Ocular Ischemic Syndrome and the Role of Carotid Artery Revascularization.

BACKGROUND: Ocular ischemic syndrome (OIS) is a rare presentation of atherosclerotic carotid artery stenosis that can result in permanent visual loss. This severely disabling syndrome remains under diagnosed and undertreated due to lack of awareness; especially since it requires expedited multidisciplinary care. The relevance of early diagnosis and treatment is increasing due to an increasing prevalence of cerebrovascular disease. METHODS: The long-term visual and cerebrovascular outcomes following intervention for nonarteritic OIS, remain poorly described and were the objective of this concise review. We conducted a PubMed search to include all English language publications (cohort studies and case reports) between 2002 and 2023. RESULTS: A total of 33 studies (479 patients) report the outcomes of treatment of OIS with carotid endarterectomy (CEA, 304 patients, 19 studies), and carotid artery stenting (CAS, 175 patients, 14 studies). Visual outcomes were improved or did not worsen in 447 patients (93.3%). No periprocedural stroke was reported. Worsening visual symptoms were rare (35 patients, 7.3%); they occurred in the immediate postoperative period secondary to ocular hypoperfusion (3 patients) and in the late postoperative period due to progression of systemic atherosclerotic disease. Symptomatic recurrence due to recurrent stenosis after CEA was reported in 1 patient (0.21%); this was managed successfully with CAS. None of these studies report the results of transcarotid artery revascularization, the long-term operative outcome or stroke rate. CONCLUSIONS: OIS remains to be an underdiagnosed condition. Early diagnosis and prompt treatment are crucial in reversal or stabilization of OIS symptoms. An expedited multidisciplinary approach between vascular surgery and ophthalmology services is necessary to facilitate timely treatment and optimize outcome. If diagnosed early, both CEA and CAS have been associated with visual improvement and prevention of progressive visual loss.

A Comparative Study of Large Language Models, Human Experts, and Expert-Edited Large Language Models to Neuro-Ophthalmology Questions.

BACKGROUND: While large language models (LLMs) are increasingly used in medicine, their effectiveness compared with human experts remains unclear. This study evaluates the quality and empathy of Expert + AI, human experts, and LLM responses in neuro-ophthalmology. METHODS: This randomized, masked, multicenter cross-sectional study was conducted from June to July 2023. We randomly assigned 21 neuro-ophthalmology questions to 13 experts. Each expert provided an answer and then edited a ChatGPT-4-generated response, timing both tasks. In addition, 5 LLMs (ChatGPT-3.5, ChatGPT-4, Claude 2, Bing, Bard) generated responses. Anonymized and randomized responses from Expert + AI, human experts, and LLMs were evaluated by the remaining 12 experts. The main outcome was the mean score for quality and empathy, rated on a 1-5 scale. RESULTS: Significant differences existed between response types for both quality and empathy (P < 0.0001, P < 0.0001). For quality, Expert + AI (4.16 ± 0.81) performed the best, followed by GPT-4 (4.04 ± 0.92), GPT-3.5 (3.99 ± 0.87), Claude (3.6 ± 1.09), Expert (3.56 ± 1.01), Bard (3.5 ± 1.15), and Bing (3.04 ± 1.12). For empathy, Expert + AI (3.63 ± 0.87) had the highest score, followed by GPT-4 (3.6 ± 0.88), Bard (3.54 ± 0.89), GPT-3.5 (3.5 ± 0.83), Bing (3.27 ± 1.03), Expert (3.26 ± 1.08), and Claude (3.11 ± 0.78). For quality (P < 0.0001) and empathy (P = 0.002), Expert + AI performed better than Expert. Time taken for expert-created and expert-edited LLM responses was similar (P = 0.75). CONCLUSIONS: Expert-edited LLM responses had the highest expert-determined ratings of quality and empathy warranting further exploration of their potential benefits in clinical settings.

Transient Bilateral Central Scotoma on Awakening: A Distinct Phenomenon.

