Validation of retinal oximetry vessel selection using fluorescein angiography in patients with optic disc drusen.

Retinal oximetry could provide insights into the pathophysiology of optic nerve disease, including optic disc drusen (ODD). Vessel selection for oximetry analysis is based on morphological characteristics of arterioles and venules and supported by an overlay of estimated blood oxygen saturations. The purpose of this cross-sectional study was to determine the validity of this vessel selection procedure by comparing it with vessel selection supported by video fluorescein angiography (FA). The study included 36 eyes of 36 patients with ODD who underwent retinal oximetry (Oxymap retinal oximeter T1) followed by FA (Heidelberg Spectralis). Two trained graders selected vessel segments in a pre-defined measurement area around the optic disc. One of these graders additionally performed the vessel segment selection with the support of FA images. When performed by the same grader, FA-supported and non-FA-supported vessel selection did not lead to significant differences in total vessel segment length, estimated oxygen saturations or vessel diameters (all p > 0.05). Inter-grader differences were found for arterial and venous segment lengths and arterial saturation (p < 0.05). A similar tendency was found for the arteriovenous saturation difference (p = 0.10). In conclusion, identifying vessel segments for retinal oximetry analysis based on vessel morphology and supported by a color-coded saturation overlay appears to be a valid method without the need for invasive angiography. A numerically small inter-grader variation may influence oximetry results. Further studies of retinal oximetry in ODD are warranted.

Diagnostic dilemma of papilledema and pseudopapilledema.

BACKGROUND: Papilledema is the optic disc swelling caused by increased intracranial pressure (ICP) that can damage the optic nerve and cause subsequent vision loss. Pseudopapilledema refers to optic disc elevation without peripapillary fluid that can arise from several optic disc disorders, with optic disc drusen (ODD) being the most frequent cause. Occasionally, pseudopapilledema patients are mistakenly diagnosed as papilledema, leading to the possibility of unneeded procedures. We aim to thoroughly examine the most current evidence on papilledema and pseudopapilledema causes and several methods for distinguishing between both conditions. METHODS: An extensive literature search was conducted on electronic databases including PubMed and google scholar using keywords that were relevant to the assessed pathologies. Data were collected and then summarized in comprehensive form. RESULTS: Various techniques are employed to distinguish between papilledema and pseudopapilledema. These techniques include Fundus fluorescein angiography, optical coherence tomography, ultrasonography, and magnetic resonance imaging. Lumbar puncture and other invasive procedures may be needed if results are suspicious. CONCLUSION: Papilledema is a sight-threatening condition that may lead to visual affection. Many disc conditions may mimic papilledema. Accordingly, differentiation between papilledema and pseudopailledema is crucial and can be conducted through many modalities.

Peripapillary Vessel Density in Relation to Optic Disc Drusen: A Multimodal Optical Coherence Tomography Study.

BACKGROUND: Optic disc drusen (ODD) are acellular calcified deposits within the optic nerve head known to cause visual field defects. An emerging gold standard for the diagnosis of ODD is enhanced depth imaging optical coherence tomography (EDI-OCT). The presence of ODD affects the adjacent peripapillary vasculature, which can be visualized using OCT angiography (OCTA). This study investigates the association between peripapillary vessel density and anatomical ODD location and volume using a newly developed method of multimodal OCT. METHODS: A case-control study with 16 patients diagnosed with ODD in the period 2008-2017 and 24 healthy controls. All patients and controls had EDI-OCT, OCTA, and demographic data collected. Using EDI-OCT and the medical imaging segmentation tool ITK-SNAP, 3-dimensional (3D) visualization of ODD in patients were created. ODD 3D visualization and corresponding OCTA scans were superimposed, making it possible to correlate ODD volume to the peripapillary vessel density in the corresponding modified Garway-Heath segments of the optic disc. RESULTS: We found that mean peripapillary vessel density across all modified Garway-Heath segments were lower in ODD patients compared with controls with significant reduction of peripapillary vessel density in the superior segment ( P = 0.03) and globally ( P = 0.05). A significant inverse proportionality between ODD volume and peripapillary vessel density in the corresponding segment was seen ( P = 0.002). CONCLUSIONS: We found a reduced peripapillary vessel density in regions with close anatomical proximity to ODD and inverse proportionality between ODD volume and peripapillary vessel density.

