg2. The MD and PSD parameters were estimated using SAP. Optical coherence tomography angiography (OCT-A) was additionally performed in 16 ODD eyes and 16 eyes of 8 healthy subjects to estimate the RNFL thickness and vessel density of the optic nerve disc and the macula. The differences in all isopter areas of SKP and SAP parameters after 16 years were not significant. The analysis of OCT-A showed a significant reduction of the vessel density and RNFL of the peripapillary area in each segment in patients with ODD, compared with the control group. The highest reduction of RNFL was observed in the superior segment of the optic disc area (92.56μm vs 126.63μm) also the macular thickness was decreased in ODD patients, compared with the control group. In the macula, there was a significant vascular defect in the whole superficial layer and in the parafoveal deep layer. A strong significant correlation of the parafoveal deep plexus with MD and PSD parameters was detected. In conclusion, VF loss due to ODD after 16 years of the follow-up was not significant both in SKP and SAP. ODD caused a reduced vessel density and RNFL, as well as macular thickness in OCT-A. SAP parameters were influenced by parafoveal deep plexus., Competing Interests: The authors have declared that no competing interests exist.

Background: To assess the utility of enhanced depth imaging optical coherence tomography (EDI-OCT), compared with other conventional imaging modalities, for detecting and characterizing optic nerve head drusen (ONHD) in children., Methods: We report a retrospective cross-sectional case series of consecutive pediatric patients (age ≤16 years) with ONHD confirmed using B-scan ultrasonography. All eyes were evaluated using spectral-domain OCT of the optic nerve head in conventional (non-EDI) and EDI modes, fundus autofluorescence (FAF), and standard automated perimetry. Detection rates and the capacity to characterize ONHD were compared between EDI-OCT, non-EDI-OCT, and FAF., Results: Twenty-eight eyes of 15 patients (mean age 11 years; 60% female) were identified with definite ONHD that were confirmed by B-scan ultrasound. Among the technologies, EDI-OCT, non-EDI-OCT, FAF, and automated perimetry had findings consistent with ONHD in 24, 21, 18, and 4 eyes, respectively. EDI-OCT had a significantly better detection capability (86% of eyes) compared with FAF (P = 0.04) but not with non-EDI-OCT (P = 0.15). Similar to results previously reported in adult patients, EDI-OCT detected ONHD at different levels of depth; most were located anterior to the lamina cribrosa. ONHD detected by EDI-OCT appeared as hypo-reflective ovoid regions bordered by hyper-reflective material or as isolated hyper-reflective bands without a hypo-reflective core. The mean greatest diameter of ONHD seen on EDI-OCT was 449.7 (SD ±114.1) μm., Conclusions: EDI-OCT detects ONHD in most eyes identified as having drusen on B-scan ultrasonography. This technique has the potential to be an effective alternative first-line diagnostic and monitoring tool for ONHD, particularly for detecting buried drusen in children.

Fibulin-3 (also known as EGF-containing fibulin extracellular matrix protein 1 (EFEMP1)) is a secreted extracellular matrix glycoprotein, encoded by the EFEMP1 gene that belongs to the eight-membered fibulin protein family. It has emerged as a functionally unique member of this family, with a diverse array of pathophysiological associations predominantly centered on its role as a modulator of extracellular matrix (ECM) biology. Fibulin-3 is widely expressed in the human body, especially in elastic-fibre-rich tissues and ocular structures, and interacts with enzymatic ECM regulators, including tissue inhibitor of metalloproteinase-3 (TIMP-3). A point mutation in EFEMP1 causes an inherited early-onset form of macular degeneration called Malattia Leventinese/Doyne honeycomb retinal dystrophy (ML/DHRD). EFEMP1 genetic variants have also been associated in genome-wide association studies with numerous complex inherited phenotypes, both physiological (namely, developmental anthropometric traits) and pathological (many of which involve abnormalities of connective tissue function). Furthermore, EFEMP1 expression changes are implicated in the progression of numerous types of cancer, an area in which fibulin-3 has putative significance as a therapeutic target. Here we discuss the potential mechanistic roles of fibulin-3 in these pathologies and highlight how it may contribute to the development, structural integrity, and emergent functionality of the ECM and connective tissues across a range of anatomical locations. Its myriad of aetiological roles positions fibulin-3 as a molecule of interest across numerous research fields and may inform our future understanding and therapeutic approach to many human diseases in clinical settings.