BACKGROUND: Transient vision loss can be an alarming symptom owing to potentially serious etiologies such as thromboembolism or giant cell arteritis. Transient monocular vision loss (TVML) on awakening has been recently described as a benign phenomenon (Bouffard et al, 2017). Our objective was to describe the unique phenomenon of transient binocular vision loss (TBVL) on awakening. METHODS: Retrospective observational case series of 5 patients who experienced TBVL on awakening seen in the neuro-ophthalmology clinic at the Mayo Clinic between 2016 and 2020. Patients who described generalized blurred vision and those with monocular transient vision loss were excluded. RESULTS: The median age was 81.4 years (range, 68-92 years), and all were male. Every patient described a stereotyped transient bilateral central scotoma on awakening with slightly irregular borders and mild asymmetry between the 2 eyes lasting between 15 and 90 minutes. Frequency ranged from 3 to 7 times per week, and there was a median of 319 episodes (range, 126-728 episodes) before evaluation in the neuro-ophthalmology clinic. All patients had normal optic nerves, and no plaques were noted in the retinal vessels. All 5 had macular drusen, which were predominantly extrafoveal and mild. Two patients underwent electrophysiology testing, which were both normal on full-field electroretinogram (ERG), but there was blunted central wave forms on multifocal ERG. Two patients underwent dark adaptation testing, which showed both prolong and diminished dark adaptation. Neuroimaging and thromboembolic workup were unrevealing. CONCLUSIONS: TBVL is a distinct phenomenon from TMVL on awakening, which has a different demographic and symptomology. The etiology is unclear but seems to be a focal macular process in conjunction with an autoregulatory failure resulting in a supply-demand mismatch during low-light conditions.

The 'Fault' Lies in the Choroid: Peripapillary Intrachoroidal Cavitation Presenting with Progressive Vision Loss.

A 55-year-old male was referred to the Neuro-ophthalmology clinic due to gradual onset, progressive vision loss. On fundus examination a subtle yellow-orange peripapillary lesion was detected in the left eye. Optical coherence tomography with radial scanning illustrated retinal nerve fibre layer thinning as well as an area of intrachoroidal cavitation that corresponded to the lesion. Visual field testing showed a left inferior arcuate defect. Magnetic resonance imaging of the brain and orbit, and laboratory testing was unremarkable. Clinical examination, imaging, and testing were consistent with peripapillary intrachoroidal cavitation (PICC). Follow-up with serial visual field testing showed mild progression of the field defect. While PICC is not well understood in the literature, studies have reported associated risk factors including pathological myopia, older age, increased ocular axial length, chorioretinal atrophy, and vascular abnormalities. Importantly, glaucoma-like visual field defects as well as structural changes have been noticed in a high proportion of patients with PICC. While these alterations are evident, the pathogenic relationship between them is yet to be uncovered. Treatment with anti-glaucoma medications has been suggested, however, the evidence remains scarce for its true benefits. Care providers must be aware of the presentation of a yellow-orange peripapillary lesion with an associated visual field defect to accurately diagnose and manage this condition.

Idiopathic Intracranial Hypertension Triggering Hemifacial Spasm.

ABSTRACT: Idiopathic intracranial hypertension (IIH) is a syndrome associated with increased intracranial pressure without a clear underlying cause that is classically seen in young women. Patients typically present with headache and ocular findings, including disc edema and, less frequently, an abduction deficit. To make a diagnosis of IIH, other than cranial nerve 6 or 7 dysfunction, patients must have a normal neurologic examination. When cranial nerve 7 is affected patients can present with hemifacial spasm. We present the case of a young woman with IIH who had hemifacial spasm as one of the presenting symptoms. Her symptoms resolved once she was treated for IIH with acetazolamide.

Abnormal Vestibular-Ocular Reflexes in Children With Cortical Visual Impairment.

BACKGROUND: To determine whether the vestibular-ocular reflexes (VORs) can be affected by central nervous system injury in children with cortical visual impairment (CVI). METHODS: Retrospective case series. Twenty consecutive children with CVI who presented to a pediatric ophthalmology practice over an 18-month period were included in the study. Horizontal and vertical VORs were assessed by a pediatric neuro-ophthalmologist using the standard doll's head maneuver. MRI studies were independently reviewed by a pediatric neuroradiologist in a masked fashion. The main outcome measures were the integrity of the VORs and the presence of brainstem abnormalities on MRI. RESULTS: VORs were found to be absent or severely impaired in 13/20 (65%) children with CVI. More surprisingly, the doll's head maneuver failed to substantially overcome the deviated eye position in 8/13 (62%) children with conjugate gaze deviations. Reduced brainstem size and signal abnormalities were found in 4/7 children with normal VORs and in 9/13 children with abnormal VORs (P = 0.6), showing noncorrelation with the integrity of the VOR. CONCLUSION: VORs are commonly impaired in children with CVI. This ocular motor deficit reflects the diffuse cortical and subcortical injury that often accompanies perinatal injury to the developing brain. Consequently, these children may lack important visual compensatory mechanisms to stabilize gaze during head movements. This knowledge can help in planning visual rehabilitation.

Orbital Cellulitis-When Computed Tomography Imaging Is Falsely Reassuring.

ABSTRACT: An 8-year-old male presented to the pediatric emergency department with subjective fever, blurry vision, and left eye pain. On physical examination, there was mild left lower lid erythema and a subtle left eye elevation deficit. Computed tomography demonstrated preseptal soft tissue swelling without orbital involvement; however, there was varying amounts of paranasal sinus opacification. Despite the negative scan, a diagnosis of orbital cellulitis associated with sinusitis was made and treatment with intravenous antibiotics was initiated. A magnetic resonance imaging performed subsequently revealed orbital fat stranding and inferior rectus enlargement and enhancement near the orbital apex, indicating the presence of orbital cellulitis. This case demonstrates the utility of magnetic resonance imaging when initial computed tomography imaging is negative in patients with concern for orbital cellulitis.

