OCT in a Myelinated Retinal Nerve Fiber Syndrome with Reduced Vision.

PURPOSE: The prognosis of success with vision therapy in refractive "amblyopia" associated with the syndrome of myelinated nerve fibers (MRNF), optic disc hypoplasia, and myopia is reported to be poorer than that of anisomyopic amblyopia without these features. The reason for the poorer prognosis has not been well understood. The purpose of this study was to perform spectral domain (SD) ocular coherence tomography (OCT) to determine if there is a structural etiology that may explain the poorer prognosis. CASE REPORTS: Case 1 was a 12-year-old male patient with anisometropic "amblyopia" in the right eye, MRNF denser superiorly, a hypoplastic disc, and a myopic fundus with a flat intact macula. The OCT demonstrated an attenuated photoreceptor integrity line (PIL) in the macula. Case 2 was a 10-year-old male patient with a constant left esotropia, MRNF denser superiorly, a hypoplastic disc, and a myopic fundus with a flat intact macula. The OCT demonstrated an absent PIL. Case 3 was a 58-year-old female patient with a history of diabetic retinopathy OU, long-standing reduced vision in the right eye, MRNF denser superiorly, optic nerve hypoplasia, and a myopic fundus with an intact macula. The OCT demonstrated an absent PIL in the macula. CONCLUSIONS: This case series identifies three patients with the syndrome of MRNF, optic nerve hypoplasia, and anisomyopia in one eye with reduced vision and reports OCT findings using SD-OCT systems. All three patients demonstrated an absence or attenuation of the photoreceptor integrity line (PIL) in the macula in the affected eye. To our knowledge, there is no known association between this syndrome and abnormality of the PIL reported in the literature. Patients with this syndrome may have a guarded prognosis in the success of vision therapy.

A novel retinal biomarker for Parkinson's disease: Quantifying the foveal pit with optical coherence tomography.

BACKGROUND: Optical coherence tomography offers a potential biomarker tool in Parkinson's disease (PD). A mathematical model quantifying symmetry, breadth, and depth of the fovea was applied. METHODS: Nintey-six subjects (72 PD and 24 healthy controls) were included in the study. Macular scans of each eye were obtained on two different optical coherence tomography devices: Cirrus and RTVue. RESULTS: The variables corresponding to the cardinal gradients of the fovea were the most sensitive indicators of PD for both devices. Principal component analysis distinguished 65% of PD patients from controls on Cirrus, 57% on RTVue. CONCLUSION: Parkinson's disease shallows the superior/inferior and to a lesser degree nasal-temporal foveal slope. The symmetry, breadth, and depth model fits optical coherence tomography data derived from two different devices, and it is proposed as a diagnostic tool in PD.

Boucher-Neuhauser Syndrome: Chorioretinal Changes in a Single Case Over Time.

PURPOSE: To describe chorioretinal changes in a single case of Boucher-Neuhauser Syndrome (BNHS) over 45 years of follow-up. Methods: Retrospective chart review was performed. Color fundus photography from 1977 to 2003 was obtained and digitized. Current fundus photography was obtained with widefield imaging. High-resolution spectral-domain optical coherence tomography (OCT) was performed. Genetic analysis was performed using an inherited retinal disorders panel. Results: Fundus examination demonstrated central chorioretinal atrophy with sclerotic choroidal vessels. Short posterior ciliary arteries became more prominent and tortuous over time. Mid-peripheral atrophy extends to the equator and demonstrates a scalloped pattern with islands of atrophy intervening with areas of normal retina. The far periphery remained minimally affected. High-resolution OCT demonstrated outer retinal atrophy and choriocapillaris loss. Genetic testing showed a homozygous variant for PNPLA6 and a heterozygous variant for TYRP1. Conclusion: Chorioretinal changes in BNHS vary in onset and severity. It is important to diagnose this condition in order to begin timely management of visual and systemic sequelae.

Buried disc drusen have hypo-reflective appearance on SD-OCT.

