Orbital Magnetic Resonance Imaging Research Articles

Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland: A case report and literature review.

The most common subtype of primary lymphoma of the ocular adnexa is the mucosa-associated lymphoid tissue (MALT) subtype. MALT lymphoma of the lacrimal gland is relatively rare among the lacrimal gland tumors, and the early clinical symptoms are atypical, which can easily lead to misdiagnosis and missed diagnosis. Here, we report a case of MALT lymphoma of the lacrimal gland and explore its clinical manifestations, pathological characteristics, management, and pathogenesis, with the aim of helping clinicians gain an in-depth understanding of ocular adnexal MALT lymphoma. A 60-year-old man presented to our hospital with proptosis and diplopia. The right eye deviated and shifted toward the lower part of the nose. Orbital enhanced magnetic resonance imaging suggested a mass with a maximum cross-section of 3.2 × 2.1 cm. T1 weighted image was isointense, and the enhancement was more uniform and obvious. The right orbital mass was treated surgically, and the final pathology report was MALT lymphoma. After the pathological report was released, the patient was transferred to the hematology department for further diagnosis and no further treatment was given eventually. Seven months later, the patient did not complain of discomfort. Whole-body positron emission tomography-computed tomography, superficial lymph node examination and orbital magnetic resonance imaging revealed no abnormal changes. The clinical manifestations of MALT lymphoma are heterogeneous. Imaging examination is important for assessing the size of the tumor and its relationship with adjacent tissues. Postoperative pathological examination may provide further evidence for the evaluation of the patient's surgical efficacy and prognosis. Management of MALT lymphoma of the lacrimal gland requires a multidisciplinary approach involving ophthalmologists, hematologists, and radiotherapists.

(What's the story) morning glory? MRI findings in morning glory disc anomaly.

Morning glory disc anomaly (MGDA) is a rare congenital ophthalmologic disorder. Historically it has been diagnosed fundoscopically, with little in the literature regarding its imaging findings. The purpose of this study is to further characterize the orbital and associated intracranial magnetic resonance imaging (MRI) findings of MGDA in our tertiary pediatric center. A retrospective review was performed of fundoscopically-diagnosed cases of MGDA, that had been referred for MRI. All MRI studies were scrutinized for orbital and other intracranial abnormalities known to occur in association with MGDA. 18 of 19 cases of MGDA showed three characteristic MRI findings: funnel-shaped morphology of the posterior optic disc, abnormal soft tissue associated with the retrobulbar optic nerve, and effacement of adjacent subarachnoid spaces. The ipsilateral (intraorbital) optic nerve was larger in one patient and smaller in six. The ipsilateral optic chiasm was larger in two patients and smaller in one. This study represents a comprehensive radiological-led investigation into MGDA. It describes the most frequently-encountered MRI findings in MGDA and emphasizes the importance of MRI in this cohort, i.e., in distinguishing MGDA from other posterior globe abnormalities, in assessing the visual pathway, and in screening for associated intracranial abnormalities - skull base/cerebral, vascular, and facial. It hypothesizes neurocristopathy as an underlying cause of MGDA and its associations. Caliber abnormalities of the ipsilateral optic nerve and chiasm are a frequent finding in MGDA. Optic pathway enlargement should not be labeled "glioma". (239/250).

Correlation between extraocular muscle enlargement and thyroid autoantibodies in thyroid eye disease

PurposeThis study aimed to investigate the factors affecting extraocular muscle enlargement in thyroid eye disease (TED).Study designRetrospective study.MethodsThe thyroid-stimulating hormone (TSH) receptor antibody (TRAb), thyroid-stimulating antibody (TSAb), antithyroid peroxidase antibody (ATPO), and antithyroglobulin antibody (ATG) levels in patients diagnosed with TED who underwent orbital magnetic resonance imaging were assessed. The control group comprised the contralateral eye of patients who underwent orbital magnetic resonance imaging (MRI) for unilateral eyelid tumors or orbital disease. The thickness of the bilateral rectus muscles and superior oblique muscles was measured on orbital MRI. Muscle enlargement was classified as unilateral/bilateral and symmetric/asymmetric. The effects of age, sex, smoking history, TSH, thyroid hormone, and thyroid autoantibodies on the muscle thickness and number of enlarged muscles were assessed by use of simple and multiple regression analyses.ResultsThe TED and control groups comprised 41 and 44 cases, respectively. The positivity rate of TSAb in patients with TED was 92.7% higher than that of the other autoantibodies. Muscle enlargement was observed in 29 of the 41 cases (70.7%). Older age and higher TSAb levels were identified as significant factors affecting the total muscle thickness and number of enlarged muscles. Bilateral muscle enlargement and asymmetrical muscle enlargement were observed in 17 (58.6%) and 23 (79.3%) of the 29 cases, respectively. The TSAb levels and age had no significant effect on the type of muscle enlargement.ConclusionsTSAb showed significant associations with extraocular muscle enlargement. Measurement of TSAb, rather than of TRAb, may be more useful for diagnosing extraocular muscle enlargement in patients with TED.

