Understanding Visual Disorders through Correlation of Clinical and Radiologic Findings.

Patients presenting with visual disturbances often require a neuroimaging approach. The spectrum of visual disturbances includes three main categories: vision impairment, ocular motility dysfunction, and abnormal pupillary response. Decreased vision is usually due to an eye abnormality. However, it can also be related to other disorders affecting the visual pathway, from the retina to the occipital lobe. Ocular motility dysfunction may follow disorders of the cranial nerves responsible for eye movements (ie, oculomotor, trochlear, and abducens nerves); may be due to any abnormality that directly affects the extraocular muscles, such as tumor or inflammation; or may result from any orbital disease that can alter the anatomy or function of these muscles, leading to diplopia and strabismus. Given that pupillary response depends on the normal function of the sympathetic and parasympathetic pathways, an abnormality affecting these neuronal systems manifests, respectively, as pupillary miosis or mydriasis, with other related symptoms. In some cases, neuroimaging studies must complement the clinical ophthalmologic examination to better assess the anatomic and pathologic conditions that could explain the symptoms. US has a major role in the assessment of diseases of the eye and anterior orbit. CT is usually the first-line imaging modality because of its attainability, especially in trauma settings. MRI offers further information for inflammatory and tumoral cases. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

An Unusual Case of an Intradiploic Arachnoid Cyst of the Orbit.

This study reported a case of an arachnoid cyst of the sphenoid bone causing orbital signs and symptoms in a 58-year-old man with progressive proptosis and nonspecific discomfort in the OS. Orbital MRI showed a 3-cm homogeneous cyst within the left greater wing of the sphenoid bone. To the best of our knowledge, this is the first report of an intradiploic arachnoid cyst in the sphenoid bone with atypical radiological features, causing clinical symptoms, and managed through an eyelid approach, achieving a complete resolution with no complications.

Orbital TFE3-Rearranged Perivascular Epithelioid Cell Tumor: A Case Report and Review of the Literature.

ABSTRACT: Perivascular epithelioid cell tumors (PEComas) are infrequent mesenchymal neoplasms that have particular histological and immunohistochemical features. Only a few cases have been described in the eye and orbit. This report presents a 28-year-old man who consulted for a painless left orbital mass. With the presumptive diagnosis of cavernous hemangioma, a surgical excisional biopsy was performed. Histopathological examination showed a well-delimited tumor composed of epithelioid cells with an eosinophilic cytoplasm and oval nucleus. The tumor cells stained diffusely for HMB-45 and transcription factor E3 (TFE3) and were focally positive for actin. There was no reactivity to S100 or desmin. Genetic testing revealed a TFE3 rearrangement. Based on these results, an extremely rare orbital TFE3-rearranged PEComa was diagnosed. Although no recurrence was seen at last follow-up, a review of the literature shows experience is limited regarding orbital PEComas and their malignant potential. Further research is needed to establish management guidelines, their association with the tuberous sclerosis complex, and the role of genetic mutations such as TFE3 rearrangement.

Cytokine production in thyroid eye disease: in vitro effects of dexamethasone and IL-6 blockade with tocilizumab.

PURPOSE: To compare IL-6, sIL-6R, and IL-17 secretion in peripheral blood mononuclear cells (PBMCs) cultured with tocilizumab (a humanized monoclonal antibody against the interleukin-6 receptor), dexamethasone, and placebo, obtained from patients with thyroid eye disease (TED) and healthy controls. METHODS: The study was a prospective proof of concept test. We cultured peripheral blood mononuclear cells from TED patients and healthy controls with tocilizumab, dexamethasone, and placebo. IL-6, sIL-6R, and IL-17 levels in supernatants obtained from PBMCs cultures were analyzed by ELISA. RESULTS: We included seventeen patients with thyroid eye disease (12 females and five males). The mean age was 49 years. Both dexamethasone and tocilizumab influenced IL-6 and IL-6Rs levels in patients' group. Supernatants obtained from PBMCs treated with dexamethasone showed 77.2% and 82.8% lower IL-6 levels compared with those cultured with placebo and tocilizumab, respectively. Furthermore, overnight culture of PBMCs with dexamethasone showed significantly lower sIL-6R secretion compared with untreated (33.71%, p = 0.04) and tocilizumab treated (58.21%, p = 0.01) PBMCs. Neither dexamethasone nor tocilizumab affected IL-17 concentrations in PBMCs cultures. CONCLUSIONS: Both dexamethasone and tocilizumab affect the IL-6/sIL-6R system. Specifically, dexamethasone reduces and tocilizumab increases the levels of these cytokines in PBMCs cultures. These results strengthen the molecular rationale for interrogating the efficacy of tocilizumab in steroid-resistant TED, as IL-6 seems to be a common target for both anti-IL-6R antibody and steroids.