PURPOSE: Buried disc drusen are an important differential diagnosis for papilledema. Spectral domain optical coherence tomography (SD-OCT) affords clinicians with new non-invasive opportunities to probe below the surface of the optic nerve. Clinicians may use the knowledge of this appearance to rule out buried disc drusen in patients with irregular optic nerve borders or a bulging, hyperemic appearance. METHODS: SD-OCTs were obtained in a patient with one surfacing disc druse, identifying the nature of the appearance of this disc druse and others in this and the contralateral eye when imaged with this technology. B-scan ultrasonography was used to confirm the presence of disc drusen. Additional scans in multiple patients with confirmed buried drusen were obtained for comparison. RESULTS: Drusen appear as rounded hyporeflectant areas on SD-OCT, similar in appearance to blood vessels. They share the appearance of cysts but show a fine hyperreflective border anteriorly. These same discrete hyporeflective areas were found at various depths within optic nerve heads with confirmed buried disc drusen. CONCLUSIONS: The hyporeflective appearance may not be anticipated by clinicians, as B-scans show calcified drusen as hyperreflective on echo. It is hypothesized that the hyporeflectant appearance of drusen is due to a constancy in refractive index through the druse, as OCT detects changes in optical reflectivity. Thus, drusen are likely dense and homogenous. SD-OCT may be more useful in those patients with buried disc drusen which are not calcified as B-scan often contributes little in such cases.

Panoramic autofluorescence: highlighting retinal pathology.

PURPOSE: Recent technological advances in fundus autofluorescence (FAF) are providing new opportunities for insight into retinal physiology and pathophysiology. FAF provides distinctly different imaging information than standard photography or color separation. A review of the basis for this imaging technology is included to help the clinician understand how to interpret FAF images. Cases are presented to illustrate image interpretation. METHODS: Optos, which manufactures equipment for simultaneous panoramic imaging, has recently outfitted several units with AF capabilities. Six cases are presented in which panoramic autofluorescent (PAF) images highlight retinal pathology, using Optos' Ultra-Widefield technology. Supportive imaging technologies, such as Optomap® images and spectral domain optical coherence tomography (SD-OCT), are used to assist in the clinical interpretation of retinal pathology detected on PAF. RESULTS: Hypofluorescent regions on FAF are identified to occur along with a disruption in the photoreceptors and/or retinal pigment epithelium, as borne out on SD-OCT. Hyperfluorescent regions on FAF occur at the advancing zones of retinal degeneration, indicating impending damage. PAF enables such inferences to be made in retinal areas which lie beyond the reach of SD-OCT imaging. PAF also enhances clinical pattern recognition over a large area and in comparison with the fellow eye. Symmetric retinal degenerations often occur with genetic conditions, such as retinitis pigmentosa, and may impel the clinician to recommend genetic testing. CONCLUSIONS: Autofluorescent ophthalmoscopy is a non-invasive procedure that can detect changes in metabolic activity at the retinal pigment epithelium before clinical ophthalmoscopy. Already, AF is being used as an adjunct technology to fluorescein angiography in cases of age-related macular degeneration. Both hyper- and hypoautofluorescent changes are indicative of pathology. Peripheral retinal abnormalities may precede central retinal impacts, potentially providing early signs for intervention before impacting visual acuity. The panoramic image enhances clinical pattern recognition over a large area and in comparison between eyes. Optos' Ultra-Widefield technology is capable of capturing high-resolution images of the peripheral retina without requiring dilation.

Visual acuity recovery in a case of idiopathic retinal vasculitis aneurysms and neuroretinitis.

PURPOSE: To describe the visual recovery after intravitreal injections of the antivascular endothelial growth factor, bevacizumab, in a case of vaso obliteration from idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN). The name IRVAN was given to the condition to highlight the key findings present in the disease. IRVAN is a severe, sight threatening condition that can lead to peripheral capillary non-perfusion and vision loss from the ischemic sequelae of vascular occlusion. Panretinal photocoagulation (PRP) is the current standard of care for IRVAN but visual outcome is poor if PRP is initiated after neovascularization develops. Intravitreal bevacizumab has success at treating neovascularization from other ischemic retinopathies and inflammatory retinal conditions that have similar characteristics to IRVAN. CASE REPORT: This case report describes a patient with decreased vision in the OS. The patient presented with best-corrected visual acuity of 20/20 in the OD and count fingers at 4 ft in the OS. Evaluation revealed findings consistent with an advanced stage of IRVAN. Anterior and posterior neovascularization had developed from extensive capillary non-perfusion in both retinas. A dense vitreous hemorrhage blocked vision OS. Bilateral intravitreal injections of bevacizumab and extensive PRP were given in the area of retinal ischemia for treatment. After 4 months, the patient's vision had improved from count fingers in the OS to 20/40. CONCLUSIONS: IRVAN has favorable outcomes when treated with a combination of PRP and intravitreal injections of antivascular endothelial growth factor. This case demonstrates the effectiveness of this combination treatment in a case of IRVAN with both posterior and anterior neovascularization.