Clinical features of COVID-19-related optic neuritis: a retrospective study.

This retrospective study aimed to investigate the clinical features of optic neuritis associated with COVID-19 (COVID-19 ON), comparing them with neuromyelitis optica-associated optic neuritis (NMO-ON), myelin oligodendrocyte glycoprotein-associated optic neuritis (MOG-ON), and antibody-negative optic neuritis (antibody-negative ON). Data from 117 patients (145 eyes) with optic neuritis at the Shantou International Eye Center (March 2020-June 2023) were categorized into four groups based on etiology: Group 1 (neuromyelitis optica-related optic neuritis, NMO-ON), Group 2 (myelin oligodendrocyte glycoprotein optic neuritis, MOG-ON), Group 3 (antibody-negative optic neuritis, antibody-negative ON), and Group 4 (optic neuritis associated with COVID-19, COVID-19 ON). Characteristics of T2 and enhancement in orbital magnetic resonance imaging (MRI) were assessed. Best-corrected visual acuity (BCVA) was compared before treatment, at a short-term follow-up (14 days), and at the last follow-up after treatment. The COVID-19-associated optic neuritis (COVID-19 ON) group exhibited 100% bilateral involvement, significantly surpassing other groups (P < 0.001). Optic disk edema was observed in 100% of COVID-19 ON cases, markedly differing from neuromyelitis optica-related optic neuritis (NMO-ON) (P = 0.023). Orbital magnetic resonance imaging (MRI) revealed distinctive long-segment lesions without intracranial involvement in T1-enhanced sequences for the COVID-19 ON group compared to the other three groups (P < 0.001). Discrepancies in optic nerve sheath involvement were noted between the COVID-19 ON group and both NMO-ON and antibody-negative optic neuritis (antibody-negative ON) groups (P = 0.028). Before treatment, no significant difference in best-corrected visual acuity (BCVA) existed between the COVID-19 ON group and other groups. At the 14-day follow-up, BCVA in the COVID-19 ON group outperformed the NMO-ON (P < 0.001) and antibody-negative ON (P = 0.028) groups, with no significant difference observed compared to the myelin oligodendrocyte glycoprotein optic neuritis (MOG-ON) group. At the last follow-up after treatment, BCVA in the COVID-19 ON group significantly differed from the NMO-ON group (P < 0.001). Optic neuritis associated with COVID-19 (COVID-19 ON) predominantly presents with bilateral onset and optic disk edema. Orbital magnetic resonance imaging (MRI) demonstrates that COVID-19 ON presents as long-segment enhancement without the involvement of the intracranial segment of the optic nerve in T1-enhanced images. Glucocorticoid therapy showed positive outcomes.

Normative measurements of orbital structures on magnetic resonance images; a cross-sectional study with mini review of the literature.

Awareness of normative values of extra orbital structures would provide useful information to interpret the radiological images better and use them for diagnostic purposes. This study aimed to reveal the average values of major extraocular structures measured on magnetic resonance images. In this retrospective cross-sectional study, magnetic resonance (MR) images of 256 orbits of 128 patients were re-interpreted regarding the measurements of major orbital structures. Extraocular muscles, superior ophthalmic vein, and optic nerve-sheath complex were measured on orbital MR images of these patients. The data distributions were presented by box-plot analyses for each parameter, and the measurement results were analyzed regarding gender and age groups. Lateral rectus muscle thickness (LR), inferior rectus muscle thickness (IR), globe position (GP), and interzygomatic line (IZL) values were higher in the male group than in the female group (p values were < 0.001, 0.003, 0.020, and < 0.001 respectively). LR, the thickness of the superior group muscles (SUP GR), IR, superior oblique muscle thickness (SOBL), and the thickness of optic nerve-sheath complex (ON) values indicated a significant relationship between age groups. There was a significant, positive, and low-level correlation between age and LR, SUP GR, and IR values (p values were < 0.001, 0.001, and < 0.001, respectively). This study provides quantitative data on normative values of orbital structures with gender and age group comparisons. Clinicians or surgeons can easily use the measured values to gather diagnostic information from the orbital region.