Early retinal atrophy predicts long-term visual impairment after acute optic neuritis.

BACKGROUND: Visual recovery after optic neuritis (ON) used to be defined as good, although patients frequently complain of poor vision. METHODS: We carried out a prospective study on 38 consecutive patients with acute ON followed monthly for 6 months and evaluated high- and low-contrast visual acuity (HCVA and LCVA, respectively), quality of vision (National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25)), visual fields, and retinal thickness by spectral domain optical coherence tomography (OCT). RESULTS: We found significant impaired LCVA and color vision in ON eyes 6 months after acute ON, which impact on quality of life. LCVA and color vision were correlated with the thicknesses of the ganglion cell and inner plexiform layer (GCIPL; 2.5% LCVA r = 0.65 and p = 0.0001; color vision r = 0.75 and p < 0.0001) and that of the peripapillary retinal nerve fiber layer (pRNFL; LCVA r = 0.43 and p = 0.0098; color vision r = 0.62 and p < 0.0001). Linear regression models that included the change in the GCIPL and pRNFL thicknesses from baseline to month 1 after onset explained 47% of the change in 2.5% LCVA and 67% of the change of color vision acuity. When adjusting for the value of visual acuity at baseline, predictors of the change in vision from baseline to month 6 achieved similar performance for all three types of vision (HCVA, LCVA, and color vision). CONCLUSION: Monitoring retinal atrophy by OCT within the first month after ON onset allows individuals at a high risk of residual visual impairment to be identified.

Dynamics of retinal injury after acute optic neuritis.

OBJECTIVE: We set out to assess the dynamics of retinal injury after acute optic neuritis (ON) and their association with clinical visual outcomes. METHODS: Thirty-one consecutive patients with acute ON were prospectively analyzed over a 6-month follow-up period. Each month, we used optical coherence tomography (OCT) to assess the thickness of peripapillary retinal nerve fiber layer (pRNFL) and segmented macular layers, as well as high-contrast visual acuity, low-contrast visual acuity (LCVA), color visual acuity (CVA), and visual fields (VF). RESULTS: In this prospective study, we found that 6 months after clinical onset, ON eyes suffered a reduction in pRNFL (-45.3 µm) and macular thickness (-17.3 µm). Macular atrophy was due to the decrease of macular RNFL thickness (-7.8 µm) and that of the ganglion cell layer and inner plexiform layer (GCIP, -11.3 µm), whereas the thickness of the outer retinal layers increased slightly. The macular RNFL and GCIP thickness decreased in parallel, yet it always occurred more rapidly and more severely for the GCIP. The change in the GCIP thickness in the first month predicted the visual impairment by month 6; a decrease ≥ of 4.5 µm predicted poor LCVA (sensitivity of 93% and specificity of 88%), and a decrease of ≥ 7 µm predicted poor VF and CVA (sensitivity of 78% and 100% and specificity of 63% and 66%, respectively). INTERPRETATION: Retinal axonal and neuronal damage develops quickly after ON onset. Assessment of ganglion cell layer thickness by OCT after ON onset can be used as an imaging marker of persistent visual disability.

Colour vision impairment is associated with disease severity in multiple sclerosis.

