Inferior Ophthalmic Vein-Dominant Dural Cavernous Fistula Embolization via Combined Orbitotomy and Direct Puncture of Inferior Ophthalmic Vein: A Case Report and Literature Review.

Dural carotid-cavernous fistulas (DCF) typically drain into the superior ophthalmic vein. Predominant involvement of the inferior ophthalmic vein (IOV) is rare, with only 4 documented cases in the literature. Here, the authors describe a case of a 51-year-old man who presented with acute left-sided proptosis, dysmotility, and vision loss and was found to have an IOV-dominant type D dural carotid-cavernous fistulas. The fistula could not be embolized by transfemoral endovascular access or orbitotomy alone and was ultimately managed with combined orbitotomy and direct IOV puncture. All previous reports of IOV-dominant dural carotid-cavernous fistulas in the literature were similarly inaccessible via the transfemoral approach. This case highlights the challenges of IOV cutdown and proposes an alternative management strategy. When IOV cutdown is precluded by the fragile, collapsed, or deep nature of the vessel, conversion to percutaneous IOV puncture may offer a safe and effective approach and mitigate the risks of direct puncture alone.

Upper Limit of Retinal Nerve Fibre Layer Thickness in Patients with Pseudopapilloedema.

An initial misdiagnosis of papilloedema in a patient with optic nerve head swelling can be anxiety-provoking and may result in unnecessary, invasive, and costly tests. Cirrus high definition, spectral domain-optical coherence tomography (Cirrus HD-OCT) may provide a rapid and non-invasive test. We sought to determine an upper limit of average retinal nerve fibre layer (RNFL) thickness in patients with pseudopapilloedema without visible drusen using Cirrus HD-OCT that could be utilised in conjunction with the clinical presentation and physical examination when managing patients with optic nerve head swelling. Inclusion criteria consisted of at least two neuro-ophthalmological visits and repeated imaging of the optic nerve head with Cirrus HD-OCT at least 6 months apart. Exclusion criteria included clinically visible drusen along with previous or concomitant diagnosis of retinal or other optic nerve pathology. Thirty-eight eyes from 19 patients with pseudopapilloedema were included in this study. The upper limit of average RNFL thickness was defined as two standard deviations above the mean of the average RNFL thickness and was calculated to be 158.65 µm for scans obtained with Cirrus HD-OCT devices. A patient with suspected optic nerve head swelling, an average RNFL thickness less than 158.65 µm, and no other evidence of papilloedema or neurological signs or symptoms can be managed with serial follow-ups with OCT imaging for at least 6 months. If the patient continues to have no clinical symptoms suggesting increased intracranial pressure and the average RNFL thickness is stable, the likelihood of papilloedema is minimal.

Vitreoretinal Findings in Nonarteritic Ischemic Optic Neuropathy.

BACKGROUND: Although nonarteritic anterior ischemic optic neuropathy (NAION) is considered a disorder that primarily affects the optic nerve head, optical coherence tomography (OCT) shows peripapillary and foveal subretinal fluid associated with optic disc swelling from NAION. We sought to further evaluate retinal and vitreous changes in patients with NAION. METHODS: Patients diagnosed with NAION at the New England Eye Center between 2013 and 2017 were evaluated using OCT. The presence and distribution of subretinal fluid was analyzed. Evidence of other vitreoretinal changes, including vitreopapillary traction (VPT) and the presence of hyperreflective dots (HRD), were also determined. RESULTS: Twenty-five eyes from 20 patients who presented within 4 weeks of symptom onset were assessed. Peripapillary subretinal fluid was seen in 16 eyes (64%). Of those eyes, the subretinal fluid extended into the macula in 4 eyes (16%). Visual acuity improved in 2 of 4 eyes after subfoveal fluid resolution. Intraretinal cysts located in the peripapillary region were seen in 8 eyes (32%), HRD were noted in 11 (44.0%). There was no evidence of VPT. CONCLUSIONS: A substantial number of patients with NAION have subretinal fluid on OCT, consistent with prior reports. Resolution of subfoveal fluid may result in some recovery of visual acuity. Other retinal changes, such as intraretinal cysts and HRD, are present but have unclear implications. We did not find evidence of a primary role of VPT in the pathophysiology of NAION.

Recent advances and future directions on the use of optical coherence tomography in neuro-ophthalmology.