New-Onset Vision Impairment in Children: Magnetic Resonance Imaging Findings by Age Groups.

Non-traumatic visual impairment is rare in the pediatric population, but early diagnosis and treatment of the cause is crucial to prevent long-term consequences affecting children's neurocognitive development. The authors aim to determine the most common causes of non-traumatic visual impairment in pediatric patients according to age groups by magnetic resonance imaging (MRI). Images of patients who underwent contrast-enhanced cranial and orbital MRI for new-onset visual impairment between June 2019 and June 2022 were retrospectively reviewed. MRI findings were categorized as tumors, idiopathic intracranial hypertension, demyelinating disorders, infections, isolated optic neuritis, and others. The patients were grouped according to age as preschoolers, schoolchildren, and adolescents. Demographic features of patients and MRI findings were collected and compared among age groups. One hundred seventeen of the 238 patients had pathologic MRI findings. The most common pathologies were tumors (26.4%), idiopathic intracranial hypertension (24.7%), demyelinating disorders (18.8%), infections (11.1%), and isolated optic neuritis (7.6%). Tumors (69.2%) in preschool children, idiopathic intracranial hypertension (36.3%) in schoolchildren, and demyelinating disorders (32.7%) in adolescents were the most common cause of vision impairment by age group. Children with acute vision impairment could have severe pathologies. Tumors in preschool children, idiopathic intracranial hypertension in schoolchildren, and demyelinating disorders in adolescents were the most common causes of new-onset vision impairment detected with MRI. Because of the difficulty of performing optimal ophthalmologic and neurologic examinations, especially in young children, cranial and orbital MRI should be considered to detect life-threatening pathologies.

Clinical Challenge: Looking beyond blurred margins

A 60-year-old man presented to an outside ophthalmology clinic with 1 month of progressive vision loss in the right eye (OD). Right optic disc edema was noted. Brain and orbit magnetic resonance imaging revealed right optic nerve and left occipital lobe enhancement. He was seen initially by neurology and neurosurgery and subsequently referred to neuro-ophthalmology for consideration of optic nerve biopsy. He was seen 3 months after his initial symptom onset where vision was light perception OD and a relative afferent pupillary defect with optic nerve edema. OS was unremarkable. A lumbar puncture with flow cytometry was negative for multiple sclerosis and lymphoma. At his oculoplastic evaluation for optic nerve biopsy, his vision was noted to be no light perception OD. Optic nerve biopsy demonstrated non-caseating granulomatous inflammation. The patient was started on high-dose oral steroids with improvement of disc edema, as well as significant improvement in optic nerve and intracranial parenchymal enhancement, although his vision never improved.

DIRECT CAROTID CAVERNOSUS FISTULA

Abstract&#x0D; Introduction : Carotid cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system, most commonly due to head trauma from a basal skull fracture creating a tear in the internal carotid artery (ICA) within the cavernous sinus.&#x0D; Case Illustration : A 15 years old girl presented with orbital pain, swelling, swishing or buzzing sounds, headache, and vision loss. Five months ago, she had a history of traumatic injury and undergone craniotomy. From physical examination Orbital ultrasound presence with dilated superior ophthalmic vein, which appears as a hollow tubular structure with no internal echo. MRI scan show a mass suggestive carotid cavernosus fistula dextra. DSA result confirmed the diagnose as direct carotid cavernosus fistula dextra as communication between internal carotid artery and sinus cavernosus. The patient was treated with neuroprotective along medication followed by endovascular treatment from the neurointervention department.&#x0D; Discussion : The classic triad of CCF includes pulsatile exophthalmos, chemosis and orbital bruit. Ophthalmic manifestations of CCFs vary widely depending on underlying aetiology, type, size, location, blood flow rate and drainage route of the CCF. Patient initially undergo noninvasive imaging with orbital ultrasound and magnetic resonance imaging (MRI). Digital subtraction angiography is the gold standard in the diagnosis of CCF and must be performed before any potential intervention.&#x0D; Conclusion : This case describes a patient who was diagnose with direct carotid cavernous fistula of the right eye.

