Compressive Optic Neuropathy Research Articles

Case Report: Periocular Steroid Injection Combined Vemurafenib Therapy in the Long-term Management of Orbital Involvement of Erdheim-Chester Disease.

Abstract Purpose: To report a rare case of vision loss from Erdheim-Chester Disease (ECD) and long term, follow up, management and results. Case Report: A 62-year-old woman presented with bilateral exophthalmos and deterioration in visual acuity of both eyes. Magnetic resonance imaging studies revealed bilateral intraconal orbital tumors. She was diagnosed optic neuropathy in both eyes. The patient was treated with pegylated interferon and systemic pulse steroid. However, recurrences were common. Subsequently, the patient underwent regular corticosteroid injection directly into the retro-orbital region of both eyes monthly, which led to intermittent improvement of vision. At the 8th month of visual impairment, Vemurafenib was initiated. Results: The visual acuity and visual field scores were stable for 6 years during the follow-up. No major complication was observed. Conclusion: Combined vemurafenib and perioculer steroid injection is a well tolerated therapy with less systemic adverse effects which may benefit patients with orbital involvement of ECD. Key words: Erdheim–Chester disease; compressive optic neuropathy; vemurafenib; Optical coherence tomography; Optical coherence tomography angiography.

A Case of Compressive Optic Neuropathy Caused by Orbital Emphysema

Purpose: We report a patient with compressive optic neuropathy (CON) in whom visual acuity (VA) was restored after removing conjunctival emphysema through the conjunctiva.Case summary: A 55-year-old man was transferred to emergency department complaining of decreased VA in the right eye after trauma to the right temporal side of the head. The best-corrected visual acuity (BCVA) was 0.6 in the right eye. We confirmed a relative afferent pupillary defect and limited eye movement inferiorly in the right eye. The patient had blown his nose several times while being transferred to the hospital. On silt-lamp examination, there was conjunctival emphysema of the right eye. Orbital computed tomography showed a medial wall fracture of the right orbit and orbital emphysema above the optic canal. The P100 wave was delayed in the visual evoked potential (VEP) exam, and there was a decreased peripheral visual field on the Humphrey visual field test. Based on these findings, we diagnosed CON and treated him accordingly. To decompress the intra-orbital area, we injected mannitol intravenously, applied intraocular pressure-lowering and antibiotic eye drops in the right eye, and removed part of the conjunctival emphysema through the conjunctiva. At the 3-week follow-up, the BCVA had improved to 1.0 in the right eye with a normalized P100 wave on the VEP exam.Conclusions: In a case of CON caused by orbital emphysema after trauma, it is important to decrease the intra-orbital pressure as soon as possible to enable a good prognosis for the VA.

Visual Field Patterns in Optic Neuropathy

The optic nerve is susceptible to a wide variety of pathological processes. In optic neuropathy, quantitative visual field analysis using standard automated perimetry is critical in initial diagnosis, monitoring disease progression, and guiding treatment plans. While acquired visual field defects may progress rapidly, congenital visual field defects may not progress or may progress slowly. Although the most common defect in congenital or acquired optic neuropathy is the central defect, many defect types that are not pathognomonic to the disease can be seen. In ischemic optic neuropathy, which is common among optic neuropathies, arcuate defect and central defect are usually seen. In disc disorders with localized nerve damage, such as optic disc coloboma or optic pit, localized defects such as arcuate scotoma or cecocentral scotoma in which the fixation point is preserved may be observed. Papilledema causes peripapillary fluid accumulation and an enlarged blind spot in the perimetry. In compressive optic neuropathies, non-specific defects such as central and subtudinal defects may be observed, as well as quadrant defects such as hemianopsia and quadrionopsia. Studies show that the combined use of optical coherence tomography and standard automated perimetry to determine structure-function relationships improves clinical care in neuro-ophthalmic disorders.

Compressive Optic Neuropathy; Clinical Findings, Etiopathogenesis, Diagnosis and Treatment

Compressive optic neuropathy (COM) is a type of optic neuropathy characterized by slow and progressive vision loss, usually unilateral, resulting from the intrinsic or extrinsic compression of any part of the optic nerve. This condition may develop because of infectious, inflammatory, vascular, traumatic, and neoplastic factors. In the early diagnosis of the disease, making differential diagnoses correctly; evaluating additional hematological, biochemical, and inflammatory blood parameters; and applying orbital and neuroimaging methods when necessary are critical. In addition, coordination with other branches of science in diagnosing and treating CON is an important step in terms of disease management and the improvement of visual prognosis.