Background: Optic disc drusen (ODD) are acellular deposits in the optic nerve head. ODD can be diagnosed using different imaging modalities, including enhanced depth imaging optical coherence tomography (EDI-OCT) and autofluorescence (AF). It is unknown which factors determine the sensitivity of AF. The aim of this study was to investigate the effect of volume and anatomic location of ODD on the sensitivity of AF., Methods: Cross-sectional study., Results: A total of 38 patients (75 eyes) with ODD were included. In 12 of 75 eyes (16%) and in 11 of 38 patients (29%), EDI-OCT detected ODD that were not detected by AF. In 24 distinctly solitary ODD, both increase in ODD volume (P = 0.0388) and a more superficial ODD location (P < 0.0001) increased the possibility of AF detection of ODD, when performing a multivariate analysis., Conclusions: EDI-OCT is superior to AF in the diagnosis of ODD. Volume and anatomic location of ODD have a significant impact on the sensitivity of AF.

Background: Making an accurate diagnosis of optic disc drusen (ODD) is important as part of the work-up for possible life-threatening optic disc edema. It also is important to follow the slowly progressive visual field defects many patients with ODD experience. The introduction of enhanced depth imaging optical coherence tomography (EDI-OCT) has improved the visualization of more deeply buried ODD. There is, however, no consensus regarding the diagnosis of ODD using OCT. The purpose of this study was to develop a consensus recommendation for diagnosing ODD using OCT., Methods: The members of the Optic Disc Drusen Studies (ODDS) Consortium are either fellowship trained neuro-ophthalmologists with an interest in ODD, or researchers with an interest in ODD. Four standardization steps were performed by the consortium members with a focus on both image acquisition and diagnosis of ODD., Results: Based on prior knowledge and experiences from the standardization steps, the ODDS Consortium reached a consensus regarding OCT acquisition and diagnosis of ODD. The recommendations from the ODDS Consortium include scanning protocol, data selection, data analysis, and nomenclature., Conclusions: The ODDS Consortium recommendations are important in the process of establishing a reliable and consistent diagnosis of ODD using OCT for both clinicians and researchers.

Objective: To determine whether at the time of diagnosis, the intraocular pressure (IOP) in patients with optic nerve head drusen (ONHD) correlates with the perimetric mean deviation (PMD) and the mean retinal nerve fiber layer (RNFL) thickness on optical coherence tomography (OCT)., Methods: This retrospective chart review included adults with ONHD from 2 academic medical centers. Inclusion criteria were age older than 18 years, definitive diagnosis of ONHD, measurement of IOP, and an automated visual field (VF) within 3 months of diagnosis. Exclusion criteria were unreliable VFs, use of IOP-lowering therapy, and visually significant ocular comorbidities. Data were collected from the initial visit. Age, IOP, method of diagnosis of ONHD, mean RNFL thickness, and PMD were recorded. Multiple and logistic regression models were used to control for potential confounders in statistical analyses., Results: Chart review identified 623 patients, of which 146 patients met inclusion criteria. Mean age was 44.2 years (range: 19-82 years). Average PMD of 236 eyes was -5.22 dB (range, -31.2 to +1.21 dB). Mean IOP was 15.7 mm Hg (range: 6-24 mm Hg). Forty eyes (16.9%) underwent RNFL measurement using OCT; mean RNFL thickness was 79.9 μm (range: 43-117 μm). There was no statistically significant association between IOP and PMD (P = 0.13) or RNFL thickness (P = 0.65). Eyes with ocular hypertension tended to have less depressed PMD than those without (P= 0.031). Stratified analyses of visible and buried subgroups yielded similar results., Conclusions: Lowering IOP in patients with ONHD has been proposed as a means to prevent progression of optic neuropathy. Our study demonstrated that among predominately normotensive eyes, higher IOP was not associated with greater VF loss or thinner RNFL at the time of presentation. This suggests that lowering IOP may not be beneficial in preventing visual loss in normotensive eyes with ONHD.