Influence of Patient Age and Presence of Optic Disc Drusen on Fluctuations in Retinal Nerve Fiber Layer Thickness.

BACKGROUND: It is generally believed that optic disc drusen (ODD) change only over long periods of time. Because, in our experience, this does not apply to younger patients, we investigated the natural course of changes of the peripapillary retinal nerve fiber layer (RNFL) in patients with ODD. METHODS: In this retrospective study, 40 eyes with and 40 eyes without ODD were examined, both cohorts were equally subdivided into younger subjects (20 years or younger) and older subjects (21 years or older). Three optical coherence tomography (OCT) scans of the peripapillary RNFL that had an interval of at least 1 month were required for each patient to be included in this study. The largest difference in total RNFL thickness (delta RNFL-t) and in RNFL thickness of the most differing sector (delta RNFL max) measured by OCT was compared. RESULTS: The differences in total RNFL thickness and in the most differing RNFL sector in the group of patients with ODD younger than 21 years were significantly larger than in each of the other 3 groups ( P = 0.0001). The other 3 groups did not differ significantly. CONCLUSIONS: Patients with ODD younger than 21 years have distinct variations in peripapillary RNFL thickness without evidence of increased intracranial pressure. In the absence of further pathological findings or neurological symptoms, an observational approach seems adequate in these patients.

Rates of Visual Field Change in Eyes With Optic Disc Drusen.

BACKGROUND: Optic disc drusen (ODD) are calcified deposits in the prelaminar portion of the optic nerve head. Although often asymptomatic, these deposits can cause progressive visual field defects and vision loss. The purpose of this study was to evaluate rates of functional loss in eyes with ODD and to investigate risk factors associated with rates of visual field progression. METHODS: This was a retrospective cohort study including 65 eyes of 43 patients with ODD from the Duke Ophthalmic Registry. All eyes had at least 12 months of follow-up and at least 3 reliable standard automated perimetry (SAP) tests. Linear mixed models were used to estimate rates of SAP mean deviation (MD) loss over time. Univariable and multivariable models were used to assess the effect of clinical variables and intraocular pressure (IOP) on rates of change. RESULTS: Subjects were followed for an average of 7.6 ± 5.3 years. The mean rate of SAP MD change was -0.23 ± 0.26 dB/year, ranging from -1.19 to 0.13 dB/year. Fifty-seven eyes (87.7%) had slow progression (slower than -0.5 dB/year), 6 eyes (9.2%) had moderate progression (between -0.5 dB/year and -1 dB/year), and 2 eyes (3.1%) had fast progression (faster than -1 dB/year). In multivariable models, older age and worse SAP MD at baseline were significantly associated with faster rates of change. Mean IOP was not associated with faster rates of MD change in both univariable and multivariable analyses. CONCLUSIONS: Most eyes with ODD had slow rates of visual field loss over time. Age and baseline severity were significantly associated with faster rates of visual field loss.

Optic nerve sheath diameter and axial length in patients with optic disc drusen: a cross-sectional study.

AIM: Measuring the optic nerve sheath diameter (ONSD) and the anteroposterior axial length of the eye in patients with optic disc drusen (ODD). METHODS: A total of 43 healthy volunteers and 41 patients with ODD were included in the study. The ONSD and axial length were measured in the posterior position using an ultrasound device (E-Z Scan AB5500 +) probe with a 10 MHz frequency. The ONSD was measured 3 mm behind the globe wall. Receiver operating characteristic (ROC) curve analysis was performed to determine patients with ODD using ONSD. Any p-value of < 0.05 was accepted to demonstrate significance. RESULTS: The ONSD was significantly higher (5.2 mm and 4.8 mm, p = 0.006, respectively), and the axial length was shorter (21.82 ± 2.15 mm and 23.27 ± 1.96 mm, p = 0.002, respectively) in the ODD group. The spherical equivalent was more commonly seen as hypermetropic in the ODD group (1.00 [- 0.85 to 1.75]). In the ROC analysis to determine the ONSD value in ODD diagnosis, the area under the curve was 0.6754 (95% confidence interval 0.559-0.788, p = 0.006). ONSD cutoff of 5.70 mm had a sensitivity of 0.366 and a specificity of 0.907 to diagnose ODD. CONCLUSION: In this study, the ONSD was significantly higher in the ODD group. The axial length was shorter in the ODD group. This study is the first in the literature to evaluate the ONSD in patients with optic disc drusen. Further studies are needed in this regard.

