Early Detection of Optic Nerve Changes on Optical Coherence Tomography Using Deep Learning for Risk-Stratification of Papilledema and Glaucoma.

BACKGROUND: The use of artificial intelligence is becoming more prevalence in medicine with numerous successful examples in ophthalmology. However, much of the work has been focused on replicating the works of ophthalmologists. Given the analytical potentials of artificial intelligence, it is plausible that artificial intelligence can detect microfeatures not readily distinguished by humans. In this study, we tested the potential for artificial intelligence to detect early optic coherence tomography changes to predict progression toward papilledema or glaucoma when no significant changes are detected on optical coherence tomography by clinicians. METHODS: Prediagnostic optical coherence tomography of patients who developed papilledema (n = 93, eyes = 166) and glaucoma (n = 187, eyes = 327) were collected. Given discrepancy in average cup-to-disc ratios of the experimental groups, control groups for papilledema (n = 254, eyes = 379) and glaucoma (n = 441, eyes = 739) are matched by cup-to-disc ratio. Publicly available Visual Geometry Group-19 model is retrained using each experimental group and its respective control group to predict progression to papilledema or glaucoma. Images used for training include retinal nerve fiber layer thickness map, extracted vertical tomogram, ganglion cell thickness map, and ILM-RPE thickness map. RESULTS: Trained model was able to predict progression to papilledema with a precision of 0.714 and a recall of 0.769 when trained with retinal nerve fiber layer thickness map, but not other image types. However, trained model was able to predict progression to glaucoma with a precision of 0.682 and recall of 0.857 when trained with extracted vertical tomogram, but not other image types. Area under precision-recall curve of 0.826 and 0.785 were achieved for papilledema and glaucoma models, respectively. CONCLUSIONS: Computational and analytical power of computers have become an invaluable part of our lives and research endeavors. Our proof-of-concept study showed that artificial intelligence (AI) algorithms have the potential to detect early changes on optical coherence tomography for prediction of progression that is not readily observed by clinicians. Further research may help establish possible AI models that can assist with early diagnosis or risk stratification in ophthalmology.

Guidelines of the International Headache Society for Controlled Clinical Trials in Idiopathic Intracranial Hypertension.

The quality of clinical trials is essential to advance treatment, inform regulatory decisions and meta-analysis. With the increased incidence of idiopathic intracranial hypertension and the emergence of clinical trials for novel therapies in this condition, the International Headache Society Guidelines for Controlled Clinical Trials in Idiopathic Intracranial Hypertension aims to establish guidelines for designing state-of-the-art controlled clinical trials for idiopathic intracranial hypertension.

Idiopathic Intracranial Venous Hypertension: Toward a Better Understanding of Venous Stenosis and the Role of Stenting in Idiopathic Intracranial Hypertension.