Non-Arteritic Ischemic Optic Neuropathy

Non-arteritic ischemic optic neuropathy (NAION); it is an optic neuropathy that can cause acute unilateral painless vision loss, optic disc edema, relative afferent pupillary defect, peripapillary hemorrhage and visual field defects in individuals over 50 years of age. Although its pathophysiology has not been fully elucidated today; It is widely accepted that it results from hypoperfusion in the short posterior ciliary arteries that supply the optic nerve head. The diagnosis of NAION is made primarily clinically. While there is no vision loss in approximately one-third of the cases, severe vision loss develops in 40-50%. It is thought that there is always a visual field defect from the beginning of NAION, therefore perimetry is the most important diagnostic test. In the differential diagnosis of NAION, vision- and life-threatening diseases, especially arteritic ischemic optic neuropathy, demyelinating, infiltrative and compressive optic neuropathies, should be considered. Perimetry, blood tests, fluorescein angiography, optical coherence tomography (OCT), OCT angiography, brain and orbital MRI provide very useful information in the differential diagnosis. Although many medical and surgical treatments have been tried, unfortunately there is no effective treatment or prophylactic prevention method for NAION today; However, neuroprotective agents, stem cell and gene therapy treatments aimed at stopping or reducing optic nerve damage appear promising in the near future. The purpose of this review is to provide a comprehensive, up-to-date overview of NAION.

Etiologies and Outcomes of Granulomatosis With Polyangiitis-Associated Optic Neuropathy: A Case Series and Review of the Literature.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare autoimmune disease characterized by inflammation of small- to medium-sized blood vessels (vasculitis). We described the 3 causes of GPA-associated optic neuropathy (compressive, inflammatory, or ischemic) and analyzed initial and final visual acuities (VAs) in each group, which could potentially help prognosticate visual outcomes depending on the etiology of optic neuropathy. This was a retrospective chart review of patients who were diagnosed with GPA-associated optic neuropathy and were seen in the Department of Ophthalmology at Mayo Clinic in Rochester, Minnesota. Only patients who met the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology GPA classification criteria were included. A total of 12 patients with GPA-associated optic neuropathy were identified. The etiology of the optic neuropathy was compressive in 6 patients, inflammatory in 4 patients, and ischemic in 3 patients. One patient had compressive optic neuropathy initially, then presented with GPA-related optic neuritis years later. Four patients in the compressive optic neuropathy group had orbital masses requiring orbitotomy for debulking, and the remaining 2 patients had compression from pachymeningitis. Average logarithm of the minimum angle of resolution (logMAR) VA at optic neuropathy onset was 1.50, 1.50, and 0.67 (Snellen equivalent 20/600, 20/600, and 20/100, respectively). At the last follow-up, average logMAR VA was 0.91, 1.73, and 1.10 (Snellen equivalent 20/160, 20/1,000, and 20/250, respectively) for each group. Visual outcomes were variable, with compressive and inflammatory optic neuropathies showing improvement in 4 eyes and worsening in 3 eyes in total. Patients with ischemic optic neuropathy from GPA were either stable or worsened over the course of the disease.

Delayed Surgical Reversal of Optic Nerve Compression Leads to Exponential Degeneration of Optic Nerve Fibers and Selective Sparing of the Small Fibers.