Background: Optic disc drusen (ODD) are seen in up to 2.4% of the general population, but the etiology and pathophysiology of the condition is still unknown. The purpose of this study was to determine the prevalence of ODD in a population-based child cohort and to determine if scleral canal diameter and fetal birth and pubertal parameters are associated with the presence of ODD., Methods: This observational, longitudinal population-based birth cohort study, with a nested case-control, included 1,406 children. Eye examinations were performed when the children were between 11 and 12 years of age. Assessment was performed of optical coherence tomography (OCT) scans from 1,304 children with gradable enhanced depth imaging scans of the optic disc., Results: ODD in one or both eyes were found in 13 (1.0%) of all children. All but one of the cases were found in children with scleral canal diameter in the lowest quartile (1,182-1,399 μm) in the nested case-control study. Children with ODD had a mean disc diameter of 1,339 μm (interquartile range, 30 μm), whereas it was 1,508 μm (interquartile range, 196 μm) in the 130 controls without ODD (P < 0.001). No differences in sex, birth weight, refractive error, and Tanner stages (of puberty) were found between children with and without ODD., Conclusions: The prevalence of ODD was 1% in a large child cohort examined by OCT. ODD was found only in eyes with a narrow scleral canal, which is consistent with the hypothesis that ODD might arise as a consequence of retinal nerve fiber congestion in the scleral canal.

Optic disc drusen may be a cause of visual field defects and visual loss. The mechanism by which this occurs is unclear. We report a patient who developed decreased vision in the right eye and was found to have a heavy burden of superficial optic disc drusen. Optical coherence tomography (OCT) confirmed focal retinal nerve fiber layer thinning that corresponded with the distribution of drusen. OCT angiography, with superficial laminar segmentation, showed focal capillary attenuation overlying the most prominent drusen. These findings demonstrate alterations in the superficial retinal capillary network associated with optic disc drusen.

Bilateral optic disc edema in a diabetic patient may be caused by diabetic papillopathy. We herein report on a patient with bilateral optic disc drusen simulating diabetic papillopathy. A 55-year-old patient with type 2 diabetes presented with decreased vision of 1-month. Diabetic papillopathy was initially considered as there was disc edema in both eyes with focal hemorrhages at the disc margin and mild visual loss. Ultrasound of the optic nerve head revealed optic disc drusen in both eyes and this was also confirmed by the control photograph. Optic nerve head drusen should be considered in the differential diagnosis of a diabetic patient presenting with disc edema.