BACKGROUND: Colour vision assessment correlates with damage of the visual pathway and might be informative of overall brain damage in multiple sclerosis (MS). OBJECTIVE: The objective of this paper is to investigate the association between impaired colour vision and disease severity. METHODS: We performed neurological and ophthalmic examinations, as well as magnetic resonance imaging (MRI) and optical coherence tomography (OCT) analyses, on 108 MS patients, both at baseline and after a follow-up of one year. Colour vision was evaluated by Hardy, Rand and Rittler plates. Dyschromatopsia was defined if colour vision was impaired in either eye, except for participants with optic neuritis (ON), for whom only the unaffected eye was considered. We used general linear models adjusted for sex, age, disease duration and MS treatment for comparing presence of dyschromatopsia and disease severity. RESULTS: Impaired colour vision in non-ON eyes was detected in 21 out of 108 patients at baseline. At baseline, patients with dyschromatopsia had lower Multiple Sclerosis Functional Composite (MSFC) scores and Brief Repeatable Battery-Neuropsychology executive function scores than those participants with normal colour vision. In addition, these patients had thinner retinal nerve fiber layer (RNFL), and smaller macular volume, normalized brain volume and normalized gray matter volume (NGMV) at baseline. Moreover, participants with incident dyschromatopsia after one-year follow-up had a greater disability measured by the Expanded Disability Status Scale and MSFC-20 and a greater decrease in NGMV than participants with normal colour vision. CONCLUSIONS: Colour vision impairment is associated with greater MS severity.

Clinical anatomy of the periocular region.

The aims of this article are twofold: (1) to provide the facial plastic surgeon with a comprehensive and up-to-date overview of periocular anatomy including the brow, midface, and temporal region and (2) to highlight important anatomical relationships that must be appreciated in order to achieve the best possible functional and aesthetic surgical outcomes.

Surveying a Sample of the Spanish Ophthalmologic Community about Vaccination against Herpes Zoster.

There are currently two authorized vaccines against herpes zoster (HZ) that have been shown to be safe and effective in its prevention: Zostavax, a zoster vaccine live (ZVL), and Shingrix, a recombinant zoster Vaccine (RZV). Because ophthalmologists work with vision-threatening complications of zoster, such as herpes zoster ophthalmicus (HZO), they are in a good position to advocate for vaccination. Our aim was to determine the current knowledge among Spanish ophthalmologists about the effectiveness of the available vaccines against HZ. A Google Forms questionnaire was created and used as the survey platform for this study. It was an anonymous online survey of 16 questions, which was shared among Spanish ophthalmologists in-training and consultants from 27 April 2022 to 25 May 2022. The survey was completed by a total of 206 ophthalmologists of all subspecialties. We obtained responses from 17 of the 19 regions of Spain. Fifty-five percent of the respondents agreed that HZ is a frequent cause of vision loss. However, 27% of the professionals were unaware of the existence of vaccines against HZ and 71% of them did not know in which cases it could be indicated. Only nine ophthalmologists (4%) had ever suggested vaccination against HZ to their patients. Despite this, 93% considered it important to recommend vaccination against HZ if it proved to be safe and effective. Considering the sequelae, complications, and the existence of safe and effective vaccines against HZ, vaccination of the target population could be considered an important public health measure. We are convinced that it is time for ophthalmologists to take an active role in HZO prevention.

Pseudopterygium: An Algorithm Approach Based on the Current Evidence.

Pseudopterygium is a non-progressive conjunctival adhesion to the peripheral cornea secondary to a corneal-limbus damage. According to the literature, the main etiology is a previous eye trauma. Nevertheless, this could be biased by the existence of other underdiagnosed causes of pseudopterygium, some of which may have severe consequences for the integrity of the eye and patient's life. This comprehensive literature review was performed based on a search on the PubMed and Google Scholar databases of relevant pseudopterygium published papers according to our current knowledge and seeks to gather the existing evidence about its diverse etiologies and clinical features, as well as to propose a diagnostic algorithm to simplify its correct approach.

Pillar tarsoconjunctival flap: An alternative approach for the management of refractory corneal ulcer.