To evaluate the effectiveness of surgical reversal of experimental optic nerve compression in treating persistent compressive optic neuropathy and to explore the relationship between surgical outcomes and the timing of the procedure. Surgical reversal procedures (decompression surgery) were conducted at five time intervals: 1, 3, and 7 days and 2 and 3 weeks following optic nerve compression in a rabbit model. The groups were labeled as DC-1d, DC-3d, DC-7d, DC-2w, and DC-3w, respectively. The study investigated changes in ganglion cell complex (GCC) thickness using spectral-domain optical coherence tomography and the percentage of surviving retinal ganglion cells (RGCs) through immunofluorescence staining and optic nerve axons stained with p-phenylenediamine at 4 weeks after decompression. Additionally, the area distribution of surviving axons was analyzed. The decline in GCC thickness was halted following decompression. The remaining thickness of the GCC in group DC-1d was found to be statistically significantly higher at 2, 3, and 4 weeks postonset compared to the no-decompression group. Similarly, GCC thickness in group DC-3d was significantly higher at 3 and 4 weeks postonset. The percentage of surviving RGCs and axons at 4 weeks postonset exhibited an exponential correlation with the onset time of decompression, with R2 values of 0.72 and 0.78, respectively. The surviving axon area declined following delayed decompression. Persistent substantial compression on the optic nerve leads to exponential degeneration of the optic nerve, initially affecting larger optic nerve fibers. Early intervention aimed at relieving the compression on the optic nerve may offer potential benefits in mitigating the degenerative effects and conserving visual function.

Are Peripapillary Hyperreflective Ovoid Mass-like Structures with an Elevated Optic Disc Still a Diagnosis Dilemma?

ObjectiveTo identify clinical characteristics that would help make or rule out the diagnosis of Idiopathic intracranial hypertension (IIH) in patients referred for papilledema (PE) with peripapillary hyperreflective ovoid mass-like structures (PHOMS) DesignA retrospective cohort study SubjectsAll patient referred for PE excluding PE with Frisén grade ≥ 3, optic neuritis, ischemic optic neuropathy, compressive optic neuropathy. Patients were divided into 2 groups:Group1= isolated PHOMSGroup2= PHOMS associated with IIH MethodsWe analyzed the location of PHOMS based on OCT-EDI and calculated their volume Main outcome MeasuresP RNFL,GCC, PHOMS’ volume Results154 patients (308 eyes) were included, mean age was 29 with female predominance (78%). PHOMS were associated to these etiologies : IIH(38.3%) isolated (35.7%), posterior uveitis (11%),optic disc drusen (ODD)(10%), and tilted optic disc (5%). An MRI was obtained in 83.1% of cases. More than half of the MRIs were interpreted as consistent with IIH. However, only 39.7% of these patients had confirmed IIH with 44.5% of sensitivity and 55.5% of sensibility. PHOMS were overrepresented in the nasal region (95.5%).The location of PHOMS in the superior and/ or inferior quadrant was significantly associated to IIH or optic dusc drusen, while their presence in the temporal or nasal sector was strongly associated to isolated lesions. PHOMS mean and median volume were 1.66 μm3 and 1.50 μm3 respectively. There was a significant difference in PHOMS volume with higher volume in IIH patients (p=0.0037). Follow up of these patients at 3 and 6 months demonstrated no significant changes in visual function, as per visual field mean deviation, and visual acuity measurements as well as Ganglion cell layer (GCL).Mean pRNFL, showed a decrease of -4.225 μm at 3 months and of –6.489 μm at 6 months, when compared to initial measurement independing of the etiology. ConclusionIsolated PHOMS should be considered as a distinct entity. In asymptomatic patients, PHOMS should be carefully studied. Nasal or temporal location, small volume, stable aspect over the course of weeks or months, are so suggestive of this entity. This strategy would considerably reduce the impact on patients’ anxiety and morbidity.

Visual morbidity in macroprolactinoma: A retrospective cohort study.

The management of visual field damage in patients with macroprolactinomas is a major therapeutic challenge. We aimed to study the visual morbidity associated with macroprolactinoma and its outcomes following medical and surgical treatment. We aimed to identify predictors of visual recovery. We retrospectively reviewed patient's data including clinical presentation, serial pituitary magnetic resonance imaging, laboratory tests, visual symptoms and neuro-ophthalmologic examination, visual field tests and optical coherence tomography tests. The main outcome was complete visual field recovery. Descriptive analyses were conducted. Predictors of visual recovery were investigated. The study cohort included 150 patients with macroprolactinoma [median follow-up, 6.0 years (interquartile range (IQR) 2.9-10.6)]. At diagnosis, visual field defects were evident in 40 patients (26.7%). At the end of follow-up, 24 out of 39 available visual field tests (61.5%) exhibited complete recovery. Patients that achieved complete visual recovery had smaller macroadenomas at diagnosis [30.5 mm (15.0-80.0) vs. 42.0 mm (30.0-85.0), p < .01], lower baseline serum prolactin levels [1414 mcg/L (489-3586) vs. 4119 mcg/L (2715-6315), p < .01], lower rates of central hypogonadism (78.3% vs. 93.3%, p = .05) and central hypothyroidism (20.8% vs. 53.3%, p = .04), lower rates of compressive optic neuropathy (35.3% vs. 87.5%, p = .02) and a better visual acuity (better than 6/8 in both eyes, 93.7% vs. 28.6%, p < .01). In our cohort of 150 patients with macroprolactinoma, 40 patients (26.7%) presented with visual field defects, of which 61.5% achieved complete visual recovery with treatment. Patients that achieved complete visual recovery presented with smaller macroadenomas, lower serum prolactin levels, lower rates of central hypogonadism and central hypothyroidism, lower rates of compressive optic neuropathy and better visual acuity.