Magnetic resonance imaging findings of high myopic strabismus with sagging eye-like symptoms, heavy eye syndrome, and non-highly myopic eyes with sagging eye syndrome

ABSTRACT Introduction Progressive esotropia accompanied by restricted abduction and supraduction due to high myopia is known as esotropia fixus with high myopia or heavy eye syndrome (HES). Some conditions, such as sagging eye syndrome (SES), show esotropia for distance or cyclovertical strabismus with no abduction limitations despite highly myopic eyes. We evaluated the magnetic resonance imaging (MRI) findings and clinical features of HES, high myopia with SES-like symptoms (highly myopic SES), and SES. Methods We reviewed all patients diagnosed with HES, highly myopic SES, and SES who underwent MRI of the orbits and brain over 6 years. To quantitatively assess the orbital anatomy, we compared the conditions of the superior rectus muscle (SR), lateral rectus muscle (LR), and inferior rectus muscle (IR) using orbital MRI among the three groups. Results Among the 14 patients (27 eyes) with high myopia, 5 (9 eyes) had HES, and 9 (18 eyes) had highly myopic SES. Eleven patients (22 eyes) with SES were also compared with these 14 patients. The mean axial length was 29.6 ± 1.0 mm in participants with HES, 29.0 ± 1.5 mm in those with HES-SES, and 23.7 ± 0.9 mm in those with SES. The average distance esotropia was 48.0 ± 19.9Δprism, 4.6 ± 1.5Δprism, and 6.1 ± 4.6Δprism for participants with HES, highly myopic SES and SES, respectively. The average distance hypertropia was 5.3 ± 5.9Δprism in participants with highly myopic SES and 4.8 ± 2.7Δprism in those with SES. The mean vertical angle of the LR was 32.6 ± 10.8°, 18.1 ± 5.4°, and 14.6 ± 6.8°; the mean tilting angle of the LR was 31.6 ± 9.2°, 15.9 ± 6.0°, and 13.8 ± 5.9°; and the mean displacement angle between the LR and SR was 152.3 ± 16.7°, 125.0 ± 7.1°, and 112.5 ± 7.5° for participants with HES, highly myopic SES and SES, respectively. The LR-SR displacement angle in HES-SES was significantly larger than in SES (p < .001) but the vertical and tilting angles were not. Also, the IR shift showed no significant difference with HES-SES and HES (5.8 ± 1.4 mm and 5.3 ± 1.2 mm) but not with SES (4.0 ± 0.8 mm) (p < .0001). Discussion SES-like symptoms can develop in highly myopic eyes; however, MRI showed that the state of the LR muscle in highly myopic SES deviated almost similarly to that in SES; however, the eyeball was more dislocated than in SES. This may be useful in deciding the appropriate operative procedure.

Radiological measurements of lacrimal gland in thyroid eye disease

PurposeLacrimal gland enlargement is a common feature of thyroid eye disease (TED) and has been positively correlated with the clinical activity score. Although lacrimal gland volume is the preferred measure of lacrimal gland size, its calculation is not clinically translatable due to the expertise, time and advanced software required. The aim of our study is to determine whether the lacrimal gland volume in patients with TED undergoing magnetic resonance imaging (MRI) can be estimated using simpler lacrimal gland linear and area measurements.MethodsA retrospective review of 102 orbits (51 patients) with TED who underwent orbital MRI was conducted. The maximum length, width, and area of the lacrimal gland were measured in axial and coronal sections. Lacrimal gland volume was calculated by using a manual segmentation technique on all consecutive axial slices on commercially available software, OsiriX. All quantitative measurements were correlated with the lacrimal gland volume.ResultsMean age of participants was 59 ± 16 years, and 67% (n = 34) were females. With multivariate analyses, combined lacrimal gland axial and coronal areas strongly correlated with volume (r = 0.843, p < 0.01). Strong univariate predictors of volume included axial area (r = 0.704, p < 0.01) and coronal area (r = 0.722, p < 0.01), while moderate predictors included axial length (r = 0.523, p < 0.01), axial width (r = 0.521, p < 0.01), coronal length (r = 0.450, p < 0.01), and coronal width (r = 0.649, p < 0.01).ConclusionIn patients with thyroid eye disease, lacrimal gland volume can be estimated using axial and coronal areas, which is simpler and more time efficient than calculating volumes.