Purpose: To explore the applicability of multifocal visual evoked potentials (mfVEPs) for research and clinical diagnosis in patients with optic disc drusen (ODD). This is the first assessment of mfVEP amplitude in patients with ODD., Methods: MfVEP amplitude and latency from 33 patients with ODD and 22 control subjects were examined. Mean amplitude, mean inner ring (IR) amplitude (0.87-5.67° of visual field) and mean outer ring amplitude (5.68-24° of visual field) were calculated using signal-to-noise ratio (SNR) and peak-to-peak analysis. Monocular latency was calculated using second peak analysis, while latency asymmetry was calculated using cross-correlation analysis., Results: Compared to normals, significantly decreased mean overall amplitude (p < 0.001), IR amplitude (p < 0.001) and outer ring amplitude (p < 0.001) were found in ODD patients when using SNR. An overall monocular latency delay of 7 ms was seen in ODD patients (p = 0.001). A significant correlation between amplitude and automated perimetric mean deviation as well as retinal nerve fibre layer thickness was found (respectively, p < 0.001 and p = 0.003). The overall highest correlation was found in this order: outer ring, full eye and IR. In the control group, SNR intersubject variability was 17.6% and second peak latency intersubject variability was 2.8%., Conclusion: Decreased mfVEP amplitude in patients with ODD suggests a direct mechanical compression of the optic nerve axons. Our results suggest that mfVEP amplitude is applicable for the assessment of optic nerve dysfunction in patients with ODD., (© 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: Optic disc drusen (ODD) is hyaline deposits in the optic nerve head seen in 1-2% of the population. Long-term evolution of ODD anatomy and visual field defects in ODD patients is a key factor for learning more about pathophysiology and prognosis of the condition. With a median follow-up period of 56 years, this is the first study that evaluates superficial optic disc anatomy and visual fields in patients with ODD over a life span., Methods: Observational case series investigating progression of superficial optic disc anatomy and visual fields in ODD patients. Eight patients with hereditary ODD were included., Results: Mean age at initial and follow-up examination was, respectively, 16.8 and 73.3 years. The median follow-up time was 56 years. A minimal or non-existing change in superficial ODD anatomy (size and number) was seen in 10 of 12 eyes. There was a tendency towards more anatomical change in subjects younger at first examination. A 27% decrease in Goldmann perimetric visual field area (cm 2 ) was found between initial and follow-up examinations., Conclusion: Minimal or no change in superficial optic disc anatomy and visual fields in ODD patients oldest at the initial examination suggests that anatomical progression of ODD in patients with hereditary ODD happens primarily before adulthood., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: Optic disc drusen (ODD) are found in up to 2.4% of the population and are known to cause visual field defects. The purpose of the current study was to investigate how quantitatively estimated volume and anatomic location of ODD influence optic nerve function., Methods: Anatomic location, volume of ODD, and peripapillary retinal nerve fiber layer and macular ganglion cell layer thickness were assessed in 37 ODD patients using enhanced depth imaging optical coherence tomography. Volume of ODD was calculated by manual segmentation of ODD in 97 B-scans per eye. Anatomic characteristics were compared with optic nerve function using automated perimetric mean deviation (MD) and multifocal visual evoked potentials., Results: Increased age (P = 0.015); larger ODD volume (P = 0.002); and more superficial anatomic ODD location (P = 0.007) were found in patients with ODD visible by ophthalmoscopy compared to patients with buried ODD. In a multivariate analysis, a worsening of MD was significantly associated with larger ODD volume (P < 0.0001). No association was found between MD and weighted anatomic location, age, and visibility by ophthalmoscopy. Decreased ganglion cell layer thickness was significantly associated with worse MD (P = 0.025) and had a higher effect on MD when compared to retinal nerve fiber layer thickness., Conclusions: Large ODD volume is associated with optic nerve dysfunction. The worse visual field defects associated with visible ODD should only be ascribed to larger ODD volume and not to a more superficial anatomic ODD location.

Purpose: To evaluate the dynamic remodeling of drusen in subjects with unilateral neovascular age-related macular degeneration (AMD) receiving a three-year course of oral docosahexaenoic acid (DHA) or placebo., Setting: Institutional setting., Methods: Three hundred subjects with age-related maculopathy and neovascular AMD in the fellow eye were randomly assigned to receive either 840 mg/day DHA or placebo for 3 years. Main outcome measures of this post-hoc sub-group analysis were progression of drusen number, total diameter, and total area on fundus photography, and their association with DHA supplementation, socio-demographic and genetic characteristics., Results: Drusen progression was analyzed in 167 subjects that did not develop CNV (87 that received DHA and 80 that received placebo). None of the drusen remodeling outcomes were significantly associated with DHA supplementation. Total drusen diameter reduction in the inner subfield was significantly associated with age (older patients: r = -0.17; p = 0.003). Women showed a tendency to decreased total drusen diameter in the inner subfield with CFH polymorphism (p = 0.03), where women with TT genotype tended to have a greater reduction in drusen diameter than other genotypes (CC and CT). Drusen area in the inner subfield was more reduced in older patients (r = -0.17) and in women (p = 0.01). Drusen number showed no significant trends., Conclusions: Dynamic drusen remodeling with net reduction in drusen load over three years was found in patients with exudative AMD in one eye and drusen in the other eye (study-eye). This reduction was correlated with increased age and female gender, and showed a tendency to be influenced by CFH genotype, but did not appear to be affected by DHA supplementation., Trial Registration: Controlled-Trials.com ISRCTN98246501.