Orbital Involvement as an Initial Presentation of Sinonasal Neuroendocrine Carcinoma

Purpose: To present a case of sinonasal neuroendocrine cancer initially manifesting with orbital involvement.Case summary: A 63-year-old female patient visited a neuro-ophthalmologic clinic due to a 3-week history of decreased visual acuity (VA), color vision abnormalities, and swelling of the upper eyelid in right eye. Best-corrected VA (BCVA) in the right eye was 0.8. Clinical findings included a relative afferent pupillary defect, restricted eye movement in lateral, superior, and medial gaze, and exophthalmos in the right eye. Wide-field fundus photography and optical coherence tomography indicated swelling of right optic disc. Magnetic resonance imaging was performed revealing a mass invading nasal cavity, sinus, and right orbital apex, and compressing the medial rectus, inferior rectus, superior oblique, and optic nerve. An otolaryngological nasal biopsy was conducted and immunohistochemical staining showed positive results for Ki-67, NSE, p16, P-53, and CD56, leading to a diagnosis of small cell neuroendocrine carcinoma with an irreducible tumor stage. Prior to initiation of treatment (concurrent chemoradiotherapy, CCRT), there was rapid worsening of VA and eye movement in the right eye. CCRT commenced alongside high-dose steroid treatment. One month following treatment, the BCVA of the right eye improved and protrusion of the right eye resolved. Six months after starting CCRT, a contrast-enhanced orbital computed tomography scan showed no residual lesion. The BCVA of the right eye stabilized at 0.3 with complete recovery of color vision and eye movement.Conclusions: Neuroendocrine cancer should be considered as a possible diagnosis in cases of rapidly progressing compressive optic neuropathy.

Optic neuropathy from hypovitaminosis A in a series of children with severe dietary restrictions.

Hypovitaminosis A is a leading cause of preventable childhood blindness, especially in developing nations. Vitamin A is a fat-soluble essential micronutrient that serves vital functions in the visual system and in regulating bone resorption. We report on a series of four children with mixed nutritional and compressive optic neuropathy and provide a review of the literature. A retrospective observational study of four males (ages 9-12), three with autism spectrum disorder who presented with loss of vision and multiple vitamin deficiencies including hypovitaminosis A. Patients presented with unexplained visual loss or a change in visual behaviour. All patients had severely restricted diet comprising of predominantly carbohydrates. Two of the four cases demonstrated optic nerve pallor at initial presentation with marked optic atrophy developing in all patients over time. Electrophysiology available in two patients demonstrated optic nerve dysfunction with preserved retinal function. Extensive investigations revealed profound deficiency in multiple vitamins including vitamin A (<0.1-0.2 μmol/L, normal = 0.9-1.7 μmol/L). Three patients also had low vitamin B12 (90-111 pmol/L, normal = 170-800 pmol/L) with normal folate. All four cases had radiological evidence of skull base thickening indicative of low vitamin A. Genetic testing did not find any relevant pathogenic variants. Hypovitaminosis A is a crucial form of nutritional deprivation that results in significant visual loss with potential hyperostosis and optic nerve compression exacerbating nutritional optic neuropathy. Additional micronutrient deficiencies usually co-exist and may contribute. Extra vigilance in vitamin replacement is required of clinicians with patients with autism who have restricted diets.