Optical coherence tomography (OCT) is a noninvasive imaging technique used to qualitatively and quantitatively analyze various layers of the retina. OCT of the retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) is particularly useful in neuro-ophthalmology for the evaluation of patients with optic neuropathies and retrochiasmal visual pathway disorders. OCT allows for an objective quantification of edema and atrophy of the RNFL and GCIPL, which may be evident before obvious clinical signs and visual dysfunction develop. Enhanced depth imaging OCT allows for visualization of deep structures of the optic nerve and has emerged as the gold standard for the detection of optic disc drusen. In the evaluation of compressive optic neuropathies, OCT RNFL and GCIPL thicknesses have been established as the most important visual prognostic factor. There is increasing evidence that inclusion of OCT as part of the diagnostic criteria for multiple sclerosis (MS) increases its sensitivity. Moreover, OCT of the RNFL and GCIPL may be helpful in the early detection and monitoring the treatment of conditions such as MS and Alzheimer's disease. OCT is an important aspect of the neuro-ophthalmologic assessment and its use is likely to increase moving forward.

Ganglion Cell Complex Analysis as a Potential Indicator of Early Neuronal Loss in Idiopathic Intracranial Hypertension.

Reliable visual field testing is the gold standard in identifying future vision loss in patients with Idiopathic Intracranial hypertension (IIH). However, when field performance is unreliable, GCC analysis may be useful. We evaluated IIH patients over three visits: initial visit, follow-up visit and a third visit, almost 1 year later. We evaluated mean deviation (MD), GCC and RNFL at presentation and the second visit and compared it to the mean deviation (MD) on fields at the third visit. As early as the second visit, GCC loss correlated with visual field results seen at the third visit.

IMMUNE RECONSTITUTION INFLAMMATORY SYNDROME CAUSING PROGRESSIVE OPTIC NERVE EDEMA IN CRYPTOCOCCAL MENINGITIS.

PURPOSE: To report an human immunodeficiency virus-positive patient undergoing therapy for cryptococcal meningitis who developed progressive optic disk edema that was steroid responsive. METHODS: Observational case report. RESULTS: One month after commencing antifungal treatment for cryptococcal meningitis, the patient developed bilateral, progressive, recurrent optic disk edema with subretinal fluid that coincided with initiation of highly active antiretroviral therapy and recovery of CD4 cell counts. Lumbar puncture revealed normal opening pressure, and cerebrospinal fluid showed no recurrence of cryptococcal infection. There was no evidence of uveitis. The patient rapidly improved with a 5-day course of high-dose intravenous methylprednisolone. CONCLUSION: Recurrent optic disk edema with loss of vision after treatment of cryptococcal meningitis in the setting of normal intracranial pressure may represent a unique manifestation of immune reconstitution inflammatory syndrome localized to the optic nerve without uveitis. This is consistent with the temporal relationship between starting highly active antiretroviral therapy, CD4 count recovery, and the development of progressive disk edema in the study patient. Isolated optic nerve inflammation as a manifestation of immune reconstitution inflammatory syndrome has not been widely reported.

Characterization of Charcot-Marie-Tooth optic neuropathy.

Varying degrees of optic neuropathy can be seen in patients with Charcot-Marie-Tooth (CMT) disease. To define and characterize the extent of optic neuropathy in patients with CMT2A and CMT1A, two patients from both sub-classifications were evaluated. All patients underwent complete neuro-ophthalmic examinations, and optical coherence (OCT) measurements of the retinal nerve fiber layer (RNFL) and ganglion cell layer complex (GCC) were obtained, along with pattern visual evoked potential (VEP) and pattern electroretinogram (ERG) recordings. RNFL thickness measurements were decreased in both patients with CMT2A, and normal in both patients with CMT1A. GCC measurements were decreased in both patients with CMT2A, mildly decreased in one patient with CMT1A and normal in the second CMT1A patient. VEP latencies were delayed in one patient with CMT2A and one patient with CMT1A. VEP latencies were immeasurable in the other CMT2A patient and not obtained in the second CMT1A patient. Pattern ERG P50-N95 amplitudes were decreased in both patients with CMT2A and normal in one patient with CMT1A. The pattern ERG was immeasurable in the second patient with CMT1A. The pattern of RNFL and GCC thinning in CMT2A with optic neuropathy, a subset of HMSN VI, closely resembles that seen in other mitochondrial optic neuropathies.

Ganglion cell layer complex measurements in compressive optic neuropathy.

