Extraocular Muscle Enlargement Research Articles

Subcutaneous Injections of Triamcinolone Acetonide for Upper Eyelid Retraction and Swelling Associated with Thyroid Eye Disease: A Retrospective Case Series Study.

To evaluate the efficacy of subcutaneous injection of triamcinolone acetonide (SCTA) in treating upper eyelid retraction and swelling in patients with thyroid eye disease (TED). This case series included consecutive patients (aged 16-69 years, monitored from June 2012 to December 2015) with TED-related eyelid symptom and without an enlarged extraocular muscle on magnetic resonance imaging (MRI). SCTA (0.5 mL, 40 mg/mL) was administered to target the orbital fat around the levator palpebrae superioris (LPS) muscle. Patients who did not exhibit improvement after the first trial received an additional injection. Follow-up was conducted for 12 months with 3-month intervals. Eyelid retraction, eyelid swelling, and eyelid lag were evaluated at each follow-up visit. In total, 116 eyelids of 102 patients were analyzed. SCTA led to significant improvement in 93% of eyes (108/116), disappearance of eyelid symptoms (74%, 87%, and 73% in retraction, swelling, and lag, respectively), and improvement of scores (from 1.64 to 0.12, 1.32 to 0.26, and 1.72 to 0.30, respectively). Improvement in eyelid symptoms was observed in eight eyes; however, additional steroid therapy was required in these cases due to the emergence of other extraocular muscle inflammation. Additional injection was required in 39.8% of patients. The clinical activity score was lower in the single SCTA group than in the multiple SCTA group (1.5 vs 0.9; p < 0.01). However, the levels of thyroid-stimulating hormone receptor antibody and MRI findings were not significantly different between the two groups. No elevation in intraocular pressure was observed. Eight female patients experienced menstrual disorder. SCTA effectively reduced LPS muscle enlargement and fat tissue swelling in patients with TED. A single SCTA was sufficient in almost 60% of the patients; nevertheless, follow-up is necessary to detect early signs of orbital inflammation even in eyelid-symptom-improved patients.

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.

Magnetic Resonance Imaging of Idiopathic Orbital Myositis.

To characterize the qualitative and quantitative features of idiopathic orbital myositis (IOM) on MRI. This was a multicenter retrospective study of patients with active IOM with MRI. Patients with incomplete clinical records, poor-quality or interval scans without active myositis, and specific orbital myositis (i.e., orbital myositis secondary to an identified condition) were excluded. An enlargement ratio was calculated by dividing the diameters of the affected extraocular muscle (EOM) by the contralateral unaffected EOM. Twenty-four patients (mean age: 44.4 ± 17.8 years-old, male: 11) between 2011 and 2022 were included. One case (4.2%) was pediatric (17 years old), and 6 cases presented with recurrence. Active IOM was characterized by fusiform EOM enlargement, high T2 signal, and contrast enhancement. Average maximal EOM diameters ranged from 4.6 to 7.7 mm (enlargement ratio: 1.4-2.2). Eighteen (75%) patients had single EOM involvement, most commonly the medial rectus. Other ipsilateral structures affected included focal orbital fat (16/24, 66.7%) and lacrimal gland (8/24, 33.3%). Contralateral changes in the EOM and/or lacrimal gland were observed in 7 patients (29.2%). Patients presenting with recurrence were likely to develop ongoing recurrent episodes (p = 0.003). Various radiological patterns of involvement described including EOM enlargement, contrast enhancement, abnormal signal, and involvement of other orbital structures are indicative of active IOM. IOM remains a heterogeneous spectrum of acute and chronic clinico-radiological presentations. Inflammation may involve other ipsilateral or contralateral orbital structures or may be bilateral despite presenting clinically as unilateral disease. Quantitative measurements may have utility in differentiating IOM from other causes of orbital myositis.

Review article: Diagnosis and management of enlarged extraocular muscles.

Extraocular muscle (EOM) enlargement may be due to a variety of causes. These causes can be classified in three ways: according to pathogenesis and histopathological features, according to the site, and according to the clinical features. Diagnosis of the cause is dependent upon history, clinical examination, and investigations. Imaging with computed tomography or magnetic resonance imaging and muscle biopsy is typically necessary to make the correct diagnosis. Treatment of the patient must be directed toward the specific cause. This review emphasizes important clinical and pathological guidelines for appropriate diagnosis and treatment of patients with EOM enlargement.