PURPOSE: To report the effectiveness of the surgical procedure of the tarsoconjunctival flap (FTC) in patients with severe ocular surface impairment refractory to previous conventional treatments. METHODS: A retrospective, noncomparative, consecutive case series. RESULTS: Pillar tarsoconjunctival flap (PTCF) was performed in eight eyes of eight patients. Three patients had neurotrophic corneal ulcer (NCU), three had exposure keratopathy and two had corneal melting. Seven of them had satisfactory postoperative results, showing total corneal re-epithelialization that lasted throughout the postoperative follow-up (mean 10.33 ± 2.65 months [SD], range 6 to 12 months). Mean time for the re-epithelization was 11.28 ± 8.97 days [SD] (range 4 to 30 days). CONCLUSION: This study suggest PTCF is a valid alternative to tarsorrhaphy in cases of persistent epithelial defect (PED) or NCU resistant to conventional treatments. Notwithstanding, prospective comparative trials comparing PTFC with conventional and/or novel therapies in PED or NCU are needed to corroborate these findings.

Opioids and Ocular Surface Pathology: A Literature Review of New Treatments Horizons.

This review discusses the role of opioids in the corneal surface and the different pathways and therapeutic methods of management. A literature review was performed using PubMed database. For the database search, the main searching words "opioid" and "topical opioid treatment" were used with the descriptors "cornea", "ocular surface", "neuropathic corneal pain", "corneal sensitivity" and "naltrexone"; original scientific articles and reviews were included to achieve the purpose of the review. The endogenous opioid system has relevant functions in the organism, and in daily use, opioids are used as painkillers. However, these drugs may be employed for other indications as opioid pathways have a wide spectrum. The corneal surface for topical treatment is easily accessible, hence sparing the side effects of systemic opioids. Instillation of opioid antagonist substances, such as naltrexone, increases corneal healing rates and stimulates the division of corneal epithelium cells without deleterious effects. The natural modulation of endogenous opioids controls different forms of pain, including inflammatory and neuropathic pain, both in the ocular surface and in the central nervous system. There are diverse methods in controlling pain using opioids, especially in refractory forms. This review attempts to collect the literature about corneal surface and opioid pathways to provide an overview image and a possible direction of the news treatments.

Neuro-ophthalmological features of primary diffuse leptomeningeal gliomatosis.

We performed an in-depth study of the neuro-ophthalmologic signs and symptoms of a rare but fatal disease known as primary diffuse leptomeningeal gliomatosis (PDLG). Two new cases of PDLG are described, and 22 published cases reviewed. Papilledema and sixth nerve palsy are the most common neuro-ophthalmic findings. Other abnormalities include third and fourth nerve palsies, nystagmus, and vision loss. Involvement of the visual system may be part of the initial presentation of PDLG.

Two cases of drug-induced orbital inflammatory disease.

Orbital inflammatory disease (OID) includes all inflammatory processes affecting the orbit. Although several aetiologies are recognised, a cause may not be elucidated. We describe 2 cases in which drugs (hyaluronidase and zoledronic acid) were the cause of OID. In patients with a clinical picture of OID simulating an orbital cellulitis, the recent drug history should be considered as a possible aetiology, and treatment with steroids with or without a biopsy should be considered after an infection has been excluded.

A tricky case of unilateral orbital inflammation: carotid cavernous fistula in Graves-Basedow disease.