A case of bilateral amyloidosis localized to extraocular muscles mimicking thyroid eye disease

BackgroundAmyloidosis is a rare condition characterized by the abnormal deposition of amyloid proteins in various tissues and organs. While systemic amyloidosis has been well-documented, amyloid deposition in extraocular muscles is an exceptionally rare occurrence, with only 35 reported cases. This case report sheds light on the importance of considering amyloidosis in the differential diagnosis of patients presenting with proptosis and diplopia, which are often associated with thyroid eye disease.Case presentationA woman in her twenties sought medical attention due to a complaint of diplopia. Her ocular examination revealed almost normal findings except for exotropia and proptosis. Orbital magnetic resonance imaging displayed fusiform enlargement of nearly all eye muscles, a presentation typically observed in thyroid eye disease. However, despite corticosteroid therapy, her symptoms showed no improvement. Given the unusual lack of response to conventional treatment, and inhomogeneous enhancement of the muscle, an extraocular muscle biopsy was conducted. This biopsy yielded a unique finding—amyloid deposition within the muscle tissue. This discovery was particularly intriguing due to the extreme rarity of amyloidosis affecting extraocular muscles, with fewer than three dozen documented cases worldwide.ConclusionThis unique case underscores the critical need for a comprehensive approach to diagnosing patients with proptosis and diplopia. While these symptoms are commonly attributed to thyroid eye disease, it is essential to consider alternative diagnoses such as amyloidosis, especially when standard treatments fail to yield results. The discovery of amyloid deposition in the extraocular muscles, although exceedingly rare, emphasizes the significance of a thorough differential diagnosis. In conclusion, this case report highlights the importance of vigilance in clinical practice, encouraging ophthalmologists to explore less common diagnostic possibilities when faced with challenging cases. Further research and clinical investigation are warranted to better understand the mechanisms and potential treatments for amyloidosis affecting the extraocular muscles.

Combined Orbital and Cranial Vessel Wall Magnetic Resonance Imaging for the Assessment of Disease Activity in Giant Cell Arteritis.

Acute visual impairment is the most feared complication of giant cell arteritis (GCA) but is challenging to predict. Magnetic resonance imaging (MRI) evaluates orbital pathology not visualized by an ophthalmologic examination. This study combined orbital and cranial vessel wall MRI to assess both orbital and cranial disease activity in patients with GCA, including patients without visual symptoms. Patients with suspected active GCA who underwent orbital and cranial vessel wall MRI were included. In 14 patients, repeat imaging over 12 months assessed sensitivity to change. Clinical diagnosis of ocular or nonocular GCA was determined by a rheumatologist and/or ophthalmologist. A radiologist masked to clinical data scored MRI enhancement of structures. Sixty-four patients with suspected GCA were included: 25 (39%) received a clinical diagnosis of GCA, including 12 (19%) with ocular GCA. Orbital MRI enhancement was observed in 83% of patients with ocular GCA, 38% of patients with nonocular GCA, and 5% of patients with non-GCA. MRI had strong diagnostic performance for both any GCA and ocular GCA. Combining MRI with a funduscopic examination reached 100% sensitivity for ocular GCA. MRI enhancement significantly decreased after treatment (P < 0.01). In GCA, MRI is a sensitive tool that comprehensively evaluates multiple cranial structures, including the orbits, which are the most concerning site of pathology. Orbital enhancement in patients without visual symptoms suggests that MRI may detect at-risk subclinical ocular disease in GCA. MRI scores decreased following treatment, suggesting scores reflect inflammation. Future studies are needed to determine if MRI can identify patients at low risk for blindness who may receive less glucocorticoid therapy.

Predicting glucocorticoid effectiveness in thyroid eye disease: combined value from serological lipid metabolism and an orbital MRI parameter.