Carotid Cavernous Fistula – Radiological Features

Carotid cavernous fistula (CCF) is an abnormal vascular shunt from the carotid artery to the cavernous sinus. The characteristic clinical features include chemosis, proptosis, ocular bruit, headache, diplopia, and ophthalmoplegia. The classical radiological features on magnetic resonance imaging (MRI) include proptosis, extraocular muscle enlargement, dilatation of the superior ophthalmic vein, and ipsilateral cavernous sinus enlargement. We report the case of a 66-year-old female who presented with headache, redness of the right eye, and double vision, who had features of CCF on MRI.

Effects of various extraocular muscle enlargement patterns on muscle diameter index in graves ophthalmopathy patients: a retrospective cohort study

Graves ophthalmopathy (GO) patients often undergo retrobulbar injection of glucocorticoids (GCs) as a common therapeutic approach. This study aimed to explore the impact of various patterns of extraocular muscle (EOM) enlargement on EOM changes following retrobulbar GCs injection in patients with GO. A retrospective analysis was conducted on GO patients who underwent retrobulbar GCs injections. Data pertaining to EOM diameter (EMD) and muscle diameter index (MDI) were collected from orbital computed tomography (CT) scans. The MDI change (ΔMDI) was calculated by comparing pre- and post-injection MDI values. The relationship between each pre EMD/MDI and ΔMDI was assessed using univariate and multivariate logistic regression analysis. A total of 68 patients with GO were included in this study, accounting for 118 eyes. After retrobulbar injections of GCs, 84 eyes showed a decrease in the MDI, while 34 eyes exhibited an increase in MDI. A threshold effect was observed in the relationship between medial pre EMD/MDI and ΔMDI. When the medial pre EMD/MDI was less than 0.28, a higher medial pre EMD/MDI was associated with a smaller ΔMDI (β = − 25.21, p = 0.0175). However, when the medial pre EMD/MDI was greater than 0.28, no significant association was found between pre EMD/MDI and ΔMDI. There was a negative correlation between medial + lateral pre EMD/MDI and ΔMDI (β = − 11.76, p < 0.0189). A higher medial + lateral pre EMD/MDI was associated with a greater decrease in MDI. Additionally, there was a positive correlation between superior rectus muscle-levator complex (SRLC) pre EMD/MDI and ΔMDI (β = 11.92, p = 0.040). The higher the value of SRLC pre EMD/MDI, the greater the ΔMDI. There was an association between pre EMD/MDI and changes in EOMs after retrobulbar injection of GCs in GO patients. In patients with predominantly enlarged medial rectus muscles and severe degrees of enlargement, retrobulbar injection of GCs should be assessed for its benefit; a combination of medial and lateral rectus muscle enlargement is beneficial for the shrinkage of EOMs following retrobulbar injections; the involvement of the SRLC rectus muscle may be a disadvantageous pattern of shrinkage of EOMs following retrobulbar injections.Trial registration This study is retrospectively registered. We have registered this study with the Chinese Clinical Trials Registry (www.chictr.org.cn, registration number: ChiCTR2200063429).

Extraocular muscle enlargement and proptosis in carotid cavernous fistulas

ABSTRACT Purpose To evaluate the prevalence and pattern of extraocular muscle enlargement and proptosis in patients with carotid cavernous fistulas (CCF). Methods We conducted a retrospective study on patients with digital subtraction angiography (DSA) confirmed CCFs with neuroimaging (computed tomography or magnetic resonance imaging) performed prior to the DSA. The maximum extraocular muscle diameters were recorded. Extraocular muscles were considered enlarged if they were greater than two standard deviations above the normal muscle diameters. Proptosis was defined as the distance between the interzygomatic line to the anterior globe of ≥2 mm compared to the contralateral orbit or ≥21 mm. Results Forty orbits from 20 patients were included. The mean age of participants was 65 ± 15 years and 13 (65%) were female. Thirteen (65%) fistulas were indirect and seven (35%) were direct. There was enlargement of at least one muscle in 11 (27.5%) orbits, and this was not correlated with the type of fistula (direct/indirect). The inferior rectus was most commonly enlarged in seven orbits (17.5%), followed by the medial rectus in five orbits (12.5%). Proptosis was found in 17 (43%) orbits and was more common ipsilateral to the fistula (58% ipsilateral group vs 19% contralateral group, p < .01). Conclusion Extraocular muscle enlargement was observed in over one-fourth of CCFs. When enlarged, the inferior and medial rectus muscles are most commonly involved. These findings may help clinicians and radiologists when evaluating the CT or MRI scans of patients with suspected CCFs.

A patient with congenitally restrictive strabismus and contralateral secondary muscle enlargement.