BACKGROUND: Venous sinus stenosis, typically at the junction of the transverse and sigmoid sinus, is increasingly recognized as a contributor to the pathophysiology of idiopathic intracranial hypertension (IIH), whether it be the intrinsic type that does not reverse with normalization of intracranial pressure or the extrinsic type, which does. Efforts to treat the stenosis and reduce the associated transstenotic gradient through placement of a stent at the site of stenosis have been studied over the past 2 decades, primarily through retrospective studies, with variable emphasis on formal visual testing and direct assessment of poststent opening pressure. Most studies have presented evidence for utilization of stenting as an alternative to cerebrospinal fluid shunting or optic nerve sheath fenestration in patients with IIH who harbor the stenosis and are refractory to or intolerant of intracranial pressure-lowering medications, but an assessment of the current data is needed to better understand the role of stenting for this patient population. EVIDENCE ACQUISITION: A search in PubMed was made for "IIH," "papilledema," and "venous stenting." Data pre and post stenting, including symptoms attributable to IIH, intracranial pressure, papilledema, retinal nerve fiber layer thickening on optical coherence tomography, and visual field assessment (mean deviation), were collected. Need for retreatment and complications were assessed among all studies. Studies using stenting for special circumstances, such as cerebrospinal leaks or for stenosis along anomalous vessels, were reviewed. RESULTS: In total, 49 studies (45 retrospective and 4 prospective) and 18 case reports (with 3 or less patients) were found and included in the analysis, for a total of 1,626 patients. In 250 patients in whom poststent intracranial pressure was measured, the mean value was 19.7 cm H 2 O, reduced from a mean of 33 cm H 2 O. Transient visual obscurations resolved in 79.6% of 201 patients who complained of it, pulsatile tinnitus resolved in 84.7% of 515, diplopia resolved in 93% of 86 patients, and nonspecific visual symptoms such as "blurry vision" improved in 76.2% of 537 patients. Headaches resolved in 36% and improved in a further 40.7% of 1,105 patients in whom they were documented before stenting. Of 1,116 with papilledema, 40.8% demonstrated resolution and 38.2% improvement. The mean retinal nerve fiber layer thickness improved from 170.2 µm to 89.2 µm among 402 eyes in which optical coherence tomography was used to measure it. Among 135 eyes in which formal visual fields were performed pre and post stenting, the prestent average mean deviation of -7.35 dB improved to -4.72 dB after stenting. Complications associated with stenting included in-stent stenosis or thrombosis, subdural hematoma, intracerebral hematoma, cerebral edema, stent migration, and death. A recurrence of symptoms requiring a follow-up surgical intervention occurred in 9%. CONCLUSIONS: A growing body of evidence supports the use of venous sinus stenting as a viable option for medically refractory IIH, especially when papilledema threatens visual function. Complication and failure rates seem to be similar to alternative surgical approaches, although serious neurological sequalae can rarely occur. Emerging studies evaluating stent type, including novel stents designed for use in the venous system, may help improve ease of the procedure and long-term success rates. Prospective head-to-head studies are needed to better understand the performance of stenting compared with other interventions.

Ophthalmic Virtual Visit Utilization and Patient Satisfaction During the COVID-19 Pandemic.

Background:Due to the COVID-19 pandemic, there was a surge in synchronous ophthalmic telehealth visits. The purpose of this study is to analyze the utilization and patient satisfaction of synchronous ophthalmic video visits over the course of the COVID-19 pandemic.Methods:In this retrospective, single-center cross-sectional study, 1,756 patients seen through synchronous video visits between March 1, 2020, and March 31, 2021, were identified using billing codes. E-mails containing a validated, 11-item, telehealth satisfaction scale were sent to patients who had at least one video visit within the study period. Questions were scored on a 1-4 scale, corresponding to poor, fair, good, and excellent. Main outcome measures included patient satisfaction scores, frequency of repeat video visits, and primary visit diagnoses.Results:The top 3 subspecialties by virtual visit volume were oculoplastic surgery (999 visits, 42.9%), neuro-ophthalmology (331 visits, 17.0%), and cornea (254 visits, 14.2%). The top 3 diagnoses seen were chalazion/hordeolum, dry eye, and meibomian gland dysfunction. The overall survey response rate was 14.3% (252 participants). The mean patient satisfaction score was 3.67 ± 0.63, with no significant difference in scores between specialties. A total of 380 (21%) patients had repeat virtual visits. Mean survey response scores were significantly higher for patients with repeat visits than those without (3.82 ± 0.42 vs. 3.62 ± 0.68, p = 0.03). Patients undergoing oculoplastic services were more likely to have repeat visits (odds ratio 2.58, 95% confidence interval 2.18-3.06, p < 0.001). Multivariate regression analysis found that provider thoroughness/skillfulness was the most predictive feature of the patient returning to a telehealth encounter (p = 0.01).Conclusions:Our study suggests that synchronous videoconferencing for ophthalmology is a highly satisfactory delivery method and will likely find continued success in select subspecialties as the pandemic fades.

An elusive case of malignant optic nerve glioma with a suprasellar mass.