Juxtapapillary Choroidal Neovascular Membrane as a Complication of Optic Disc Drusen: Multimodal Imaging With Swept Source-Optical Coherence Tomography and Optical Coherence Tomography Angiography.

ABSTRACT: A 55-year-old Caucasian man presented to the neuro-ophthalmology department for follow-up evaluation due to long-standing bilateral optic nerve head drusen (ONHD). On examination, the BCVA was 20/20-2 in both eyes. Dilated fundus examination revealed extensive ONHD in both eyes, retinal hemorrhages, exudates inferonasal to the macula, and macular edema inferotemporal to the disc margin. Automated visual field testing revealed generalized depression in both eyes. Late phase leakage was observed on fluorescein angiography (FA). Optical coherence tomography angiography identified a small juxtapapillary choroidal neovascular membrane inferonasal to the macula in the right eye correlating with the area of retinal hemorrhage and exudates.

Optic Disc Drusen in Patients With Ocular Hypertension: A Case Series and Review of the Literature.

BACKGROUND: The identification of glaucomatous optic neuropathy in the setting of optic disc drusen (ODD) is a challenge, and the decision of whether to offer treatment in the form of intraocular pressure (IOP) reduction is controversial. Here, we present a series of patients with coexisting ocular hypertension and ODD to evaluate clinical features, treatment options, and progression of optic neuropathy. In addition, a review of the literature on ODD with elevated IOP is provided. METHODS: Six patients with ODD and a history of ocular hypertension are presented. Components of the examination and imaging modalities used to establish the diagnosis of ODD were recorded and a description of ocular hypertension history, glaucoma testing, and the potential treatment of IOP were also provided. RESULTS: In this series, 4 of 6 patients with concurrent ocular hypertension and ODD showed progression of optic neuropathy as assessed by visual field or retinal nerve fiber layer thickness. Of the 2 patients who did not show evidence of progression, 1 was treated with IOP-lowering medications and 1 was observed off treatment. Of the 4 patients who showed evidence of progression, all 4 were initially treated with IOP-lowering medications and 2 ultimately went on to have trabeculectomy surgery. In the patients with progressive optic neuropathy, lowering the IOP seemed to halt the progression suggesting there was a pressure-sensitive component. CONCLUSIONS: Distinguishing changes to the optic nerve, particularly the structural changes at the lamina cribrosa of true glaucomatous optic neuropathy in the setting of ODD, is a challenge. Careful consideration of risk factors including age, presenting features, progression indicators, and management goals is to be accounted for in the decision to offer treatment. We see the presence ODD in the patients with ocular hypertension as an additional risk for progressive changes to the nerve fiber layer and visual field that needs to be considered when determining whether to initiate therapy. Our data suggest that treatment of IOP in the patients with ocular hypertension with ODD and evidence of progression reduces the risk of further progression. Further work is needed to determine whether progression of optic neuropathy in the setting of coexisting ODD and ocular hypertension is related mechanistically to predominantly an ODD-type process, a glaucomatous process, or a combination thereof.

The status of the choroid in patients with optic disc drusen.