Optic nerve head drusen (ONHD) are incidental ophthalmologic finding in the optic nerve. Patients with ONHD are often asymptomatic, but sometimes present with transient visual obscuration's (TVO), the reported incidence of which is 8.6%. Optic nerve head drusen are of two types: Superficial; visible and deep. The deep-buried drusen mimic papilledema. Because of the varied presentation deep-buried drusen pose a diagnostic challenge to the ophthalmologists. In young patients, they are mistaken for papilledema as it is clinically difficult to detect a buried drusen in the optic nerve head, but are seen on the surface with aging as the retinal nerve fiber layer thins out. They are observed as pale yellow lesions more often located towards the poles. Clinical examination aided with diagnostic tests like computed tomography (CT) orbits and ultrasound B scan can help establish the diagnosis. Herein, we report a rare case of optic nerve head drusen in a young lady, who presented with loss of vision and clinical evaluation and investigations suggested ONHD with anterior ischemic optic neuropathy.

Purpose: (1) To characterize the retrobulbar circulation in patients with optic disc drusen (ODD) using colour Doppler imaging; (2) to explore the differences in retrobulbar hemodynamics in patients with primary open-angle glaucoma (POAG)., Methods: Retrospective analysis using three diagnostic groups: patients with ODD, age-matched healthy controls and age- and visual defect-matched patients with POAG. Subjects with history of ocular trauma or ocular diseases including vascular-related diseases were excluded. Kruskal-Wallis and Spearman's correlation tests were performed to compare and explore the correlations between the hemodynamic parameters and visual field damage., Results: Sixty patients were included and equally divided between the three groups. Patients with ODD had lower systolic and diastolic flow velocities in the central retinal artery (CRA) when compared to healthy individuals, but no difference was found with the glaucoma group (p < 0.005 versus healthy; p range 0.30-0.37 versus POAG). The systolic shift in flow patterns correlated with the extent of visual field defect in ODD patients (p < 0.001, r = -0.78, confidence interval: -0.43 to -0.92), but not in POAG (p = 0.73). Short ciliary arteries from ODD patients had lower mean systolic flow velocities when compared to both healthy individuals and patients with POAG (p < 0.01 versus healthy; p ≤ 0.04 versus POAG). The ophthalmic artery in ODD group had significantly higher mean blood velocities than in the POAG (p = 0.04), while showing a significant overlap in Doppler waveform variables to the control group (p range 0.08-0.97)., Conclusions: Similar to patients with glaucoma, individuals with ODD have low blood flow velocities in the vessels around the optic nerve head. Unlike patients with glaucoma, however, their CRA's blood flow velocity patterns correlate with the extent of the visual field defects., (© 2014 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: The relationship between clinical severity of age-related macular degeneration (AMD) and macular function has not been well established. In this study, we investigated the correlation between clinical severity and functional deficits as detected by static and flicker perimetry., Methods: This cross-sectional study consisted of 279 AMD subjects and 24 control participants. AMD subjects were allocated into 1 of 10 AMD severity groups depending on the status of the designated study eye and the fellow eye, as assessed by color fundus photographs. Visual acuity, and static and flicker perimetry were tested on one eye during the same session. The geometric means, SDs, and percentage of abnormal eyes of static and flicker sensitivity of each AMD severity group were determined and compared., Results: The pattern of change in sensitivity and percentage of abnormal eyes for static perimetry across all AMD severity groups were similar to flicker perimetry. Eyes with drusen > 125 μm (P[static] = 0.018, P[flicker] = 0.024), drusenoid epithelial detachment (P[static and flicker] < 0.001) and noncentral geographic atrophy (GA; P[static and flicker] < 0.001) had significant reductions in static and flicker sensitivities compared to normal eyes. Static (β-coefficient -1.59, 95% confidence interval [CI] -4.78-1.60) and flicker (β-coefficient -1.29, 95% CI -4.66-2.08) sensitivities declined at a similar rate in eyes that showed clinical signs of progression., Conclusions: Static and flicker perimetry were affected similarly across the spectrum of AMD severity, and methods appeared to be valid techniques for assessing retinal sensitivity in AMD once drusen > 125 μm are present, but before the development of late AMD.