Purpose: To describe a rare clinical case of Carotid Cavernous Fistula (CCF) in Graves-Basedow disease (GBD). Method: A 62-year-old female with history of GBD and inactive Graves' ophthalmopathy (GO) was admitted with progressive exophthalmos in her right eye (RE) and diagnosed with GO reactivation the previous month. Results: On examination, dilated and tortuous conjunctival blood vessels, chemosis and exophthalmos were observed in the RE. There was an asymmetry in the intraocular pressures of 20 mmHg in the RE and 10 mmHg in the LE. Laboratory results showed normal thyroid function and positivity of some of the antibodies related to immune thyroid disorders. Neuroimaging showed an early and abnormal filling of the cavernous sinus (CS) and an enlarged superior ophthalmic vein. Consequently, the diagnosis of CCF was established. Interventional treatment was performed with good clinical outcome and no recurrence after 6 months of follow-up. Conclusions: CCFs are abnormal arteriovenous connections between the carotid arteries and CS. CCF picture can mimic the GO's manifestations. Thus, CCF should be considered in the differential diagnosis of GO, especially in unilateral, asymmetric, and atypical cases. We reported herein a case of CCF in a patient diagnosed with GBD, having a previous history of inactive GO, a diagnostic challenge, since the first presumed diagnosis in patients with GBD is always GO. To the best of our knowledge, there are only three previous reports in the medical literature in which the CCF was diagnosed in a GBD patient with a history of GO. Abbreviations: CCF = Carotid Cavernous Fistula, GBD = Graves-Basedow disease, GO = Graves' ophthalmopathy, CS = cavernous sinus, SOV = superior ophthalmic vein, ICA = internal carotid artery, IOP = intraocular pressure.

High Incidence of Cataracts in the Follow-Up of Patients Undergoing Percutaneous Coronary Intervention for Chronic Coronary Total Occlusion.

Development of cataracts is a well-known adverse effect of ionizing radiation, but little information is available on their incidence in patients after other medical procedures, such as cardiac catheterizations. The study objective was to determine the incidence of cataracts in a cohort of patients undergoing percutaneous coronary intervention (PCI) for chronic coronary total occlusion (CTO) and its association with radiation dose. The study analyzed the incidence of cataracts during the follow-up of 126 patients who underwent chronic total coronary PCI, using Cox regression to identify predictive factors of cataract development. The study included 126 patients, 86.9% male, with a mean age of 60.5 years (range, 55.0-68.0 years). Twenty-three (18.2% n = 23) developed cataracts during a mean follow-up of 49.5 months (range 37.3-64.5 months). A higher incidence was observed in patients who received more than 5 Gy (29.0% vs. 14.7%, Hazard ratio (HR = 2.84 [1.19-6.77]). Multivariate analysis revealed a relationship between cataract development during the follow-up and a receipt of radiation dose >5 Gy (HR = 2.60, 95% confidence interval [CI 1.03-6.61]; p = 0.03), presence or history of predisposing eye disease (HR = 4.42, CI:1.57-12.40), diabetes (HR = 3.33 [1.22-9.24]), and older age, as in >57 (HR, 6.40 [1.81-22.61]). An elevated incidence of cataracts was observed in patients after PCI for CTO. The onset of cataracts is related to the radiation dose during catheterization, which is a potentially avoidable effect of which operators should be aware.

Hypertrichosis of the upper cheek area associated with travoprost treatment of glaucoma.

Travoprost is a prostaglandin analog used in the treatment of open-angle glaucoma. This drug is safe and efficacious and has a low incidence of systemic and local side effects. Common local side effects are conjunctival hyperemia, iris pigmentation, and hypertrichosis of the eyelashes. The authors present a case of a patient who developed marked hypertrichosis of the cheek vellus 3 months after starting treatment with travoprost.

Combined and simultaneous endoscopic endonasal and transorbital surgery for a Meckel's cave schwannoma: technical nuances of a mini-invasive, multiportal approach.