A rare presentation of saccular aneurysm at junction of middle carotid artery and internal carotid artery mimicking the normal tension glaucoma

Acquired (pathologic) excavation of the optic cup is not always due to glaucoma. Ophthalmological signs of pathological disc cupping have been recognised in patients with normal intraocular pressures and with optic atrophy of various causes including ischaemic and compressive optic neuropathy, optic neuritis and trauma. Intracranial lesions have been reported to mimic the clinical presentation of glaucomatous optic disc changes and have also resulted in misdiagnosis. Optic atrophy caused by intracranial lesions shows pallor more than cupping. The macular nasal–temporal ratio compares the nasal and temporal thickness of the macular ganglion cell and inner plexiform layer and can be used to differentiate between compressive and non-compressive lesions as depicted in this case. This case report also suggests the importance of different imaging modalities in diagnosing compressive lesions and differentiating them from similar conditions.

Orbital Myositis Leading to Compressive Optic Neuropathy after COVID-19 mRNA Vaccination: A Case Report

Purpose: We present a case of bilateral compressive optic neuropathy secondary to orbital myositis after COVID-19 messenger ribonucleic acid (mRNA) vaccination.Case summary: A 51-year-old man developed acute bilateral visual loss and painful ophthalmoplegia shortly after receiving the second dose of the BNT162b2 COVID-19 mRNA vaccine (Comirnaty, Pfizer®, New York, NY, USA). His best-corrected visual acuity was counting fingers at 30 cm and 50 cm in the right and left eyes, respectively. Bilateral eyelid swelling, erythema, conjunctival injection, and color vision deficit were observed. Orbital magnetic resonance imaging revealed diffuse enhancement and hypertrophy of the bilateral extraocular muscles. The patient had no significant ophthalmic history and systemic work-up revealed no evidence of underlying inflammatory disease. Based on the clinical presentation and imaging findings, he was tentatively diagnosed with bilateral compressive optic neuropathy secondary to orbital myositis after COVID-19 mRNA vaccination. He was treated with intravenous corticosteroids for 3 days followed by low-dose oral corticosteroids. One month later, his visual acuity and color vision improved; moreover, the inflammatory signs in the eyelids, conjunctiva, and extraocular muscles resolved. However, after developing COVID-19, he experienced a recurrence of symptoms and was retreated with corticosteroids. Radiotherapy was administered to address persistent color vision deficit in the right eye and recurrent eyelid edema, erythema, and conjunctival injection. This treatment effectively resolved all inflammatory signs and color vision deficits, without signs of recurrence.Conclusions: Acute fulminant orbital myositis with associated compressive optic neuropathy can manifest after COVID-19 mRNA vaccination and onset of COVID-19. Anti-inflammatory treatment measures can effectively control these manifestations.

Endoscopic endonasal optic nerve decompression in children younger than 2 years old with congenital optic canal stenosis: illustrative cases.

Congenital optic canal stenosis causing compressive optic neuropathy is a rare disorder that presents unique diagnostic and treatment challenges. Endoscopic endonasal optic nerve decompression (EOND) has been described for optic nerve compression in adults and adolescents but has never been reported for young children without pneumatized sphenoid sinuses. The authors describe preoperative and intraoperative considerations for three patients younger than 2 years of age with congenital optic canal stenosis due to genetically confirmed osteopetrosis or chondrodysplasia. Serial ophthalmological examinations, with a particular focus on object tracking ability, fundoscopic examination, and visual evoked potential trends in preverbal children, are important for detecting progressive optic neuropathy. The lack of pneumatization of the sphenoid sinus presents unique challenges and requires the surgical creation of a sphenoid sinus with the use of neuronavigation to determine the limits of bony exposure given the lack of easily identifiable anatomical landmarks such as the opticocarotid recess. There were no perioperative complications. EOND for congenital optic canal stenosis is safe and technically feasible even given the lack of pneumatization of the sphenoid sinus in young patients. The key operative step is surgically creating the sphenoid sinus through careful bony removal with the aid of neuronavigation. https://thejns.org/doi/10.3171/CASE23559.

Surgical Outcomes of Revision Orbital Reconstruction in Patients With Inadequate Primary Orbital Fracture Repair.