A 67-year-old man with multiple comorbidities presented with 3 weeks of orbital pain, proptosis, and no light perception vision in the left eye. Examination was notable for a left orbital apex syndrome with CN III, VI palsies, an optic neuropathy, and central retinal vein occlusion. Magnetic resonance imaging of the orbits was notable for extensive enhancement, enlargement, and T2 hyperintensity of the optic nerve, with perineural sheath enhancement, and chiasmal hyperintensity. Inflammatory workup and lumbar puncture were unremarkable. No improvement was seen after a 3-day course of intravenous solumedrol. Initial optic nerve biopsy revealed necrotic nerve tissue, macrophage infiltration, increased vascularization, and peripheral gliosis. The volume of tissue was inadequate for genomic analysis. The patient was lost to follow-up but returned 5 months later with right-sided vision loss. Repeat neuroimaging showed a new suprasellar mass and progressive expansion and enhancement of both optic nerves. Biopsies of the suprasellar mass and left nerve at this time were consistent with a high-grade glial neoplasm, WHO grade IV. This is a rare case of glioblastoma involving the optic nerves and suprasellar region. In such cases, molecular profiling can improve diagnosis and may provide for targeted treatments in the future.

Telemedicine Evaluations in Neuro-Ophthalmology During the COVID-19 Pandemic: Patient and Physician Surveys.

BACKGROUND: The novel coronavirus 2019 (COVID-19) pandemic has transformed health care. With the need to limit COVID-19 exposures, telemedicine has become an increasingly important format for clinical care. Compared with other fields, neuro-ophthalmology faces unique challenges, given its dependence on physical examination signs that are difficult to elicit outside the office setting. As such, it is imperative to understand both patient and provider experiences to continue to adapt the technology and tailor its application. The purpose of this study is to analyze both neuro-ophthalmology physician and patient satisfaction with virtual health visits during the time of the COVID-19 pandemic. METHODS: Across three institutions (NYU Langone Health, Indiana University Health, and Columbia University Medical Center), telemedicine surveys were administered to 159 patients. Neuro-ophthalmologists completed 157 surveys; each of these were linked to a single patient visit. Patient surveys consisted of 5 questions regarding visit preparation, satisfaction, challenges, and comfort. The physician survey included 4 questions that focused on ability to gather specific clinical information by history and examination. RESULTS: Among 159 patients, 104 (65.4%) reported that they were satisfied with the visit, and 149 (93.7%) indicated that they were comfortable asking questions. Sixty-eight (73.9%) patients found the instructions provided before the visit easy to understand. Potential areas for improvement noted by patients included more detailed preparation instructions and better technology (phone positioning, Internet connection, and software). More than 87% (137/157) of neuro-ophthalmologists surveyed reported having performed an examination that provided enough information for medical decision-making. Some areas of the neuro-ophthalmologic examination were reported to be easy to conduct (range of eye movements, visual acuity, Amsler grids, Ishihara color plates, and pupillary examination). Other components were more difficult (saccades, red desaturation, visual fields, convergence, oscillations, ocular alignment, and smooth pursuit); some were especially challenging (vestibulo-ocular reflex [VOR], VOR suppression, and optokinetic nystagmus). Clinicians noted that virtual health visits were limited by patient preparation, inability to perform certain parts of the examination (funduscopy and pupils), and technological issues. CONCLUSIONS: Among virtual neuro-ophthalmology visits evaluated, most offer patients with appointments that satisfy their needs. Most physicians in this cohort obtained adequate clinical information for decision-making. Even better technology and instructions may help improve aspects of virtual health visits.

Increased frequency of cataract surgery in patients over age 50 with pituitary macroadenomas and chiasmal compression.