PURPOSE: To investigate the subfoveal and peripapillary choroidal thickness (CT) and the choroidal vascularity index (CVI) in patients with optic disc drusen (ODD). METHODS: This cross-sectional study examined the eyes of 17 patients with ODD and 18 healthy control subjects. The CT values were calculated manually from the images captured by enhanced depth imaging-optical coherence tomography (EDI-OCT). The CVI was defined as the proportion of the vascular area to the total choroidal area at the subfoveal and peripapillary areas after binarization of the EDI-OCT images. RESULTS: It was found that the mean subfoveal CVI value in the ODD group was significantly lower than that in the control group (p = 0.006). The mean peripapillary CVI values were significantly lower in all of the quadrants in the ODD group when compared with the control group (p = 0.008 for the temporal quadrant, p = 0.014 for the nasal quadrant, p = 0.024 for the superior quadrant, and p = 0.038 for the inferior quadrant). Regarding the CT, there were no significant differences in the subfoveal and peripapillary CT values between the ODD group and the control group (p >  0.05 for all values). CONCLUSION: The findings of this study indicate ODD to be associated with decreased subfoveal and peripapillary CVI, even though the subfoveal and peripapillary CT values were within the normal range. This result may prove important in relation to identifying a choroidal vascular network that appears to be morphologically normal but microstructurally impaired due to ODD. Further studies are required to verify the significance of CVI in the pathogenesis and complications of ODD.

[Observation of microstructure and vessel density changes in the superficial retinal layer in buried optic disc drusen patients].

Objective: To investigate the changes of the microstructure and vascular density in the superficial retinal layer of buried optic disc drusen (ODD) patients. Methods: Retrospective case-control study. A total of 36 ODD eyes (20 patients) and 26 normal control eyes were recruited in Beijing Union Medical College Hospital from January 2018 to July 2020. Measurement of best corrected visual acuity (BCVA), intraocular pressure, slit lamp, fundus examination and visual field examination were performed. The images and data of spectral domain-optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA) were analyzed and summarized. The differences of nasal retinal nerve fiber layer (RNFL), ganglion cell complex (GCC) thickness and macular superficial vascular density (VD) between ODD patients and normal controls were compared by independent sample t-test or Mann Whitney U test (the right eye was selected in bilateral ODD patients). Results: The 20 ODD patients and 26 normal controls were all female. There was no significant difference in age between the two groups (P>0.05). The BCVA and visual field examination was normal in all ODD patients. The SD-OCT examination showed an oval low signal shadow under the nasal outer nuclear layer of the optic disc, or local accumulation like a medium signal shadow with a clear boundary, and a high signal capsule in ODD patients. The RNFL in the upper nasal side of the ODD group was significantly different from the normal control group [(102.6±19.1) µm vs. (119.0±13.8) µm; t=-2.81; P<0.01]. Compared with normal control group [101.0 (100.0, 102.0) µm], the average GCC thickness in the ODD group [97.0 (89.3, 99.8) µm] was significantly different (U=48.50; P<0.01). The OCTA en-face scan showed that the vascular network in the macular area of the affected eyes was sparser than that of the control eyes. There was significant difference in superficial macular VD beteeen the ODD group (48.5%±2.8%) and the control group (51.0%±2.3%) (t=-2.63; P<0.05). Conclusions: There is thinning in the RNFL upper nasal side and GCC layer of the macular region in buried ODD patients, and the superficial VD of the macular region in buried ODD patients is lower than that in the normal controls.

Updates on ophthalmic imaging features of optic disc drusen, papilledema, and optic disc edema.

PURPOSE OF REVIEW: Optic nerve head elevation can be associated with vision loss. This review provides an update regarding key features of optic disc drusen (ODD) compared with papilledema from increased intracranial pressure and optic disc edema from other causes. RECENT FINDINGS: Clinical history and funduscopic examination are not sufficient to correctly diagnose different causes of optic nerve head elevation. Multimodal ophthalmic imaging is noninvasive and should be used as first-line diagnostic testing to distinguish optic disc edema or papilledema from pseudoedema. Advanced ophthalmic imaging, including enhanced depth imaging optical coherence tomography (EDI-OCT) and autofluorescence imaging, can visualize ODD at high resolution and determine whether there is optic disc edema. OCT angiography does not require contrast and can rapidly visualize papillary, peripapillary, and macular microvasculature and identify important vascular biomarker of ischemia and, potentially, visual prognosis. SUMMARY: Multimodal ophthalmic imaging can help in the diagnosis of ODD and optic disc edema and identify patients at high risk of vision loss and neurological issues in order to ensure appropriate diagnosis and treatment.