This study aimed to investigate the clinical characteristics of patients with complications following inadequate primary orbital fracture repair and to evaluate surgical outcomes of secondary revision orbital reconstruction. The authors retrospectively reviewed data from 41 patients who underwent revision orbital reconstruction by a single surgeon following complications from primary orbital fracture repair performed elsewhere. Clinical characteristics, including enophthalmos, exophthalmos, diplopia, ocular motility limitation, epiphora, infraorbital hypoesthesia, infection, eyelid malposition, lagophthalmos, hypoglobus, and compressive optic neuropathy, were assessed. Surgical outcomes of revision surgery were evaluated to determine improvements in clinical deficits and postoperative patient satisfaction. The most common postoperative complications of primary orbital fracture repair were enophthalmos (n=20/41) and diplopia (n=20/41). The mean time between primary and revision surgeries was 67.2 months (range: 1-276mo). Revision surgery significantly improved enophthalmos, diplopia (Hess area ratio), epiphora (Munk score), periorbital pain, and exophthalmos ( P =0.003, P =0.001, P <0.001, P <0.001, and P =0.007, respectively) compared to the pre-revision state. In addition, 6 patients experienced improved infraorbital hypoesthesia. Among the 41 patients, 23 were very satisfied, 17 were satisfied, and 1 was neutral after revision orbital reconstruction. Our study highlights the positive impact of revision orbital reconstruction in addressing complications from inadequate primary orbital fracture repair. Surgeons should consider revision surgery to address clinical deficits following prior surgery, especially when anatomic abnormalities are evident in imaging studies, regardless of the time lapse since the initial surgery or concerns about tissue fibrosis and fat atrophy.

Acute vision threatening thyroid orbitopathy associated with cataract surgery: A case series and review of the literature

Thyroid eye disease can be associated via cataract surgery with devastating visual consequences. A systematic review of the clinical records of patients with aggravation of their thyroid orbitopathy after eye surgery at a tertiary facility was performed. Patients who had worsening of their thyroid orbitopathy within the post-operative period of cataract surgery were included. Clinical findings were reviewed including clinical examination and imaging. A literature review was also performed. Two patients who underwent cataract surgery and presented with newly diagnosed and severe thyroid orbitopathy post-operatively were identified. Both patients were male, averaging 71 years of age. Both patients noticed symptoms of diplopia within one week after cataract surgery and had clinical and imaging findings consistent with severe thyroid orbitopathy. Both patients required urgent bilateral orbital decompression due to compressive optic neuropathy. Visual acuity was preserved in both patients. The average follow-up was 6 years. Males who have activation of TED after cataract surgery appear to have a higher risk of developing compressive optic neuropathy and require immediate intervention, which is consistent with male gender as a risk factor for severe disease. Patients with thyroid disease should be counselled pre-operatively about the risk of disease aggravation after cataract surgery.

Deep-Learning Based Automated Segmentation and Quantitative Volumetric Analysis of Orbital Muscle and Fat for Diagnosis of Thyroid Eye Disease.

Thyroid eye disease (TED) is characterized by proliferation of orbital tissues and complicated by compressive optic neuropathy (CON). This study aims to utilize a deep-learning (DL)-based automated segmentation model to segment orbital muscle and fat volumes on computed tomography (CT) images and provide quantitative volumetric data and a machine learning (ML)-based classifier to distinguish between TED and TED with CON. Subjects with TED who underwent clinical evaluation and orbital CT imaging were included. Patients with clinical features of CON were classified as having severe TED, and those without were classified as having mild TED. Normal subjects were used for controls. A U-Net DL-model was used for automatic segmentation of orbital muscle and fat volumes from orbital CTs, and ensemble of Random Forest Classifiers were used for volumetric analysis of muscle and fat. Two hundred eighty-one subjects were included in this study. Automatic segmentation of orbital tissues was performed. Dice coefficient was recorded to be 0.902 and 0.921 for muscle and fat volumes, respectively. Muscle volumes among normal, mild, and severe TED were found to be statistically different. A classification model utilizing volume data and limited patient data had an accuracy of 0.838 and an area under the curve (AUC) of 0.929 in predicting normal, mild TED, and severe TED. DL-based automated segmentation of orbital images for patients with TED was found to be accurate and efficient. An ML-based classification model using volumetrics and metadata led to high diagnostic accuracy in distinguishing TED and TED with CON. By enabling rapid and precise volumetric assessment, this may be a useful tool in future clinical studies.