BACKGROUND: Patients with visual loss from macroadenomas compressing their optic apparatus may also have concomitant age-related visual pathology such as cataracts. How these two pathologies interact with each other is not well documented. OBJECTIVE: The interaction between these two pathologies in elderly patients is the subject of this study. METHODS: We identified a series of non-functioning macroadenoma patients over age 50 years with tumors compressing the chiasm who underwent transsphenoidal surgery at our institution between 2004 and 2018. Pre- and post-operative visual complaints, tumor size and extent of resection were analyzed. Prevalence of the diagnosis of cataract and prevalence of cataract surgery in each decade were compared with national averages. RESULTS: We identified 200 patients who met selection criteria. 18% of these patients had a diagnosis of cataract and 12.5% had cataract surgery. Compared with the Eye Diseases Prevalence Research Group (EDPRG) study, the prevalence of cataract surgery was 2.5 times the national average of 5.1%. 32% of these patients had no improvement in their vision after cataract surgery but 76% improved after transsphenoidal surgery. CONCLUSIONS: We reported a high prevalence of cataract surgery in patients over age 50 in patients with pituitary macroadenomas compressing the optic pathway compared with national averages in patients without adenomas. While visual loss from adenoma likely precipitated more cataract surgeries in this group of patients, some who may not have required it, those patients with cataracts who did not have their cataracts extracted were less likely to recover vision after transsphenoidal surgery. Addressing both pathologies is beneficial.

Venous Sinus Stenting in Idiopathic Intracranial Hypertension: Results of a Prospective Trial.

BACKGROUND: Our goal was to evaluate the safety and efficacy of stenting of venous sinus stenosis (VSS) in patients with medically-refractory, medically-intolerant or fulminant idiopathic intracranial hypertension (IIH) in a prospective, observational study. METHODS: Thirteen patients with IIH who were refractory or intolerant to medical therapy or who presented with fulminant visual field (VF) loss underwent stenting of VSS at the transverse-sinus sigmoid sinus junction, using a Precise Pro carotid stent system (Cordis). Inclusion criteria included papilledema-related VF loss with mean deviation (MD) worse than or equal to -6.00 dB, elevated opening pressure (OP) on lumbar puncture (LP), VSS (either bilateral or unilateral in a dominant sinus), and an elevated (≥8 mm Hg) trans-stenotic gradient (TSG). The main outcome measures were pre- to post-stent change in symptoms related to intracranial hypertension, MD (in dB) on automated (Humphrey) VFs, grade of papilledema (1-5), retinal nerve fiber layer (RNFL) thickness as measured by spectral domain optical coherence tomography (SD-OCT), TSG (mm Hg), and OP on LP (cm H20). RESULTS: Improvement or resolution of headaches occurred in 84.7% of patients, pulse-synchronous tinnitus in 100%, diplopia in 100%, and transient visual obscuration in 100%. Out of 26 eyes, 21 showed an improvement in MD, with an average improvement of +5.40 dB. Of 24 eyes with initial papilledema, 20 showed an improvement in Frisen grade, (mean change in grade of 1.90). Of 23 eyes undergoing SD-OCT, 21 (91.3%) demonstrated a reduction in RNFL thickness, with a poststent mean thickness of 90.48 µm. Mean change in OP was -20 cm H2O (reduction in mean from 42 to 22 cm H20) with all subjects demonstrating a reduction, although a second stenting procedure was necessary in one patient. Complications of the stenting procedure included one small, self-limited retroperitoneal hemorrhage, transient head or pelvic pain, and one allergic reaction to contrast. No serious adverse events occurred. CONCLUSIONS: Stenting of VSS is safe and results in reduction of intracranial pressure in patients with IIH. This is associated with improvement in papilledema, RNFL thickness, VF parameters, and symptoms associated with intracranial hypertension.

Ocular Myasthenia Gravis Associated With Thymic Amyloidosis.

A 45-year-old woman with ptosis and diplopia was found to have myasthenia gravis (MG) associated with amyloidosis of the thymus gland. Systemic MG is frequently associated with thymomas or thymic hyperplasia but has only once previously been reported in association with amyloidosis of the thymus. This case demonstrates that isolated ocular MG rarely may also be associated with amyloidosis of the thymus.

Corresponding Ganglion Cell Atrophy in Patients With Postgeniculate Homonymous Visual Field Loss.