The aim was to determine the combined value of serological lipid metabolism and an orbital MRI quantitative parameter in predicting the effectiveness of glucocorticoid (GC) therapy in patients with thyroid eye disease (TED). This study retrospectively enrolled 46 patients with active and moderate-to-severe TED (GC-effective group, n = 29; GC-ineffective group, n = 17). Serological lipid metabolism, the orbital MRI-based minimum signal intensity ratio of extraocular muscles (EOM-SIRmin), as well as other clinical parameters before GC therapy were collected and compared between the two groups. Multivariate logistic regression and receiver operating characteristic curve analysis were adopted to identify independent predictable variables and assess their predictive performances. Compared to the GC-ineffective group, the GC-effective group showed lower serum total cholesterol levels (P = 0.006), lower serum low-density lipoprotein cholesterol levels (P = 0.019), higher EOM-SIRmin values (P = 0.005), and shorter disease durations (P = 0.017). Serum total cholesterol and EOM-SIRmin were found to be independent predictors of GC-effective TED through multivariate analysis (odds ratios = 0.253 and 2.036 per 0.1 units, respectively) (both P < 0.05). The integration of serum total cholesterol ≤4.8 mmol/L and EOM-SIRmin ≥ 1.12 had a better predictive efficacy (area under the curve, 0.834) than EOM-SIRmin alone, with a sensitivity of 75.9% and a specificity of 82.4% (P = 0.031). Serological lipid metabolism, combined with an orbital MRI-derived parameter, was a useful marker for predicting the effectiveness of GCs in patients with active and moderate-to-severe TED.

A Case of Retinal Vasculitis, Uveitis in Myelin Oligodendrocyte Glycoprotein Antibody Associated Optic Neuritis

Purpose: To report a case of recurrent optic neuritis with uveitis in a patient with myelin oligodendrocyte glycoprotein (MOG)- associated disease.Case summary: A 16-year-old female presented with left eye hyperemia, glare, and headache. The best corrected visual acuity was 1.2 in both eyes with a left relative afferent pupillary defect. Cells in the anterior chamber, optic disc edema, and subretinal fluid were present, and the distal optic nerve was enhanced on orbital magnetic resonance imaging. Although it improved with steroid treatment, it recurred 3 times in 9 months. At the second recurrence, her visual acuity was reduced to light perception, but she responded well to steroids. During the third recurrence, she visited our hospital for the first time and improved with steroid treatment. However, 18 months after treatment, optic neuritis, uveitis, and macular edema occurred, so oral steroids and eye drops were maintained. Serum anti-MOG antibody came out positive, and at the last visit, visual acuity of 1.2 in the left eye and visual function were maintained well.Conclusions: MOG antibody disease (MOGAD) frequently recurs and invades various structures of the eye and various clinical manifestations have been reported. Therefore, if optic neuritis is accompanied by inflammation of the anterior chamber and retinal vessels, the possibility of MOGAD should be considered.

Unilateral Idiopathic Hemolacria: A Case Report

Purpose: To present a case of a patient diagnosed with idiopathic hemolacria.Case Summary: A healthy 19-year-old man without pre-existing medical conditions presented to the hospital for treatment of intermittent bloody tears from his right eye. The patient reported experiencing bloody tears in his right eye approximately once per month during stressful events for the preceding 6 years. These episodes were associated with ocular pain, right-eye hyperemia, headaches, and nausea. However, these symptoms of bloody tears spontaneously resolved without intervention. Corrected visual acuity in the right eye was 1.0, and the intraocular pressure was 16 mmHg. There were no abnormalities in the ocular appendages, anterior chamber, or eyelids. Nasolacrimal duct probing and irrigation, as well as fundus examination, revealed normal findings. Brain and orbital magnetic resonance imaging did not show abnormalities in the brain or the orbital region, although slight mucosal thickening was present around the right lacrimal gland. Other examinations, including an electrocardiogram, chest X-ray, blood test, and nasal endoscopy, all revealed normal findings.Conclusions: Recurrent bloody tears (hemolacria) occurred in the right eye of a 19-year-old man with no history of trauma, intraocular surgery, or underlying medical conditions. Comprehensive ophthalmic examination and brain and orbital magnetic resonance imaging failed to identify any specific abnormalities leading to a diagnosis of idiopathic hemolacria.