OBJECTIVE: Over the years, Meckel's cave pathologies have been judged off-limits because of high rates of morbidity. Even though several studies have defined various surgical routes with tolerable morbidity and mortality rates, controversies related to the optimal avenue to treat different categories of Meckel's cave and cavernous sinus neoplasms persist. With unceasing energy to cultivate minimally invasive neurosurgical approaches, the endoscopic endonasal route has been tested, and the approach effectively performed, to provide a valid surgical window to these areas. In this dynamic and challenging scenario, another ventral endoscopic minimally invasive route-that is, the superior eyelid endoscopic transorbital approach-has been very recently proposed, and used in selected cases, to access the cavernous sinus and Meckel's cave regions. METHODS: The authors report the technical nuances of a combined and simultaneous endoscopic endonasal and transorbital surgical treatment of a patient with a Meckel's cave schwannoma. The operation involved collaboration among neurosurgery, otorhinolaryngology, and ophthalmology (oculoplastic surgery). The patient recovered well, had no neurological deficits, and was discharged to home 3 days after surgery. RESULTS: The multiportal combined route was proposed for the following reasons. The endonasal approach, considered to be more familiar to our skull base team, could allow control of possible damage of the internal carotid artery. From the endonasal perspective, the most inferior and medial portion of the tumor could be properly managed. Finally, the transorbital route, by means of opening the lateral wall of the cavernous sinus via the meningoorbital band, could allow control of the superolateral part of the tumor and, most importantly, could permit removal of the portion entering the posterior cranial fossa via the trigeminal pore. Simultaneous surgery with two surgical teams working together was planned in order to reduce operative time, hospital stay, and patient stress and discomfort, and to ensure "one-shot" complete tumor removal, with minimal or no complications. CONCLUSIONS: This study represents the translation into the real surgical setting of recent anatomical contributions related to the novel endoscopic transorbital approach and its simultaneous integration with the endoscopic endonasal pathway. Accordingly, it may pave the way for future applications related to minimally invasive, multiportal endoscopic surgery for skull base tumors.

Using Acute Optic Neuritis Trials to Assess Neuroprotective and Remyelinating Therapies in Multiple Sclerosis.

Importance: Neuroprotective and remyelinating therapies are required for multiple sclerosis (MS), and acute optic neuritis (AON) is a potential condition to evaluate such treatments. Objective: To comprehensively assess key biological and methodological aspects of AON trials for testing neuroprotection and remyelination in MS. Design, Setting, and Participants: The AON-VisualPath prospective cohort study was conducted from February 2011 to November 2018 at the Hospital Clinic of University of Barcelona, Barcelona, Spain. Consecutive patients with AON were prospectively enrolled in the cohort and followed up for 18 months. Data analyses occurred from November 2018 to February 2019. Exposures: Participants were followed up for 18 months using optical coherence tomography, visual acuity tests, and in a subset of 25 participants, multifocal visual evoked potentials. Main Outcomes and Measures: Dynamic models of retinal changes and nerve conduction and their associations with visual end points; and eligibility criteria, stratification, and sample-size estimation for future trials. Results: A total of 60 patients (50 women [83%]; median age, 34 years) with AON were included. The patients studied displayed early and intense inner retinal thinning, with a thinning rate of approximately 2.38 µm per week in the ganglion cell plus inner plexiform layer (GCIPL) during the first 4 weeks. Eyes with AON displayed a 6-month change in latency of about 20 milliseconds, while the expected change in the eyes of healthy participants by random variability was 0.13 (95% CI, -0.80 to 1.06) milliseconds. The strongest associations with visual end points were for the 6-month intereye difference in 2.5% low-contrast letter acuity, which was correlated with the peripapillary retinal nerve fiber layer thinning (adjusted R2, 0.57), GCIPL thinning (adjusted R2, 0.50), and changes in mfVEP latency (adjusted R2, 0.26). A 5-letter increment in high-contrast visual acuity at presentation (but not sex or age) was associated with 6-month retinal thinning (1.41 [95% CI, 0.60-2.23] µm less peripapillary retinal nerve fiber layer thinning thinning; P = .001; adjusted R2, 0.20; 0.86 [95% CI, 0.35-1.37] µm less GCIPL thinning; P = .001; adjusted R2, 0.19) but not any change in multifocal visual evoked potential latency. To demonstrate 50% efficacy in GCIPL thinning or change in multifocal visual evoked potential latency, a 6-month, 2-arm, parallel-group trial would need 37 or 50 participants per group to test a neuroprotective or remyelinating drug, respectively (power, 80%; α, .05). Conclusions and Relevance: Acute optic neuritis is a suitable condition to test neuroprotective and remyelinating therapies after acute inflammation, providing sensitive markers to assess the effects on both processes and prospective visual recovery within a manageable timeframe and with a relatively small sample size.