PURPOSE OF REVIEW: Neuroophthalmologists and neurosurgeons are often asked by their patients what their visual prognosis will be after decompression of an optic chiasm lesion. Previous methods have been studied but have not provided consistent guidance. However, a recent algorithm which allows for retinal ganglion cell analysis from optical coherence tomography (OCT) may be more helpful. RECENT FINDINGS: Recent studies have shown that ganglion cell layer complex (GCC) measurements from OCT strongly correlate with the visual field loss associated with compressive optic neuropathies. For example, GCC measurements show corresponding binasal thinning with bitemporal hemianopia. Some investigators have also shown that more preserved GCC thickness is preoperatively associated with better postsurgical outcome. Interestingly, some patients experience almost complete recovery of visual fields despite considerable GCC thinning, and others may have GCC loss before they develop demonstrable visual field defects. SUMMARY: GCC measurements on OCT strongly correlate with visual field defects from optic chiasm compressive lesions and may help with regard to prognosis following treatment. However, considerable visual recovery is possible despite persistent GCC loss.

Optical Coherence Tomography Angiography in Nonarteritic Anterior Ischemic Optic Neuropathy.

BACKGROUND: Optical coherence tomography angiography (OCTA) has demonstrated good utility in qualitative analysis of retinal and choroidal vasculature and therefore may be relevant in the diagnostic and treatment efforts surrounding nonarteritic anterior ischemic optic neuropathy (NAION). METHODS: Retrospective, cross-sectional study of 10 eyes of 9 patients with a previous or new diagnosis of NAION that received imaging with OCTA between November 2015 and February 2016. Two independent readers qualitatively analyzed the retinal peripapillary capillaries (RPC) and peripapillary choriocapillaris (PCC) for flow impairment. Findings were compared with automated visual field and structural optical coherence tomography (OCT) studies. RESULTS: Flow impairment seen on OCTA in the RPC corresponded to structural OCT deficits of the retinal nerve fiber layer (RNFL) and ganglion cell layer complex (GCC) in 80% and 100% of eyes, respectively, and to automated visual field deficits in 90% of eyes. Flow impairment seen on OCTA in the PCC corresponded to structural OCT deficits of the RNFL and GCC in 70% and 80% of eyes, respectively, and to visual field deficits in 60%-80% of eyes. CONCLUSIONS: OCTA can noninvasively visualize microvascular flow impairment in patients with NAION.

Ganglion Cell Complex Loss in Chiasmal Compression by Brain Tumors.

BACKGROUND: Patterns of ganglion cell complex (GCC) loss detected by optical coherence tomography provide an objective measure of optic nerve injury. These patterns aid in early diagnosis and localization of chiasmal lesions. METHODS: Twenty-three patients with chiasmal compression seen between 2010 and 2015 were imaged with the Cirrus high-definition optical coherence tomography macular cube 512 × 128, retinal nerve fiber layer (RNFL) scan protocols and automated (30-2 Humphrey) visual fields (VFs). Age-matched controls were included for comparison. Generalized estimating equations were performed comparing RNFL and GCC thicknesses between patients and their controls. Effect size (d) was calculated to assess the magnitude of difference between patients and controls. The average GCC and RNFL thicknesses also were correlated with VF mean deviation (MD). Pre operative average GCC thickness was correlated to post operative VF MD. RESULTS: Patterns of GCC thinning corresponded to VF defects. The average GCC thickness was 67 ± 9 µm in patients and 86 ± 5 µm in controls (P < 0.001). The effect size was the greatest for GCC thickness (d = 2.72). The mean deviation was better correlated with GCC thickness (r =0.25) than RNFL thicknesses (r =0.15). Postoperatively, VF MD improved in 7 of 8 patients with persistent nasal GCC thinning. Six patients had no VF defect and showed statistically significant loss of GCC compared with controls (P = 0.001). CONCLUSIONS: Distinct patterns of GCC loss were identified in patients with chiasmal compression. Binasal GCC loss was typical and could be seen with minimal or no detectable VF loss. Thinning of the GCC may be detected before loss of the RNFL in some patients. After decompression, the majority of patients showed improvement in VF despite persistent GCC loss. Patients with less GCC loss before decompression had better postoperative VFs. Therefore, GCC analysis may be an objective method to diagnose and follow patients with chiasmal lesions.

Ocular fundus photography of patients with focal neurologic deficits in an emergency department.