Whole-orbit radiomics: machine learning-based multi- and fused- region radiomics signatures for intravenous glucocorticoid response prediction in thyroid eye disease

BackgroundRadiomics analysis of orbital magnetic resonance imaging (MRI) shows preliminary potential for intravenous glucocorticoid (IVGC) response prediction of thyroid eye disease (TED). The current region of interest segmentation contains only a single organ as extraocular muscles (EOMs). It would be of great value to consider all orbital soft tissues and construct a better prediction model.MethodsIn this retrospective study, we enrolled 127 patients with TED that received 4·5 g IVGC therapy and had complete follow-up examinations. Pre-treatment orbital T2-weighted imaging (T2WI) was acquired for all subjects. Using multi-organ segmentation (MOS) strategy, we contoured the EOMs, lacrimal gland (LG), orbital fat (OF), and optic nerve (ON), respectively. By fused-organ segmentation (FOS), we contoured the aforementioned structures as a cohesive unit. Whole-orbit radiomics (WOR) models consisting of a multi-regional radiomics (MRR) model and a fused-regional radiomics (FRR) model were further constructed using six machine learning (ML) algorithms.ResultsThe support vector machine (SVM) classifier had the best performance on the MRR model (AUC = 0·961). The MRR model outperformed the single-regional radiomics (SRR) models (highest AUC = 0·766, XGBoost on EOMs, or LR on OF) and conventional semiquantitative imaging model (highest AUC = 0·760, NaiveBayes). The application of different ML algorithms for the comparison between the MRR model and the FRR model (highest AUC = 0·916, LR) led to different conclusions.ConclusionsThe WOR models achieved a satisfactory result in IVGC response prediction of TED. It would be beneficial to include more orbital structures and implement ML algorithms while constructing radiomics models. The selection of separate or overall segmentation of orbital soft tissues has not yet attained its final optimal result.

Rapidly progressive neuromyelitis optica, report of a case

Aims/Purpose: To describe a clinical case of rapidly sequential bilateral optic neuritis associated with positive serological antibodies against myelin oligodendrocyte glycoprotein (MOG) in a 54‐year‐old Moroccan woman, who present loss of visual acuity in her left eye (OS) of 1 week of evolution.Methods: In the ophthalmological examination, she presented a visual acuity (VA) of 4/10 in the right eye (RE) and counting fingers at 1 meter in OS, with an afferent pupillary defect in OS. The ocular fundus shows papilledema in OS, with bilateral retinal nerve fibre layer (RNFL) thickening by optical coherence tomography (OCT). Rest of exploration normal. A complete analytical study and magnetic resonance imaging (MRI) are requested. While waiting for results, she returned to the emergency room due to greater visual loss, showing VA of hand movement in OD and perception of light in OS. OCT showed increased RNFL thickening and fluorescein angiography showed papillary hyperfluorescence. The visual field revealed severe generalized depression in both eyes.Results: Brain and orbital MRI showed bilateral enhancement of the anterior optic nerves, with extension of enhancement to their intracranial portion, chiasm, and right optic tract. The analytical study showed a positive result for MOG antibodies with a titre of 1:80. We proceeded to intravenous treatment with methylprednisolone 1 gd/day for 5 days followed by oral corticosteroids and plasmapheresis. The patient presented a remarkable clinical improvement with VA improvement to 6/10 in both eyes, disappearance of optic nerve edema, and normalization of RNFL.Conclusions: The discovery of MOG‐Immunoglobulin G as a biomarker for inflammatory demyelinating diseases of the Central Nervous System other than Multiple Sclerosis represents a paradigm shift in the field of neuroimmunology. Myelin oligodendrocyte glycoprotein‐associated disorder has the potential to cause devastating long‐term visual impairment.

Immune-related visual dysfunction in thyroid eye disease: a combined orbital and brain neuroimaging study.