Magnetic Resonance Imaging Findings and Genetic Testing Results in Children With Congenital Corneal Opacities.

PURPOSE: This study investigates brain and globe abnormalities identified on magnetic resonance imaging (MRI) in children with congenital corneal opacities (CCO). DESIGN: Retrospective cohort study. METHODS: Clinical notes, radiology records, and genetic testing results were reviewed for patients diagnosed with corneal opacification within the first 6 months of life at a tertiary referral academic center between August 2008 and January 2018. Ocular findings, systemic anomalies, neuroimaging, and genetic testing results were summarized. RESULTS: A total of 135 patients presenting at age 1 day to 12 years (mean age, 1 year) were identified. Children with bilateral CCO were more likely to have systemic disease (P = 0.018). Of the entire cohort, 43 (31.8%) patients received MRI, of whom 27 (62.8%) had abnormal brain findings and 30 (69.7%) had abnormal orbital findings. The most common abnormal brain findings were ventriculomegaly (n = 16, 59.2%) and corpus callosum abnormalities (n = 10, 37.0%) followed by brainstem/pons anomalies (n = 5, 18.5%), and cerebellar anomalies (n = 2, 7.4%). Abnormal brain MRI findings were associated with the presence of neurologic (P = .003) and craniofacial (P = .034) disease. A total of 44 (32.1%) patients underwent genetic testing, of whom 29 (65.9%) had pathogenic results. CONCLUSIONS: More than 60% of the children with CCO who underwent MRI had abnormal brain and orbit findings that were correlated with significant neurologic disease. Furthermore, almost two-thirds of patients with CCO who underwent genetic testing had pathogenic results. These data demonstrate the value of systemic workup in children with CCO, and highlight the role of ophthalmologists in facilitating the diagnosis of systemic comorbidities associated with CCO.

Visual acuity and optic nerve size assessed by magnetic resonance imaging in optic nerve hypoplasia.

We investigated the relationship between optic nerve (ON) size and visual acuity in children with optic nerve hypoplasia (ONH). The medical records of patients <19 years with ONH who underwent brain magnetic resonance imaging (MRI) and visual acuity assessment were reviewed. ON diameter at orbital and cisternal segments was assessed independently by two neuroradiologists and compared with visual acuity. ON diameter <1.7 mm represented a cutoff, below which was significantly associated with visual acuity of 20/200 or worse (P = 0.04) and above which was significantly associated with visual acuity of 20/40 or better (P = 0.004). ON diameter measured with MRI may provide an early prognostic indication of visual potential for children with ONH.

Ocular Neuromyotonia: Clinical Features, Diagnosis, and Outcomes.

PURPOSE: The purpose of this study was to describe the clinical features, management, outcomes, and diagnostic pitfalls in a large series of patients with ocular neuromyotonia. DESIGN: Retrospective cohort. METHODS: Patients diagnosed with ocular neuromyotonia from January 1, 2004, through January 1, 2023, seen at one of the 3 Mayo Clinic sites in Rochester, MN, Scottsdale, AZ, and Jacksonville, FL, comprised the study population. We ascertained patients with ocular neuromyotonia through a search using the medical records database. Only patients with an observed episode of ocular neuromyotonia were included and the medical records were reviewed. The main outcome measures were clinical features and outcomes of patients with ocular neuromyotonia. RESULTS: Forty-two patients who were diagnosed with ocular neuromyotonia were included. The median age was 58 years (range, 16-80 years). A history of cranial radiation therapy was present in 39 patients (93%). The sixth cranial nerve was involved in 31 patients (74%). Bilateral disease was found in 2 patients (5%). The median time from onset of diplopia to diagnosis was 8 months (range, 1 month-25 years), with a high rate of initial misdiagnosis in 52%. Twenty of 42 patients (48%) were treated with oral medication, of whom 95% had significant improvement or resolution of symptoms. CONCLUSION: Prior cranial irradiation is the most common cause for ocular neuromyotonia, affecting the sixth cranial nerve most often. Although delayed and initial misdiagnosis is common, most patients show improved symptoms on medical treatment.

Appropriateness of Ophthalmology Recommendations From an Online Chat-Based Artificial Intelligence Model.