To report the case of a congenital restrictive strabismus with a contralateral enlargement of extraocular muscles. The clinical presentation, findings, and postoperative outcomes of a 4 years old boy referred to evaluation for presenting a left eye deviation since birth are detailed. A unilateral congenital restrictive strabismus (congenital hypotropia and esotropia) can be the cause of contralateral secondary enlarged extraocular muscle and It must be included in the differential diagnosis.

Extraocular muscle enlargement of indeterminate cause in pediatric patients: case series and literature review

To specify the clinical characteristics of enlarged extraocular muscles of indeterminate cause in pediatric patients based on a case series and literature review. The medical records of pediatric patients who presented between January 2019 and January 2022 with enlarged extraocular muscles, where the underlying cause could not be determined, were retrospectively reviewed. Four patients were included. The main reason for presentation was evaluation of abnormal head posture. Head tilt or turn with duction deficit was observed in all patients. The age of onset ranged from 6 months to 1 year. Two patients presented with esotropia and hypotropia; the other 2 patients, with large-angle esotropia. Orbital imaging was performed in all cases and revealed unilateral rectus muscle enlargement sparing the muscle tendon. All 4 patients were found to have enlarged medial rectus muscle. In the 2 patients with hypotropia, the inferior rectus muscle was also involved. No underlying systemic or orbital disease was found. There were no changes in the orbit or extraocular muscles on follow-up imaging test. The intraoperative forced duction test revealed severe restriction in the direction of gaze opposite to the primary field of action of the enlarged muscles. Enlargement of extraocular muscles should be considered in the differential diagnosis when large-angle incomitant vertical or horizontal misalignment and abnormal head posture are observed in infancy.

Extraocular Muscle Enlargement in Acromegaly.

To determine whether acromegaly is associated with increased extraocular muscle (EOM) size at time of presentation. Patients with a new diagnosis of acromegaly in a single tertiary care clinic with a CT scan that adequately delineated the EOMs were included. Control subjects were age- and sex-matched patients with a new diagnosis of nonfunctioning pituitary adenoma. Retrospective chart review was performed to extract baseline clinical and laboratory parameters including growth hormone, insulin-like growth factor 1, thyroid stimulating hormone, free T3, and free T4. A single neuroradiologist analyzed all CT scans and measured the maximum diameter and cross-sectional area of the superior rectus, inferior rectus, medial rectus, and lateral rectus in both eyes of all patients. We evaluated 17 patients with acromegaly and 18 control subjects. Mean maximum diameter of the superior, inferior, medial, and lateral recti were 4.80 mm (SD = 0.81), 4.67 mm (SD = 0.54), 4.86 mm (SD = 0.77), and 4.53 mm (SD = 0.70) respectively, in the acromegaly group. In the control group, they were 3.62 mm (SD = 0.58),3.71 mm (SD = 0.46), 3.66 mm (SD = 0.32), and 3.21 mm (SD = 0.44), respectively. The maximum diameter and cross-sectional area of all 4 EOMs measured in the acromegaly group were significantly larger ( P < 0.001) compared with the control group. Patients with acromegaly present with significantly enlarged EOMs compared with control subjects with nonfunctioning pituitary adenomas.

McKittrick-Wheelock syndrome presenting with presumed paraneoplastic syndrome extra-ocular muscle enlargement masquerading as thyroid eye disease.

Paraneoplastic syndromes (PS) are uncommon and are known to mimic other clinical entities, often carrying significant morbidity and mortality. The commonest cause of extra-ocular muscle enlargement (EOME) is thyroid eye disease (TED). Rarely, PS can cause EOME and masquerade as TED. We describe a 52-year-old female who presented with diarrhoea, acute kidney injury and electrolyte imbalance. An ophthalmic review identified right upper lid retraction. MRI orbits showed increased thickness of the inferior and medial recti bilaterally, presumed as TED. Whilst investigating her diarrhoea, imaging revealed a large rectosigmoid tumour which required surgical excision. In the context of electrolyte disturbance and acute kidney injury, a diagnosis of McKittrick-Wheelock syndrome (MWS) was made. Following successful surgery, electrolyte imbalance, diarrhoea and eyelid retraction improved. Repeat MRI orbits displayed complete resolution of EOME. To our knowledge, this is the first case of MWS presenting with PS-EOME masquerading as TED. McKittrick-Wheelock syndrome (MWS) is a rare disorder, although likely under-recognised, which is characterised by diarrhoea, dehydration and electrolyte depletion that results from a hypersecretory colorectal neoplasm. Definitive treatment of MWS involves the resection of the colorectal neoplasm. Bilateral ophthalmopathy that appears to be Graves' ophthalmopathy on imaging, though clinical and biochemical evidence fails to identify a thyroid pathology, has been associated with malignancy on rare occasions. Such patients should be investigated for potential malignant causes of their ophthalmopathy.