A proposed mechanism influencing structural patterns in X-linked retinoschisis and stellate nonhereditary idiopathic foveomacular retinoschisis.

OBJECTIVE: To explore the structural differences between X-linked retinoschisis (XLR) and stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) using swept-source optical coherence tomography angiography (SS-OCTA). METHODS: A case series of two patients, a 9-year-old male with XLR and a 58-year-old woman with SNIFR were imaged with swept-source optical coherence tomography angiography (SS-OCTA; PLEX Elite 900, Carl Zeiss Meditec, Inc, Dublin, CA). Automated segmentation was manually adjusted to include the areas of retinoschisis within en face flow and structural slabs. The flow data were binarized using ImageJ 1.51s (Wayne Rasband, National Institutes of Health, USA, http://imagej.nih.gov.ij ) and superimposed onto the structural slab. RESULTS: In the eye with XLR, OCTA flow data superimposed on the structural slab demonstrated flow signal within numerous bridging structures connecting the inner and outer plexiform layers containing the intermediate (ICP) and deep (DCP) capillary plexuses. In contrast, the same technique applied to the eye with SNIFR demonstrated an absence of flow signal in the cystic retinal spaces within Henle's fiber layer. CONCLUSIONS: The vascular pattern of bridging vessels between the ICP and DCP is closely related to the structural "retinoschisis" pattern of XLR and appears to be structurally different from that seen in SNIFR. Moreover, the connecting vessels appear to be highly represented and regularly distributed, thereby supporting a serial arrangement of the retinal capillary plexuses within the perifoveal macula.

Curvilinear, symmetrical, and profound pigment deposition on the posterior lens capsule in a patient with bilateral pigmentary dispersion syndrome.

INTRODUCTION: The classic presentation of pigmentary dispersion syndrome (PDS) often consists of midperipheral iris transillumination defects, Krukenberg's spindle, and dense homogeneous trabecular pigmentation. Other subtle, sometimes overlooked features include pigment on the lens zonules, pigment on the anterior lens capsule and pigment along the equator of the posterior lens capsule. CASE: This unique presentation of PDS presented with bilateral, dense, oblique, and symmetrical pigment deposition along the posterior lens capsule that changed in shape, density, and extent over the span of 3 years. DISCUSSION: There have been few reports in the literature that describe a central accumulation of pigment along the posterior lens capsule associated with PDS. There are reported cases of pigment deposition along the central aspect of the posterior lens capsule, some changing over time, although none were bilateral and symmetrical. There are suggestions that perhaps this central pigment deposition is related to a break in the ligament of Weiger, allowing communication between the posterior chamber and posterior lens capsule. This is a case in which curvilinear, symmetrical, and changing pigment deposition on the posterior lens capsule is suggestive of perhaps another key features of PDS.

Male prevalence of acquired color vision defects in asymptomatic carriers of Leber's hereditary optic neuropathy.

PURPOSE: Leber's hereditary optic neuropathy (LHON) is a maternally inherited disease resulting in loss of central vision and dyschromatopsia, caused by mitochondrial DNA point mutations. However, only a subset of the mutation carriers becomes affected, with a higher penetrance in males. This study was conducted to investigate chromatic losses in asymptomatic carriers of the LHON mutation. METHODS: Monocular chromatic discrimination was studied with the Cambridge Colour Test (CCT; Cambridge Research Systems, Ltd., Rochester, UK) along the protan, deutan, and tritan cone isolation axes in 46 LHON carriers (15 men) belonging to the same LHON maternal lineage and 74 age-matched control subjects (39 men). Inclusion criteria were absence of ophthalmic complaints and clear ocular media. A detailed neuro-ophthalmic examination was performed in all the LHON carriers. RESULTS: The differences in threshold between carriers and control subjects were significant for the three cone isolation axes at P < 0.0001. Sixty-five percent of the carriers had abnormal protan and/or deutan thresholds; some of those with higher thresholds also had elevated tritan thresholds (13%). The male thresholds were higher and more frequent than those of the women for the protan and deutan axes (ANOVA; P < 0.05), but not for tritan thresholds. In the most severe losses, the women had instances of diffuse defect whereas all the men displayed a red-green defect. CONCLUSIONS: Male LHON asymptomatic carriers had color vision losses with the red-green pattern of dyschromatopsia typical of patients affected with LHON, which includes elevation of tritan thresholds as well. This predominantly parvocellular (red-green) impairment is compatible with the histopathology of LHON, which affects mostly the papillomacular bundle. In contrast with male losses, female losses were less frequent and severe. These gender differences are relevant to understanding LHON pathophysiology, suggesting that hormonal factors may be of great importance.