Purpose: The aim of this study was to investigate the relationship between clinical macular changes and retinal function in age-related macular degeneration (AMD)., Methods: We recruited 357 participants with visual acuity of better than 20/60 in the study eye, including 64 individuals with normal fundi and 293 AMD participants classified into 12 subgroups based upon the International Classification and Grading System. Visual function in the study eye was assessed using two steady-state tests (achromatic 14 Hz flicker [F14Hz] and isoluminant blue color [BCT]) and two adaptation measurements (cone photo-stress recovery rate [CRR] and rod dark adaptation recovery rate [RRR]). The groups were compared on their average psychophysical measurements and ranked according to functional deficiency., Results: Both adaptation parameters were significantly abnormal when only hard and/or intermediate drusen were evident (compared to controls, P < 0.023) and yielded considerably worse outcomes in cases with more advanced fundus changes (P < 0.001), but provided limited ability to discriminate between these cases (linear trend, CRR t = 0.68, P = 0.50 and RRR t = 1.76, P = 0.08). Steady-state measurements, however, declined gradually along the entire hierarchy of fundus changes (linear trend, F14Hz t = 10.16, P < 0.001 and BCT t = 11.19, P < 0.001) with F14Hz being able to detect significant functional change as early as in the intermediate drusen group, when compared to controls (P = 0.003)., Conclusions: Steady state thresholds (F14Hz and BCT) and clinical signs showed significant concordance across the spectrum of early AMD fundus changes. This suggests that these tests may be an effective tool for monitoring progression of AMD to supplement clinical grading.

Background: The fluorescein angiographic criteria for differentiating optic disc drusen (ODD) from optic disc edema have been unclear. We designed a study to identify distinguishing angiographic features of each and to apply them to cases where both drusen and edema were present., Methods: A computer search was performed for cases evaluated in a university academic neuro-ophthalmology consultative practice and coded as ODD; all cases were reviewed, and those with fluorescein angiography were selected for further study. Cases were classified as either buried or surface ODD. Ten cases with papilledema were selected for comparison. Eight cases of coexistent drusen and edema were identified. Autofluorescence, early leakage, early blockage, early and late nodular staining, late peripapillary staining, and late leakage were tabulated., Results: Two hundred sixteen cases of ODD were identified; 62 (116 eyes) had adequate fluorescein angiography for study. Twenty-three eyes were classified as surface ODD; 90% demonstrated early nodular staining of the disc, with late nodular staining in 90% and late circumferential peripapillary staining in 22%; autofluorescence was visible in 93% with preinjection photography. Eighty-three eyes were classified as buried ODD; 25% demonstrated early nodular staining, with late nodular staining in 29% and late circumferential peripapillary staining in 80%; autofluorescence was visible in 12% of those with preinjection photography. In 9 eyes, buried ODD were present with superimposed true edema. In these eyes, early dye leakage, late nodular hyperfluorescence, and late leakage were present., Conclusion: Early and late fluorescein angiographic features reliably distinguish ODD from edema and may be particularly useful when the conditions coexist.

Background: Several studies have suggested racial differences in the prevalence of optic nerve head drusen (ONHD). We aimed to determine the percentage of patients with ONHD who are black and to describe the clinical, ophthalmoscopic, and perimetric findings in these patients., Methods: We conducted a retrospective chart review of all patients with ONHD seen at our institution between 1989 and 2010. Only black patients with ONHD confirmed on either funduscopy or B-scan ultrasonography were included. Demographic and clinical findings in these patients were recorded and analyzed., Results: Of the 196 patients with confirmed ONHD, 10 (5.1%) were black. This included 7 females and 3 males with ages ranging from 8 to 61 years. Six of the 10 patients had bilateral ONHD. The ONHD were buried in 11 of 16 eyes and exposed in 5 of 16 eyes. Fifteen of 16 eyes with ONHD had small cupless optic nerve heads. Visual fields were normal in 4 of 16 eyes with ONHD. In the remainder, visual field defects included an enlarged blind spot (5 eyes), constricted field (5 eyes), nasal defect (2 eyes), central defect (1 eye), and generalized depression (1 eye). Visual field defects were present in 4 of 5 eyes (80%) with exposed ONHD and 8 of 11 eyes (72.7%) with buried ONHD. None of the patients were related, and none of their examined family members had exposed ONHD on funduscopic examination., Conclusions: ONHD are rare in blacks, possibly due to the presence of a larger cup-to-disc ratio or a lack of predisposing genetic factors. Visual field defects are common in black patients with both exposed and buried ONHD.