Enhanced Depth Imaging Optical Coherence Tomography Technology Reveals a Significant Association Between Optic Nerve Drusen Anterior Displacement and Retinal Nerve Fiber Layer Thinning Over Time.

BACKGROUND: Optic disc drusen (ODD) are a dynamic phenomenon, and their appearance, size, and relative location may change. The purpose of this study is to evaluate and quantify the longitudinal changes of buried ODD with enhanced depth imaging (EDI) optical coherence tomography (OCT) over time. METHODS: ODD were analyzed with Spectralis OCT (Heidelberg Engineering, Heidelberg, Germany) and EDI technology. The peripapillary retinal nerve fiber layer (RNFL) thickness was also measured. The size and depth of ODD were compared between the baseline and study visit (at least 2 years of follow-up), and the changes were correlated with mean RNFL thickness. The repeatability, coefficient of variation, and cutoff values for size and depth on EDI-OCT were calculated. RESULTS: Of 21 previously identified patients with ODD, only 12 patients (21 eyes) met the most recent diagnostic criteria according to the ODD Studies Consortium recommendations for using OCT-EDI technology. The 21 eyes were reanalyzed after a mean period of 44.7 ± 13.2 months (range: 24-71 months). Overall, 132 ODD were evaluated with a mean of 6.1 ± 2.5 ODD per eye and 1.9 ± 1.1 ODD per scan. Overall, we found a significant forward movement of the drusen between visits (P = 0.01). Most drusen (67.4%) moved anteriorly, and in approximately one-third (35.6%), this displacement exceeded the cutoff value (64.28 µm). Furthermore, we found a significant correlation between ODD shallowing and RNFL thinning during the follow-up (P ≤ 0.03; R ≥ 0.370). We did not find any significant changes in size measurements (P = 0.10) over time. CONCLUSIONS: In approximately one-third of buried ODD, a significant anterior movement occurred over 2 years of follow-up, and this movement was associated with significant RNFL thinning. By contrast, no significant change was detected in the size of the buried ODD.

Prevalence of Optic Disc Drusen in Young Patients With Nonarteritic Anterior Ischemic Optic Neuropathy: A 10-Year Retrospective Study.

BACKGROUND: Nonarteritic anterior ischemic optic neuropathy (NAION) in young patients (age ≤50) accounts for a minority of all cases of NAION and is more highly associated with crowding of the optic nerves and bilateral involvement than NAION in older patients. Optic disc drusen (ODD) are likewise associated with crowded optic nerves and are located in the prelaminar optic nerve head where they could contribute to NAION pathogenesis. The purpose of this study was to determine the prevalence of ODD in the eyes of young NAION patients using modern imaging methods and to compare it to the baseline 1.8%-2.0% prevalence of ODD in the general population. METHODS: In this retrospective study, all young NAION patients (ages 18-50 years, inclusive) seen in 2 tertiary care neuro-ophthalmology clinics (in London, Canada and Copenhagen, Denmark) in the ten-year interval between April 1, 2009, and March 31, 2019, were identified and their medical charts reviewed. Patients were included in the study if ODD were diagnosed by any method (including ophthalmoscopy, ultrasound [US], fundus autofluorescence [FAF], computed tomography [CT], or any optical coherence tomography [OCT] method), or if ODD were excluded by enhanced-depth imaging OCT (EDI-OCT) using the ODD Studies (ODDS) Consortium protocol. The presence or absence of ODD was recorded for each eye. RESULTS: There were 37 eligible patients (74 eyes). Mean age of NAION onset was 38.5 ± 10.0 years, and 23 patients (62%) were men. Patients had undergone the following methods of ODD detection: ophthalmoscopy (37 patients), EDI-OCT (36 patients), FAF (31 patients), US (9 patients), and CT orbits (8 patients). We found a prevalence of ODD of 56.7% in NAION-affected patients and 53.3% in NAION-affected eyes. Only 35.9% of ODD were visible on ophthalmoscopy. Twenty of 21 ODD patients (95.2%) had bilateral ODD. Age of onset and sex did not differ significantly between the ODD-positive group and the ODD-negative group. EDI-OCT outperformed any combination of ophthalmoscopy, US, FAF, and CT at detecting ODD. CONCLUSION: ODD were found with much higher prevalence in young patients with NAION than in the general population and were usually bilateral and buried. ODD may contribute to NAION pathogenesis by exacerbating an underlying compartment syndrome in the crowded "disc at risk." EDI-OCT may be the best imaging modality for ODD detection in future studies.