To investigate the pathological interplay between immunity and the visual processing system (VPS) in thyroid eye disease (TED). A total of 24 active patients (AP), 26 inactive patients (IP) of TED, and 27 healthy controls (HCs) were enrolled. Orbital magnetic resonance imaging (MRI) and resting-state functional MRI (rs-fMRI) were conducted for each participant. Multiple MRI parameters of the intraorbital optic nerve (ON) were assessed. The amplitude of low-frequency fluctuations (ALFF) and regional homogeneity (ReHo) were calculated. Correlation analyses were carried out on the above parameters and clinical characteristics. Visual functioning scores differentiated between the AP and IP groups. The ON subarachnoid space and ON sheath diameter were significantly higher in AP than in IP. Six vision-related brain regions were identified in TED patients compared with HCs, including right calcarine (CAL.R), right cuneus (CUN.R), left postcentral gyrus (PoCG.L), right middle temporal gyrus (MTG.R), left superior frontal gyrus (SFG.L), and left caudate (CAU.L). The brain activity of MTG.R, SFG.L, and CAU.L differentiated between the AP and IP groups. The correlation analysis revealed a close association among the vision-related brain regions, MRI parameters of ON, and clinical characteristics in AP and IP, respectively. Combined orbital and brain neuroimaging revealed abnormalities of the VPS in TED, which had a close correlation with immune statuses. Vision-related brain regions in TED might be possibly altered by peripheral immunity via a direct or indirect approach. The discovery of this study explained the disparity of visual dysfunction in TED patients with different immune statuses. With the uncovered neuroimaging markers, early detection and intervention of visual dysfunction could be achieved and potentially benefit TED patients. • Patients with different immune statuses of thyroid eye disease varied in the presentation of visual dysfunction. • The combined orbital and brain neuroimaging study identified six altered vision-related brain regions, which had a significant correlation with the MRI parameters of the intraorbital optic nerve and immunological characteristics. • Peripheral immunity might possibly give rise to alterations in the central nervous system part of the visual processing system via a direct or indirect approach.

Retinal Changes in a Patient with a Traumatic Orbital Subperiosteal Hematoma and Superior Ophthalmic Vein Compression

Purpose: We describe a case with retinal alterations similar to those of nonischemic central retinal vein occlusion, but also with superior ophthalmic vein compression attributable to a traumatic, subperiosteal orbital hematoma.Case summary: A 13-year-old male presented with left periorbital edema, a decrease in vision, and diplopia after blunt periorbital trauma. In ophthalmological evaluations, the best-corrected visual acuity (BCVA) was 0.4 (20/50) and a severe supraduction limitation was apparent (grade -4). Fundus examination revealed optic disc swelling, retinal vein dilation/tortuosity, and an arteriovenous transit time delay of 26s. Fluorescein angiography evidenced optic disc leakage. Ocular sonography and orbital magnetic resonance imaging revealed a superior subperiosteal hematoma in the left orbit accompanied by superior ophthalmic vein compression. We scheduled emergency hematoma evacuation. One month later, the BCVA had increased to 1.0 (20/20) and ocular movement was no longer limited. Orbital computed tomography showed that the superior ophthalmic vein compression had resolved and that the abnormalities observed in the initial fundus examination and fluorescein angiography had improved.Conclusions: Retinal changes that resemble nonischemic central retinal vein occlusion including retinal venous dilation and tortuosity may develop after a traumatic, orbital subperiosteal hematoma. Compressive obstruction of the superior ophthalmic vein may then be in play. Early surgical intervention featuring hematoma evacuation may prevent irreversible visual deterioration.

A Case of Orbital Infarction Syndrome in a Patient with Hemophagocytic Lymphohistiocytosis

Purpose: To report a case of orbital infarction syndrome in a patient with hemophagocytic lymphohistiocytosis (HLH).Case summary: A 70-year-old woman with diabetes mellitus and hypertension was referred to the Department of Ophthalmology for sudden-onset left upper eyelid ptosis after being diagnosed with HLH. After 3 days, the best corrected visual acuity in the right eye was 0.8, while there was no light perception in the left eye. In the left eye, the ocular motility examination showed limitations in all fields of gaze. On fundus examination, optic disc pallor, retinal hemorrhage, and narrowed retinal arteries were observed in the left eye. Fluorescein angiography showed no blood flow in the retinal arteries and veins in the left eye. On neck computed tomography angiography, the left distal internal carotid artery was narrowed. Orbit computed tomography showed exophthalmos and extraocular muscle hypertrophy in the left eye. Orbit magnetic resonance imaging confirmed optic nerve edema, enhancement of the optic nerve sheath, and high signal intensity of the intraocular fat in the left eye. Slit-lamp examination revealed ischemia of the anterior segment and ischemic necrosis of the eyelid in the left eye. The patient was diagnosed with orbital infarction syndrome.Conclusions: Several factors may lead to ischemia of the orbital tissues in patients with HLH, including coagulopathy, an increased incidence of blood clots, and inflammation in the orbit. It is necessary to consider the possibility of orbital infarction syndrome when HLH patients present with visual loss, ocular movement limitations, and anterior segment ischemia.