Unlabelled: The aim of this study was to determine the association between color perception determined by maximum color contrast sensitivity and Farnsworth-Munsell 100-hue tests and visual acuity in the presence of optic nerve disk drusen., Material and Methods: A case-control study, including 137 persons, was performed. Thirty-seven patients (67 eyes) with optic nerve disk drusen and 100 healthy controls (200 eyes) were examined. For visual acuity testing, a typical Snellen chart (the direction of the gap in Landolt C) was used. A computerized Farnsworth-Munsell 100-hue test and maximum color contrast sensitivity programs were used for color discrimination., Results: The results of maximum color contrast sensitivity test, and the Farnsworth-Munsell 100-hue test were better in healthy controls than patients (1.94 ± 0.66 vs. 2.2 ± 0.85, P=0.02; 94.1 ± 53.9 vs. 120.6 ± 61, P=0.003, respectively)., Conclusion: The study revealed that the presence of optic nerve disk drusen was associated with decreased perception of colors.

Purpose: Until now there has been no standardized, systemic approach to diagnostics in patients with optic nerve head drusen (ONHD). This study compares visual field (VF) results obtained with static automated perimetry (SAP) and semi-automated kinetic perimetry (SKP) in patients with bilateral visible ONHD., Methods: Visual fields in 26 eyes (13 patients) with ONHD were obtained by Humphrey Field Analyser II (SAP) and Octopus 101 (SKP) equipment and classified by three masked observers. Pairs of VFs were considered equivalent if the descriptions given by both methods matched. Individual reaction time (RT) was assessed during SKP. Fifteen healthy volunteers were examined as control material., Results: Visual field pairs matched in 19 eyes (three normal VFs, 16 arcuate defects). In the remaining eight eyes SKP provided more information in concentric constriction of the VF (two eyes) and SAP provided more information on paracentral scotomas (five eyes). Sensitivity was 69% for SKP, 80% for SAP and 88% for both methods together. Mean RT assessed using SKP amounted to 909 ms in eyes with ONHD and 568 ms in normal subjects (p < 0.0003). The median examination duration was 13 mins with SKP and 11 mins with SAP (p = 0.05) in eyes with ONHD, and 8 mins in control eyes (p < 0.0001)., Conclusions: In clinical practice it is necessary to perform both SAP and SKP in patients with ONHD because the VF defects are diverse. In SKP, RT is prolonged in eyes with ONHD compared with normal eyes and SKP takes longer than SAP in ONHD eyes.

Purpose: To determine the effect of optic disc drusen on the latency of the pattern-reversal checkerboard visual evoked potentials (VEPs) and multifocal (mf)VEPs and to better understand the pathophysiology of the condition., Methods: Eighteen eyes with optic disc drusen (10 patients) and 38 control eyes (19 subjects) underwent VEP, mfVEP, and visual field testing. Only one eye of each individual, the one with the more affected visual field, was used in the analyses. The VEPs were recorded with a 15' and 60' reversing checkerboard pattern, and the mfVEPs were elicited by a 60-sector dartboard display., Results: Unlike the VEP results, the mfVEP revealed a significant increase in the average monocular latency of the optic disc drusen group compared with that of the control group. The average mfVEP relative latency for the optic disc drusen group (4.1 ms) was greater than that (0.8 ms) in the control group. For monocular and interocular analyses, the average percentage of points delayed in the drusen group was significantly greater than that in the control group., Conclusions: Optic disc drusen produced significant latency delays on the mfVEP test but not on the VEP test, presumably due to the mfVEP's ability to detect the effects of local changes. The results are consistent with the hypothesis that local mechanical compression by optic disc drusen leads to abnormal retinal ganglion cell activity.