Peripapillary vessel density in pediatric cases with buried optic disk drusen.

BACKGROUND: To evaluate the retinal nerve fiber layer (RNFL) and peripapillary vascular density (VD) changes in the pediatric group with optic disk drusen (ODD). METHODS: Sixty eyes of 30 patients with buried ODD referred by the pediatric or neurology physicians to ophthalmology clinic with a preliminary diagnosis of papillary edema were included in this retrospective study. Sixty eyes of 30 healthy children were included as the control group. Thickness of RNFL (micrometer) and VD percentages (%) of the superior, inferior, nasal, and temporal quadrants of the peripapillary region of all cases were evaluated with optical coherence tomography angiography (OCT-A) device. RESULTS: The study and control groups were homogeneous in terms of age and gender. VD values were significantly lower in the study group for all four quadrants, when compared to controls (p < 0.001, p < 0.001, p = 0.003, and p < 0.001, for inferior, superior, nasal, and temporal quadrants, respectively. For RFNL thickness measurements, a significant difference between groups was only evident for the nasal quadrant, where the study group had significantly higher nasal RFNL thickness (p = 0.001). CONCLUSION: This study detected decreases in peripapillary VD values in all quadrants and peripapillary RNFL thickening in nasal quadrant in pediatric cases with buried drusen compared to healthy controls. Further studies are necessary to reveal the effects of drusen pathogenesis on optic nerve head perfusion and to understand the underlying mechanisms of related complications.

Inter-Device Comparison of Blue-Light Autofluorescence in Optic Disc Drusen.

PURPOSE: Monochromatic blue-light fundus autofluorescence has proven to be particularly useful for the detection of optic disc drusen (ODD). The aim of this study was to investigate how accurately novel confocal scanners can detect ODD by means of color fundus and autofluorescence images. METHODS: Images were taken in 20 consecutive patients' eyes with funduscopically visible ODD using the TrueColor Eidon AF (60 × 55°) and the spectral domain Spectralis HRA+OCT (30 × 30°). The features of ODD, including localization, extent, and intensity patterns of autofluorescence were compared and correlated with retinal nerve fiber layer (RNFL) thickness in OCT and perimetry findings. RESULTS: Mean patient age was 46 ± 6 years (6 females, 4 males). The TrueColor Eidon AF enabled accurate localization and extent estimation of the drusen area. Drusen presented as a homogeneous signal. The predilection site of ODD, which was nasally pronounced in >80% of cases, was associated with thinning of the RNFL and corresponding visual field defects. CONCLUSION: The TrueColor Eidon AF allows reliable detection of superficial ODD in nondilated eyes. Extended observational studies are needed to determine the value of this noninvasive, nonmydriatic procedure in terms of follow-up and progression analyses of ODD.

Progression Over 5 Years of Prelaminar Hyperreflective Lines to Optic Disc Drusen in the Copenhagen Child Cohort 2000 Eye Study.