OBJECTIVES: We evaluated the frequency and predictive value of ocular fundus abnormalities among patients who presented to the emergency department (ED) with focal neurologic deficits to determine the utility of these findings in the evaluation of patients with suspected TIA and stroke. METHODS: In this cross-sectional pilot study, ocular fundus photographs were obtained using a nonmydriatic fundus camera. Demographic, neuroimaging, and ABCD(2) score components were collected. Photographs were reviewed for retinal microvascular abnormalities. The results were analyzed using univariate statistics and logistic regression modeling. RESULTS: Two hundred fifty-seven patients presented to the ED with focal neurologic deficits, of whom 81 patients (32%) had cerebrovascular disease (CVD) and 144 (56%; 95% confidence interval: 50%-62%) had retinal microvascular abnormalities. Focal and general arteriolar narrowing increased the odds of clinically diagnosed CVD by 5.5 and 2.6 times, respectively, after controlling for the ABCD(2) score and diffusion-weighted imaging. These fundus findings also significantly differentiated TIA from non-CVD, even after controlling for the ABCD(2) score. CONCLUSIONS: Focal and general arteriolar narrowing were independent predictors of CVD overall, and TIA alone, even after controlling for the ABCD(2) score and diffusion-weighted imaging lesions. The inclusion of nonmydriatic ocular fundus photographs in the evaluation of patients presenting to the ED with focal neurologic deficits may assist in the differentiation of stroke and TIA from other causes of focal neurologic deficits.

Intact retinal tissue and retinal pigment epithelium identified within a coloboma by high-speed, ultrahigh-resolution optical coherence tomography.

PURPOSE: The purpose of this study was to report on a posterior segment coloboma manifesting unusual morphology as determined by high-speed, ultrahigh-resolution optical coherence tomography imaging. METHODS: A 47-year-old woman with bilateral colobomas was evaluated by fundus examination and high-speed, ultrahigh-resolution optical coherence tomography imaging. RESULTS: Imaging with high-speed, ultrahigh-resolution optical coherence tomography showed intact retinal pigment epithelium within the posterior segment coloboma. Most of the retinal layers seemed to continue into the coloboma, although they exhibited slight attenuation. The external limiting membrane was clearly visible continuing within the coloboma, suggesting that Müller cells and the inner segments of the photoreceptors were still present in this area. The junction between the inner and outer segments of the photoreceptors ended at the margin of the coloboma, which may be because of either photoreceptor disruption or a change in the orientation of the outer segments. CONCLUSION: High-speed, ultrahigh-resolution optical coherence tomography imaging showed the presence of Müller cells and photoreceptor inner segments within a posterior segment coloboma. The retinal pigment epithelium was intact within the coloboma, representing an unusual morphology.

Comparison of spectral/Fourier domain optical coherence tomography instruments for assessment of normal macular thickness.

PURPOSE: The purpose of this study was to report normal macular thickness measurements and assess reproducibility of retinal thickness measurements acquired by a time-domain optical coherence tomography (OCT) (Stratus, Carl Zeiss Meditec, Inc., Dublin, CA) and three commercially available spectral/Fourier domain OCT instruments (Cirrus HD-OCT, Carl Zeiss Meditec, Inc.; RTVue-100, Optovue, Inc., Fremont, CA; 3D OCT-1000, Topcon, Inc., Paramus, NJ). METHODS: Forty randomly selected eyes of 40 normal, healthy volunteers were imaged. Subjects were scanned twice during 1 visit and a subset of 25 was scanned again within 8 weeks. Retinal thickness measurements were automatically generated by OCT software and recorded after manual correction. Regression and Bland-Altman plots were used to compare agreement between instruments. Reproducibility was analyzed by using intraclass correlation coefficients, and incidence of artifacts was determined. RESULTS: Macular thickness measurements were found to have high reproducibility across all instruments with intraclass correlation coefficients values ranging 84.8% to 94.9% for Stratus OCT, 92.6% to 97.3% for Cirrus Cube, 76.4% to 93.7% for RTVue MM5, 61.1% to 96.8% for MM6, 93.1% to 97.9% for 3D OCT-1000 radial, and 31.5% to 97.5% for 3D macular scans. Incidence of artifacts was higher in spectral/Fourier domain instruments, ranging from 28.75% to 53.16%, compared with 17.46% in Stratus OCT. No significant age or sex trends were found in the measurements. CONCLUSION: Commercial spectral/Fourier domain OCT instruments provide higher speed and axial resolution than the Stratus OCT, although they vary greatly in scanning protocols and are currently limited in their analysis functions. Further development of segmentation algorithms and quantitative features are needed to assist clinicians in objective use of these newer instruments to manage diseases.

Assessment of artifacts and reproducibility across spectral- and time-domain optical coherence tomography devices.