Objective: To determine the appropriateness of ophthalmology recommendations from an online chat-based artificial intelligence model to ophthalmology questions. Patients and Methods: Cross-sectional qualitative study from April 1, 2023, to April 30, 2023. A total of 192 questions were generated spanning all ophthalmic subspecialties. Each question was posed to a large language model (LLM) 3 times. The responses were graded by appropriate subspecialists as appropriate, inappropriate, or unreliable in 2 grading contexts. The first grading context was if the information was presented on a patient information site. The second was an LLM-generated draft response to patient queries sent by the electronic medical record (EMR). Appropriate was defined as accurate and specific enough to serve as a surrogate for physician-approved information. Main outcome measure was percentage of appropriate responses per subspecialty. Results: For patient information site-related questions, the LLM provided an overall average of 79% appropriate responses. Variable rates of average appropriateness were observed across ophthalmic subspecialties for patient information site information ranging from 56% to 100%: cataract or refractive (92%), cornea (56%), glaucoma (72%), neuro-ophthalmology (67%), oculoplastic or orbital surgery (80%), ocular oncology (100%), pediatrics (89%), vitreoretinal diseases (86%), and uveitis (65%). For draft responses to patient questions via EMR, the LLM provided an overall average of 74% appropriate responses and varied by subspecialty: cataract or refractive (85%), cornea (54%), glaucoma (77%), neuro-ophthalmology (63%), oculoplastic or orbital surgery (62%), ocular oncology (90%), pediatrics (94%), vitreoretinal diseases (88%), and uveitis (55%). Stratifying grades across health information categories (disease and condition, risk and prevention, surgery-related, and treatment and management) showed notable but insignificant variations, with disease and condition often rated highest (72% and 69%) for appropriateness and surgery-related (55% and 51%) lowest, in both contexts. Conclusion: This LLM reported mostly appropriate responses across multiple ophthalmology subspecialties in the context of both patient information sites and EMR-related responses to patient questions. Current LLM offerings require optimization and improvement before widespread clinical use.

Incidence and distribution of ocular disorders in the first year of life.

PURPOSE: To describe the incidence and distribution of eye diseases affecting children in the first year of life in Olmsted County, Minnesota. METHODS: We conducted a population-based, retrospective medical record review of infants (≤1 year of age) residing in Olmsted County diagnosed with an ocular disorder from January 1, 2005, through December 31, 2014. RESULTS: A total of 4,223 infants were diagnosed with an ocular disorder, yielding an incidence of 20,242/100,000 births per year, or 1 in 4.9 live births (95% CI, 19,632-20,853). The median age at diagnosis was 3 months, and 2,179 (51.5%) were female. The most common diagnoses included conjunctivitis, in 2,175 (51.5%), nasolacrimal duct obstruction, in 1,432 (33.6%), and pseudostrabismus, in 173 (4.1%). Visual acuity was decreased in one or both eyes in 23 (0.5%) infants because of strabismus in 10 (43.5%) and cerebral visual impairment in 3 (13.0%). A majority of the infants (3,674 [86.9%]) were diagnosed and managed by a primary care provider, and 549 (13.0%) were evaluated and/or managed by an eye care provider. CONCLUSIONS: Although ocular disorders occurred in 1 in 5 infants in this cohort, most conditions were evaluated and managed by primary care providers. Understanding the incidence and distribution of ocular diseases among infants is useful for planning clinical resources.

Hepatic dysfunction in children with tuberculosis on treatment with antituberculous therapy.

AIM: To determine the incidence and factors associated with drug induced hepatic dysfunction in children on anti-tuberculous treatment (ATT). SETTING: Pediatric Tuberculosis Clinic at B.J. Wadia Children's Hospital, Mumbai. MATERIAL AND METHODS: 46 children with tuberculosis on ATT between April 2007 and February 2008 were included. Serum glutamic pyruvic transaminase (SGPT) level was measured at the beginning, after 15 days of starting ATT, at the end intensive phase and then if the patient developed symptoms of hepatic dysfunction. A value 3 times the normal value of the testing laboratory was considered to be significant for liver dysfunction. Liver dysfunction was analysed for association with factors like age, sex, weight, malnutrition, type of tuberculosis and severity of tuberculosis using SPSS Statistics software, Version 15.0. RESULTS: Seven (15.2 %) out of 46 children developed drug induced hepatic dysfunction, of which 2 (28.6%) patients had 2 episodes of liver dysfunction while 5 (71.4%) had 1 episode of liver dysfunction. One (14.3%) developed symptom of hepatitis in the form of jaundice and hepatomegaly. All the patients developing liver dysfunction were in the intensive phase of treatment. The mean age of the children developing liver dysfunction was 4.0 ± 3.76 years. Liver dysfunction was associated with age younger than 3½ years (p = 0.025). Liver dysfunction was not associated with sex, weight, malnutrition, type of tuberculosis and severity of tuberculosis. CONCLUSION: Regular monitoring of SGPT levels is recommended in all children on ATT below the age of 3 ½ years.