BACKGROUND: The goal of our study was to look for the presence of homonymous ganglion cell layer-inner plexiform layer complex (GCL-IPL) thinning using spectral-domain optical coherence tomography (SD-OCT) in patients with a history of adult-onset injury to the postgeniculate pathways with rigorous radiological exclusion of geniculate and pregeniculate pathology. METHODS: We performed a retrospective review of twenty-two patients (ages 24-75 y, 6 men, 16 women) with homonymous visual field (VF) defects secondary to postgeniculate injury examining the GCL-IPL with SD-OCT. An additional fifteen patients (ages 28-85 y, 5 men, 10 women) with no visual pathway pathology served as controls. Using segmentation analysis software applied to the macular scan, a normalized asymmetry score was calculated for each eye comparing GCL-IPL thickness ipsilateral vs contralateral to the patient's brain lesions. RESULTS: We found that 15 of the twenty-two subjects had a relative thinning of the GCL-IPL ipsilateral to the postgeniculate lesion in both eyes (represented by a positive normalized asymmetry score in both eyes), whereas a similar pattern of right/left asymmetry was found in 4 controls (P = 0.0498). The magnitude of asymmetry was much greater in subjects compared with controls (P = 0.0004). There was no association between the degree of GCL-IPL thinning and the mean deviation on automated VF testing. A moderate correlation (R = 0.782, P = 0.004) between the magnitude of thinning and latency from onset of retrogeniculate injury was observed only after excluding patients beyond a cutoff point of 150 months. CONCLUSIONS: This data provides compelling new evidence of retrograde transsynaptic degeneration causing retinal ganglion cell loss after postgeniculate visual pathway injury.

Multilayered macular hemorrhages as an unusual complication of transorbital neuroendoscopic surgery.

Purpose: To report a case of multilayered intraocular hemorrhage at the posterior pole as a complication of transorbital neuroendoscopic surgery. Observations: Our patient underwent an uncomplicated endoscopic transorbital resection of a left sphenoid wing meningioma. In the immediate post-operative period, the patient reported blurred vision of her left eye, and dilated fundus examination demonstrated multilayered hemorrhages at the posterior pole. No intracranial hemorrhage was identified on post-operative imaging. Due to persistent subnormal visual acuity and non-clearing hemorrhage over several weeks of follow-up, a pars plana vitrectomy with peeling of the internal limiting membrane was performed to clear the hemorrhagic component obscuring the macula. Conclusions and Importance: We report the first case of multilayered intraocular hemorrhages at the posterior pole, mimicking Terson syndrome, in the absence of intracranial hemorrhage or elevated intracranial pressure as a complication of transorbital surgery.

Unexpected late radiation neurotoxicity following bevacizumab use: a case series.

The purpose of this case series is to report the unexpected occurrence of four cases of late radiation-induced neurotoxicity with bevacizumab use following radiotherapy to the CNS. We retrospectively reviewed the case records of four patients, three with glioblastoma and one with bone metastases secondary to metastatic breast cancer, who were treated with radiotherapy and developed late radiation-induced neurotoxicity following bevacizumab use. Three cases of optic neuropathy in glioblastoma patients and a single case of Brown-Séquard syndrome in the thoracic spine of a patient with metastatic breast cancer are reported. We hypothesize that bevacizumab use following radiotherapy to the CNS may inhibit vascular endothelial growth factor-dependent repair of normal neural tissue, and thus may increase the risk of late radiation neurotoxicity. Phase III data on the safety and efficacy of bevacizumab use with radiation in the setting of glioblastoma is awaited.

Traumatic ptosis and mydriasis masking Horner syndrome from an internal carotid pseudoaneurysm.

A 46-year-old woman presented after blunt facial trauma for evaluation of bilateral orbital floor fractures, which were confirmed by clinical and radiographic examination. In addition, findings of right traumatic mydriasis and left traumatic ptosis were seen. On close investigation, miosis was noted on the side of the traumatic ptosis. Therefore, a workup for Horner syndrome was obtained, including neuro-ophthalmic consultation and magnetic resonance angiography of the neck. The magnetic resonance angiography showed an internal carotid pseudoaneurysm, an uncommon but potentially fatal complication of blunt carotid injury. The authors describe an interesting case where a life-threatening diagnosis was nearly masked by more common, benign pathology.