Pervasive ocular anomalies in posterior microphthalmos.

BACKGROUND: Posterior microphthalmos is a relatively rare condition that has been reported to coexist with several other ophthalmic conditions. However, to the best of the authors' knowledge, there are no previous reports that have found posterior microphthalmos and refractive, binocular, retinal, and neurologic considerations, along with a possible hereditary component. The following report documents the coexistence of posterior microphthalmos with severe hyperopia, esotropia, macular folds, and optic nerve hypoplasia in a pair of siblings. CASE REPORT: A 9-year-old Hispanic girl presented for a comprehensive eye examination. Best-corrected visual acuity (VA) was reduced in both eyes with poorer VA in the right eye. Binocular testing found a small angle constant right esotropia (ET). On dilated fundus examination, a peculiar, elevated, dolphin-shaped folding of the macula was identified, the right eye greater than the left eye, that extended toward an anomalous optic nerve head in both eyes (OU), presumed to be a disc hypoplasia. The patient's brother, who also exhibited severe hyperopia and ET, presented with a similar acuity reduction, a nearly identical folded macular appearance, the right eye more so than the left eye, and a probable optic nerve hypoplasia. Pachymetry, ultrasonography, and ocular coherence tomography imaging of both siblings found thickened corneas in the presence of posterior microphthalmos OU and macular folds affecting only the retina, leaving the choroid and sclera unaffected. CONCLUSIONS: Posterior microphthalmos may exist in the presence of ocular anomalies along with refractive, binocular, retinal, neurologic, and genetic considerations. In this case, optical coherence tomography provided information on the internal morphology of the macular folds, which helped direct the differential diagnosis. The similar presentation among siblings supports a hereditary component that warrants further investigation.

A Review of Mitochondrial Optic Neuropathies: From Inherited to Acquired Forms.

In recent years, the term mitochondrial optic neuropathy (MON) has increasingly been used within the literature to describe a group of optic neuropathies that exhibit mitochondrial dysfunction in retinal ganglion cells (RGCs). Interestingly, MONs include genetic aetiologies, such as Leber hereditary optic neuropathy (LHON) and dominant optic atrophy (DOA), as well as acquired aetiologies resulting from drugs, nutritional deficiencies, and mixed aetiologies. Regardless of an inherited or acquired cause, patients exhibit the same clinical manifestations with selective loss of the RGCs due to mitochondrial dysfunction. Various novel therapies are being explored to reverse or limit damage to the RGCs. Here we review the pathophysiology, clinical manifestations, differential diagnosis, current treatment, and promising therapeutic targets of MON.

ACUTE ZONAL OCCULT OUTER RETINOPATHY AFFECTING THE PERIPHERAL RETINA WITH CENTRIPETAL PROGRESSION.

PURPOSE: To describe a variant of acute zonal occult outer retinopathy (AZOOR) that has concentric involvement of the peripheral retina with centripetal progression toward the posterior pole. METHODS: Three patients with AZOOR were reported to show peripheral concentric zonal involvement with centripetal progression of their disease from the periphery to the posterior fundus. RESULTS: All three cases involved elderly hyperopic women with a history of autoimmune disease. All six eyes showed bilateral central peripapillary AZOOR lesions that progressed in a centrifugal manner to the periphery. Five of the six eyes showed the presence of concentric peripheral zonal abnormalities that progressed in a centripetal manner to the posterior pole. In one case, the peripheral and central zonal abnormalities became confluent, leaving only a small island of normal retina temporal to the fovea. CONCLUSION: A variant of AZOOR may involve the peripheral retina, causing concentric zonal atrophy with centripetal progression, with central peripapillary zonal abnormalities that have centrifugal progression. This may eventually lead to widespread atrophic degeneration with severe visual field loss. Wide-field imaging of the peripheral retina and monitoring of the visual fields are important to document this rare atypical presentation of AZOOR and any subsequent disease progression.

Novice Reviewers Retain High Sensitivity and Specificity of Posterior Segment Disease Identification with iWellnessExam™.