BACKGROUND: The purpose of the study was to examine 5-year changes in eyes with optic disc drusen at baseline on optical coherence tomography (OCT) scans and the relation of incident drusen to hyperreflective prelaminar lines. METHODS: The study included children who presented at baseline, when participants were aged 11-12 years, and again 5 years later. Grading for optic disc drusen was made in all. Grading for prelaminar lines was made in all children at follow-up and in eyes with optic disc drusen at baseline. Analyses included associations with scleral canal diameter at baseline in all children with optic disc drusen and a nested control group of 115 children without optic disc drusen. Data are reported as the number of children having at least one drusen or at least one hyperreflective line per person. RESULTS: The analysis included 724 children who attended both rounds of the study. Of these, 11 (1.5%) had optic disc drusen at baseline. Five additional children had developed optic disc drusen at follow-up, whereas optic disc drusen had disappeared in none, so that 16 (2.2%) children had optic disc drusen in one or both eyes at follow-up. Children with optic disc drusen at the 5-year follow-up had had a mean scleral canal diameter of 1,364 µm (interquartile range [IQR] 81 µm), compared with 1,457 µm (IQR 197) µm in 115 nested controls without optic disc drusen (P < 0.001). Optic disc drusen at follow-up were associated with more hypermetropic refraction. All children who had optic disc drusen at follow-up also had prelaminar hyperreflective lines. In addition, such lines were found at follow-up in 24 of the remaining 708 children without optic disc drusen (P < 0.001). Prelaminar hyperreflective lines with or without optic disc drusen were associated with a narrower scleral canal (diameter 1,364 µm, IQR 119 µm) compared with absence of prelaminar lines (1,486 µm, IQR 206 µm; P < 0.0001). CONCLUSION: This study provides the first evidence from a prospective study that small optic discs and prelaminar hyperreflective lines on OCT are risk factors for the development of optic disc drusen. The association between prelaminar hyperreflective lines, hypermetropia, and a narrow scleral canal supports that a crowded disc is an essential predisposing factor for the development of optic disc drusen.

Enhanced Depth Imaging Optical Coherence Tomography of Optic Nerve Head Drusen in Children.

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.

Optic Disc Drusen Associated Anterior Ischemic Optic Neuropathy: Prevalence of Comorbidities and Vascular Risk Factors.

BACKGROUND: Nonarteritic anterior ischemic optic neuropathy (NA-AION) associated with optic disc drusen (ODD) is termed ODD-AION, where NA-AION with no evidence of ODD is simply termed NA-AION. Patients with ODD-AION have been found to be younger than those with NA-AION but with similar vascular risk factors. This study compares the known risk factors for NA-AION between a group with ODD-AION and a similarly aged group with NA-AION. METHODS: A case-control study of 13 patients with ODD-AION and 14 patients with NA-AION diagnosed in the period 2008-2017. All patients underwent an interview designed to evaluate history of vascular risk factors and comorbidities and re-examination including enhanced depth imaging optical coherence tomography to confirm the presence or absence of ODD. RESULTS: No significant differences were found in demographic or clinical characteristics between the ODD-AION and the NA-AION group. Significantly more ODD-AION patients than NA-AION patients had no vascular risk factors (smoking, arterial hypertension, diabetes mellitus, and dyslipidemia) present (P = 0.047). Significantly fewer patients in the ODD-AION group were diagnosed with arterial hypertension or dyslipidemia than in the NA-AION group. CONCLUSIONS: In this cross-sectional study, the ODD-AION patients more often had no vascular risk factors as compared to NA-AION patients, which supports the hypothesis that ODD are an independent risk factor for AION.

Optic disc drusen: a systematic review : Up-to-date and future perspective.

BACKGROUND: Optic disc drusen (ODD) are acellular deposits in the prelaminar optic nerve head. The most accredited theory is that they are secondary to abnormalities in axonal metabolism and degeneration, but the pathogenesis is not clear to date. CLINICAL MANIFESTATION: Although ODD are often considered a benign condition, the great majority of patients with ODD show visual field defects and are at higher risk for developing anterior ischemic optic neuropathy. ODD are classified as superficial or buried, with the latter being often misdiagnosed as papilledema with optic nerve head swelling, leading to an unnecessary investigation for causes of increased intracranial pressure. AIM: The recent technological improvements in OCT imaging which allowed an earlier and more certain diagnosis even of the smallest ODD, renovated the interest around this pathology. However, an updated systematic review is still missing. Therefore, the aim of this work is to provide a concise yet comprehensive overview of the current state of art, focusing on pathophysiology, clinical presentation, diagnostic methods, treatment modalities and potential future perspectives of this condition.

The Influence of Volume and Anatomic Location of Optic Disc Drusen on the Sensitivity of Autofluorescence.

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.