Background: New technology allows more precise definition of structural alterations of all retinal layers although it has not been used previously in cases of optic disc drusen., Methods: Using Stratus and Fourier domain (FD) optical coherence tomography (OCT) and adaptive optics (AO) through a flood-illuminated fundus camera, we studied the retinas of a patient with long-standing optic disc drusen and acute visual loss at high altitude attributed to ischemic optic neuropathy., Results: Stratus OCT and FD-OCT confirmed severe thinning of the retinal nerve fiber layer (RNFL). FD-OCT revealed disturbances in the photoreceptor layer heretofore not described in optic disc drusen patients. AO confirmed the FD-OCT findings in the photoreceptor layer and also showed reduced cone density at retinal locations associated with reduced visual sensitivity., Conclusions: Based on this study, changes occur not only in the RNFL but also in the photoreceptor layer in optic nerve drusen complicated by ischemic optic neuropathy. This is the first reported application of FD-OCT and the AO to this condition. Such new imaging technology may in the future allow monitoring of disease progression more precisely and accurately.

Background: Peripapillary subretinal hemorrhage (PSH) is often found together with optic disc drusen, optic disc edema, peripapillary subretinal neovascular membranes, vitreous traction, and bleeding diatheses. Previous reports have stated that such optic disc hemorrhages are associated with intrapapillary bleeding largely in patients of Asian origin who are visually symptomatic from this process. We have encountered patients with PSH who have clinical features that differ from those described in these reports., Methods: This is a retrospective observational case series. Medical records of 10 patients with isolated peripapillary subretinal hemorrhages were reviewed for clinical characteristics and ancillary testing, including demographics, history, complete eye examination, visual fields, fundus photos, ultrasound, and fluorescein angiography (four patients) at presentation and follow-up. We excluded patients with drusen, neovascular membranes, disc edema, and intrapapillary hemorrhages., Results: There were 10 patients, all white women without visual symptoms, who had isolated, monocular, nasal, or superonasal peripapillary subretinal hemorrhage, a dysplastic crowded-tilted optic disc, myopia, and normal visual function. The hemorrhages resolved without sequelae over 3-6 months. The findings were frequently discovered on routine examination and suspected of representing papilledema., Conclusions: We have described a benign syndrome of isolated PSH in crowded and tilted optic discs in myopic eyes of white women. The PSHs do not cause visual symptoms and resolve spontaneously. We propose that an interplay of ocular motor forces, scleral thinning, and vitreopapillary traction acting on a morphologically vulnerable optic disc explains these hemorrhages.

Purpose: To describe a case where detection of blood flow in the central retinal artery and the central retinal vein was limited by an artifact evoked by the optic disc drusen. METHODS Case report., Results: During color Doppler sonography, examination of optic disc drusen may generate an artifact--the so-called twinkling artifact--which can simulate blood flow and make evaluation of the central retinal vessels impossible., Conclusions: Twinkling artifact does not allow evaluation of color Doppler sonography in the imaging of ocular vessels.

Background: It has been reported that visible optic disc drusen are associated with decreased visual acuity and loss of peripheral visual field. Patients with buried optic disc drusen have not been as well characterized., Methods: An observational, retrospective review was made of 92 eyes with funduscopic or ultrasonographic evidence of optic disc drusen. Demographics, presenting symptoms, visual acuity, refractive error, intraocular pressure, presence of an afferent pupillary defect, cup-to-disc ratio, appearance of the optic nerve, and visual field were recorded., Results: Fifty-one (55%) of the eyes were symptomatic; among them, 63% had symptoms of visual acuity loss, and 49% had symptoms of visual field loss. Seventy-nine (86%) of the optic discs appeared abnormal on ophthalmoscopy, but only 42% of these had visible drusen. Forty-five (49%) of the eyes had a visual field defect, and 73% of these were nerve fiber bundle in type. Overall, 73% of optic discs with visible drusen had abnormal visual fields compared with only 36% of optic discs with buried drusen (P < 0.05)., Conclusions: Among 92 eyes with optic disc drusen, only slightly more than 50% produced visual symptoms. Fewer than 50% of drusen were visible ophthalmoscopically. Buried optic disc drusen identified by ultrasound had a significantly lower frequency of associated visual field defects than did visible optic disc drusen.

A 44-year-old woman with progressive monocular visual loss was found to have ipsilateral optic disc drusen and an ipsilateral orbital apex mass compressing the optic nerve. The mass, not the drusen, was considered responsible for the worsening vision. Visual loss should not be glibly attributed to drusen, particularly if the visual loss is rapidly progressive. Retrobulbar imaging should be considered in such cases.