Metastasis of small cell lung cancer to bilateral extraocular muscles: a case report

BackgroundOrbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in literature.Case presentationA 46-year-old white male with a past medical history of smoking and stage IV small cell lung carcinoma presented with loss of vision and pain in the left eye. Examination revealed bilateral proptosis and left afferent pupillary defect, and visual acuity was hand motion on the left eye and 4/10 on the right eye. An orbital computed tomography scan showed a compression of the left optic nerve between the extraocular muscles at the apex, and a lateral canthotomy was performed for a new-onset compressive optic neuropathy, with residual visual improvement. There was also significant enlargement of the extraocular muscles in the right orbit. The patient was maintained in palliative treatment with both chemotherapy and local medical and surgical (amniotic membrane cover for exposure keratopathy) ophthalmological treatments until he eventually died 5 months after.ConclusionBilateral metastasis to the extraocular muscles is a very rare manifestation of small cell lung cancer and the palliative treatment in these cases is challenging.

To study visual evoked potential in patients with optic nerve diseases

Background: To study Visual Evoked Potential in patients with Optic Nerve diseases. Study design and settings: Cross – Sectional study. Material and Methods: 72 patients were included in our study, aged 12 years and above, diagnosed as optic nerve diseases like optic neuritis, Nonarteritc ischemic optic neuropathy (NAION), hereditary, nutritional and compressive optic neuropathy etc. Flash and Pattern VEPs were used. VEP amplitude and latency were noted. Results: Out of total 144 eyes, 107 eyes were affected. Ethambutol induced Toxic optic neuropathy was the most common etiology, followed by secondary optic neuropathy, idiopathic, traumatic optic neuropathy, optic neuritis, NAION, compressive and post CRAO (Central Retinal Artery Occlusion) optic neuropathy. Mean latency of all affected eyes was 132±22.5 msec. mean amplitude of the all affected eyes was 6.36±2.1 microvolt. Latency was moderately delayed (110-140 msec) in 45.8%, while severely delayed (&gt;140 msec) in 40.3% of total eyes. Amplitude was reduced in 36.5% of total eyes. Prolonged latency with normal amplitude was found in most of the eyes with optic neuritis while latency was also prolonged in fellow eyes of optic neuritis. In case of NAION prolonged latency and reduced amplitude was found in all eyes. Conclusion: Flash or pattern VEP can be used as an important tool for diagnosis of optic neuropathies or revealing of recurrence of disease and for follow up. VEP can also be used to detect subclinical damage in the fellow eyes of optic neuritis and also useful in differentiating the optic neuritis from ischemic optic neuropathy.

Applying the 2022 optic neuritis criteria to noninflammatory optic neuropathies with optic nerve T2-hyperintensity: an observational study.

Recent diagnostic criteria for optic neuritis include T2-hyperintensity of the optic nerve (ON), even without associated contrast enhancement. However, isolated ON-T2-hyperintensity is a nonspecific finding found in any optic neuropathy or severe retinopathy. We applied the 2022 optic neuritis diagnostic criteria to a cohort of patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one eye, to assess the rate of optic neuritis misdiagnosis using these criteria. Retrospective study of consecutive patients who underwent brain/orbit MRI with/without contrast between 07/01/2019 and 06/30/2022. Patients with ON-T2-hyperintensity in at least one eye were included. The 2022 optic neuritis diagnostic criteria were applied to patients with noninflammatory optic neuropathies who had an ophthalmologic examination available for review. Of 150 patients included, 85/150 had compressive optic neuropathy; 32/150 had glaucoma; 12/150 had papilledema; 8/150 had hereditary (3), radiation-induced (3), nutritional (1), traumatic (1) optic neuropathies (none fulfilled the criteria); 13/150 had ischemic optic neuropathy and 4 fulfilled the criteria as definite optic neuritis due to contrast enhancement of the ON head. Seven additional patients would have satisfied the diagnostic criteria if red flags for alternative diagnoses had been overlooked. The application of the 2022 optic neuritis diagnostic criteria in patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one ON resulted in misdiagnosis of optic neuritis in only 4 patients because of ON head enhancement, all with nonarteritic anterior ischemic optic neuropathy. Neuro-ophthalmologic evaluation and exclusion of the ON head as a location in the MRI criteria would have prevented optic neuritis misdiagnosis in our study.