PURPOSE: To report the frequency of optical coherence tomography (OCT) scan artifacts and to compare macular thickness measurements, interscan reproducibility, and interdevice agreeability across 3 spectral-domain (SD) OCT (also known as Fourier domain; Cirrus HD-OCT, RTVue-100, and Topcon 3D-OCT 1000) devices and 1 time-domain (TD) OCT (Stratus OCT) device. DESIGN: Prospective, noncomparative, noninterventional case series. PARTICIPANTS: Fifty-two patients seen at the New England Eye Center, Tufts Medical Center Retina Service, between February and August 2008. METHODS: Two scans were performed for each of the SD OCT protocols: Cirrus macular cube 512 x 128 (software version 3.0; Carl Zeiss Meditec, Inc., Dublin, CA), RTVue (E)MM5 and MM6 (software version 3.5; Optovue, Inc., Fremont, CA), Topcon 3D Macular and Radial (software version 2.12; Topcon, Inc., Paramus, NJ), in addition to 1 TD OCT scan via Stratus macular thickness protocol (software version 4.0; Carl Zeiss Meditec, Inc.). Scans were inspected for 6 types of OCT scan artifacts and were analyzed. Interscan reproducibility and interdevice agreeability were assessed by intraclass correlation coefficients (ICCs) and Bland-Altman plots, respectively. MAIN OUTCOME MEASURES: Optical coherence tomography image artifacts, macular thickness, reproducibility, and agreeability. RESULTS: Time-domain OCT scans contained a significantly higher percentage of clinically significant improper central foveal thickness (IFT) after manual correction (11-mum change or more) compared with SD OCT scans. Cirrus HD-OCT had a significantly lower percentage of clinically significant IFT (11.1%) compared with the other SD OCT devices (Topcon 3D, 20.4%; Topcon Radial, 29.6%; RTVue (E)MM5, 42.6%; RTVue MM6, 24.1%; P = 0.001). All 3 SD OCT devices had central foveal subfield thicknesses that were significantly more than that of TD OCT after manual correction (P<0.0001). All 3 SD OCT devices demonstrated a high degree of reproducibility in the central foveal region (ICCs, 0.92-0.97). Bland-Altman plots showed low agreeability between TD and SD OCT scans. CONCLUSIONS: Out of all OCT devices analyzed, cirrus HD-OCT scans exhibited the lowest occurrence of any artifacts (68.5%), IFT (40.7%), and clinically significant IFT (11.1%), whereas Stratus OCT scans exhibited the highest occurrence of clinically significant IFT. Further work on improving segmentation algorithm to decrease artifacts is warranted.

High-speed ultrahigh resolution optical coherence tomography before and after ranibizumab for age-related macular degeneration.

OBJECTIVE: To evaluate intraretinal anatomy in patients with exudative age-related macular degeneration (AMD) using high-speed ultrahigh resolution optical coherence tomography (hsUHR-OCT) before and 1 month after intravitreal injection of ranibizumab. DESIGN: Retrospective case series. PARTICIPANTS: Twelve eyes of 12 patients. METHODS: A broad bandwidth superluminescent diode laser light source and spectral/Fourier domain signal detection were used to create a prototype hsUHR-OCT instrument with 3.5 mum axial image resolution and approximately 25,000 lines/second acquisition speed. Twelve eyes of 12 patients with exudative AMD were imaged with hsUHR-OCT before and 1 month after intravitreal ranibizumab injection. High pixel density and raster-scanned 3-dimensional (3D) OCT data sets were generated. Three-dimensional imaging software was used to calculate subretinal/retinal pigment epithelium fluid volume and volume of the fibrovascular lesion. MAIN OUTCOME MEASURES: Qualitative and quantitative analysis of hsUHR-OCT images and 3D data sets. RESULTS: All eyes had some degree of normalization of macular contour after intravitreal ranibizumab. The inner/outer photoreceptor segment junction visualized on hsUHR-OCT was discontinuous, overlying the fibrovascular lesion in all 12 of 12 eyes both before and after treatment; 9 of 12 eyes had focal areas of thinning of the outer nuclear layer, which remained after treatment. Volumetric measurements were possible in 8 of 12 eyes with 3D-rendering software. Fibrovascular lesion volume did not change significantly after treatment. CONCLUSIONS: hsUHR-OCT is capable of unprecedented imaging speed and resolution, making it a valuable instrument in measuring in vivo intraretinal pathology. All 12 eyes had some normalization of macular contour. Fibrovascular lesion volume did not change significantly 1 month after treatment, suggesting that ranibizumab does not cause much initial regression of preexisting neovascular tissue. Photoreceptor abnormalities remained in all patients after treatment of wet AMD, suggesting that although ranibizumab improves overall retinal architecture, some photoreceptor damage may be irreversible. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.