Introduction. Four novices to Spectral Domain Optical Coherence Tomography (SD-OCT) image review were provided a brief lecture on the interpretation of iVue iWellnessExam™ findings (available on iVue® SD-OCT, Optovue, Inc., Fremont, CA). For a cohort of 126 (Confirmed) Normal, 101 (Confirmed) Disease subjects, iWellnessExam™ OD, OS, and OU reports were provided. Each novice independently reviewed and sorted the subjects into one of four categories: normal, retinal disease, optic nerve (ON) disease, and retinal + ON disease. Their accuracy is compared between the novices and with an expert reviewer. Results. Posterior segment disease was properly detected by novices with sensitivities of 90.6%, any disease; 84.3%, retinal disease; 88.0%, ON disease; expert sensitivity: 96.0%, 95.5%, and 90.0%, respectively; specificity: 84.3%, novices; 99.2%, expert. Novice accuracy correlates best with clinical exposure and amount of time spent reviewing each image set. The novices' negative predictive value was 92.0% (i.e., very few false negatives). Conclusions. Novices can be trained to screen for posterior segment disease efficiently and effectively using iWellnessExam™ data, with high sensitivity, while maintaining high specificity. Novice reviewer accuracy covaries with both clinical exposure and time spent per image set. These findings support exploration of training nonophthalmic technicians in a primary medical care setting.

New insights into Stargardt disease with multimodal imaging.

A 20-year-old woman with bilateral mild blurring of vision presented with a bull's eye maculopathy and was diagnosed with Stargardt disease, confirmed with genetic testing. The authors present several novel multimodal imaging findings including multicolor and multi-spectral imaging that enhanced visualization of perifoveal flecks, fundus autofluorescence that revealed both perifoveal and perimacular rings of hyperautofluorescence, adaptive optics imaging that revealed unprecedented visualization of cones at the fovea due to decreased cone density, and spectral-domain optical coherence tomography that identified thickening and increased hyperreflectivity of the external limiting membrane as a possible transient biomarker of early Stargardt disease.

Optic disk evaluation and utility of high-tech devices in the assessment of glaucoma.

BACKGROUND: Every clinician has at one time or another examined a patient who was misdiagnosed as having glaucoma or whose diagnosis of glaucoma was missed. Although glaucoma can exist with normal intraocular pressures, clinicians often rely on the presence of visual-field defects and the degree of optic disk cupping to direct care. However, assessment of cupping is but one small part of optic disk evaluation in glaucoma, and other features of the optic nerve head and retinal nerve fiber layer must be closely inspected to help diagnose borderline cases. In addition, glaucoma can exist without visual-field loss. High-tech devices offer an added dimension in the objective assessment of structure when subjective tests of function and/or ophthalmoscopic observations are equivocal. METHODS: This article details the various parameters of optic disk and retinal nerve fiber layer evaluation and their significance in the assessment of glaucoma. In addition, the role of four high-tech devices is evaluated for their utility in the assessment and progression of glaucomatous damage. CONCLUSIONS: When one attempts to classify a patient as having glaucoma, the degree of cupping and the presence or absence of visual field loss can be misleading. Prior to definitive diagnosis, a thorough evaluation of the optic disk and retinal nerve fiber layer, and appropriate use of high-tech devices, should help reduce the under-diagnosis and overdiagnosis of this disease.

Glaucoma without cupping.

BACKGROUND: Although glaucoma can exist with normal intraocular pressures (IOPs), clinicians still rely on the presence of a large cup to "flag" suspects, regardless of IOP, whereas a small cup at the same pressure level is often ignored. High-tech instruments offer a new dimension of evaluation in the objective assessment of structure when subjective tests of function and/or ophthalmoscopic observations are equivocal. CASE REPORTS: Thirteen cases are presented and show evidence of glaucoma based on glaucomatous visual-field defects, often with steadily rising intraocular pressures and retinal nerve fiber layer loss. Surprisingly, these patients maintained small C/D ratios. Accordingly, ophthalmoscopy and/or disk topography classified these disks as normal. CONCLUSIONS: Although unrecognized in virtually the entire world's ophthalmic literature, normal cup glaucoma is a real clinical entity. At least half the normal cup glaucoma cases presented herein have disk drusen (obvious, subtle, or occult), while others are highly myopic and/or have documented IOP spikes. Several of the cases defy classification and explanation at the present time.

Spectral-domain optical coherence tomography staging and autofluorescence imaging in achromatopsia.

IMPORTANCE Evidence is mounting that achromatopsia is a progressive retinal degeneration, and treatments for this condition are on the horizon. OBJECTIVES To categorize achromatopsia into clinically identifiable stages using spectral-domain optical coherence tomography and to describe fundus autofluorescence imaging in this condition. DESIGN, SETTING, AND PARTICIPANTS A prospective observational study was performed between 2010 and 2012 at the Edward S. Harkness Eye Institute, New York-Presbyterian Hospital. Participants included 17 patients (aged 10-62 years) with full-field electroretinography-confirmed achromatopsia. MAIN OUTCOMES AND MEASURES Spectral-domain optical coherence tomography features and staging system, fundus autofluorescence and near-infrared reflectance features and their correlation to optical coherence tomography, and genetic mutations served as the outcomes and measures. RESULTS Achromatopsia was categorized into 5 stages on spectral-domain optical coherence tomography: stage 1 (2 patients [12%]), intact outer retina; stage 2 (2 patients [12%]), inner segment ellipsoid line disruption; stage 3 (5 patients [29%]), presence of an optically empty space; stage 4 (5 patients [29%]), optically empty space with partial retinal pigment epithelium disruption; and stage 5 (3 patients [18%]), complete retinal pigment epithelium disruption and/or loss of the outer nuclear layer. Stage 1 patients showed isolated hyperreflectivity of the external limiting membrane in the fovea, and the external limiting membrane was hyperreflective above each optically empty space. On near infrared reflectance imaging, the fovea was normal, hyporeflective, or showed both hyporeflective and hyperreflective features. All patients demonstrated autofluorescence abnormalities in the fovea and/or parafovea: 9 participants (53%) had reduced or absent autofluorescence surrounded by increased autofluorescence, 4 individuals (24%) showed only reduced or absent autofluorescence, 3 patients (18%) displayed only increased autofluorescence, and 1 individual (6%) exhibited decreased macular pigment contrast. Inner segment ellipsoid line loss generally correlated with the area of reduced autofluorescence, but hyperautofluorescence extended into this region in 2 patients (12%). Bilateral coloboma-like atrophic macular lesions were observed in 1 patient (6%). Five novel mutations were identified (4 in the CNGA3 gene and 1 in the CNGB3 gene). CONCLUSIONS AND RELEVANCE Achromatopsia often demonstrates hyperautofluorescence suggestive of progressive retinal degeneration. The proposed staging system facilitates classification of the disease into different phases of progression and may have therapeutic implications.

Sensitivity and specificity of the iVue iWellnessExam™ in detecting retinal and optic nerve disorders.

BACKGROUND: The purpose of this study was to assess the specificity and sensitivity of the iWellnessExam™ screening protocol available on iVue® spectral domain optical coherence tomography (SD-OCT), in a cohort of confirmed normal subjects and subjects with confirmed disease. METHODS: In total, 126 of 132 confirmed normal subjects and 101 of 107 subjects with confirmed disease were included for analysis. Of the patients with confirmed disease, 67 had retinal disease, 50 had optic nerve disease, and 16 had both retinal and optic nerve pathology. All subjects were screened on the iWellnessExam protocol. Screen shots of the OD, OS, and OU comparison data were obtained and deidentified for reviewer analysis. Based on these data alone, each subject was sorted by a well trained eye care clinician into one of four categories (1, normal; 2, retinal disease; 3, optic nerve disease; 4, retinal and optic nerve disease). RESULTS: Of the confirmed normal subjects, 125 of 126 were correctly identified as normal (specificity 99%). Retinal and/or optic nerve disease was correctly detected in 97 of 101 patients with confirmed disease (category 2, 3, 4), retinal pathology was correctly detected in 64 of 67 patients with retinal disease (category 2, 4), and optic nerve pathology was properly detected in 45 of 50 patients with optic nerve disease (category 3, 4), with a sensitivity of 96%, 95.5%, and 90%, respectively. CONCLUSION: The iWellnessExam offers the health care provider an excellent method for identifying eyes at risk using very reliable technology. High specificity and sensitivity was obtained when reviewed by a well trained eye care clinician. It would be valuable to repeat the study with a novice and/or student clinician reviewing the same data set to ascertain interobserver variability, as well as the impact of clinical experience on accurate referral, based on the screening data.