Graves' Eye Disease: Clinical and Radiological Diagnosis.

Graves' disease is an autoimmune disorder in which hyperthyroidism results in various systematic symptoms, with about 30% of patients presenting with Graves' eye disease (GED). The majority of patients with GED develop mild symptoms, including eyelid retraction, exposure of the globe, superior rectus-levator muscle complex inflammation, and fat expansion, leading to exophthalmos. More severe cases can result in extraocular muscle enlargement, restricted ocular movement, eyelid and conjunctival edema, and compression of the optic nerve leading to compressive optic neuropathy (CON). GED severity can be classified using the Clinical Activity Score, European Group on Graves' Orbitopathy scale, NO SPECS Classification system, and VISA system. CT and MRI aid in the diagnosis of GED through the demonstration of orbital pathology. Several recent studies have shown that MRI findings correlate with disease severity and can be used to evaluate CON. Mild cases of GED can be self-limiting, and patients often recover spontaneously within 2-5 years. When medical treatment is required, immunomodulators or radiotherapy can be used to limit immunologic damage. Surgery may be needed to improve patient comfort, preserve the orbit, and prevent vision loss from optic nerve compression or breakdown of the cornea.

Commentary: Dysthyroid optic neuropathy: A lurking danger in thyroid eye disease.

Poonam et al., in their retrospective study, highlighted male gender, hyperthyroidism, and smoking as risk factors of developing dysthyroid optic neuropathy (DON) over four years. They highlighted the potential of recurrence of DON in patients with diabetes.[1] DON is the most dreaded complication of thyroid eye disease (TED), warranting an urgent intervention. Fortunately, the occurrence of DON is noted only in 5%–7% of TED cases.[2] The diagnosis of established DON is majorly clinical, but the diagnosis of subclinical DON may need detailed ophthalmic examination and imaging. Patients with subclinical DON may not experience a sudden diminution of vision, abnormal pupillary reflexes, and features of orbital inflammation—classical of severe TED.[2] The risk factors for clinical and subclinical DON include older age, male gender, smoking, diabetes (controlled or uncontrolled), radioisotope therapy, hyperthyroidism or Grave’s ophthalmopathy, and high level of thyroid autoantibodies.[12] The pathogenesis of DON involves mechanical, inflammatory, and vascular factors causing damage to the nerve cells and axons of the optic nerve.[2] TED patients can be classified as type I (predominantly fat enlargement) or type II disease (predominantly extraocular muscle enlargement). In type II TED, the mechanical compression of optic nerve blood supply and axonal flow at the crowded orbital apex occurs due to enlarged extraocular muscles. The reduced arterial supply to the optic nerve and sluggish outflow of the blood from the superior ophthalmic vein comprises of major vascular insults contributing to the DON.[2] Moreover, these phenomena occurring at the orbital apex do not contribute much to clinical proptosis; hence many remain underdiagnosed. In ophthalmic examination, relative afferent pupillary defect (RAPD), blue-yellow (Tritan) color deficiency, and reduced contrast sensitivity in patients of TED are sensitive parameters for detecting DON. Clinical examination of the optic disc and optic disc imaging are important for the monitoring of DON.[2] Orbital magnetic resonance imaging with or without contrast may provide sufficient details about the extraocular muscles, orbital fat, optic nerve, orbital apex, superior ophthalmic vein, and other essential orbital structures. Apparent diffusion coefficient readings from magnetic resonance imaging (MRI) may provide the activity details in the extraocular muscles. Barrett’s muscle index of >60% is considered highly sensitive and specific for DON.[3] It is calculated on coronal computed tomography (CT) or MRI at a mid-point between the posterior globe and the orbital apex. Barrett’s index includes two indices, horizontal and vertical, and the larger of the two is taken as significant. Other parameters are extraocular muscle volume/orbital volume (MV/OV) and crowding index (CI) are considered reliable indicators of DON. The 3D MRI-based volumetric analysis showed the larger extraocular muscle volume as the most relevant factor in DON.[4] Optical coherence tomography angiography (OCTA) has been used to measure retinal nerve fiber layer thickness and peripapillary capillary vessel density (whole image and radial vessel density) as DON parameters. The patients with subclinical DON have shown significantly lower vessel densities as compared to normal individuals.[25] Intravenous steroids and orbital decompression surgery remain the mainstay of treatment in DON patients. The best outcomes for DON patients are provided by combined intravenous methylprednisolone and 3-wall orbital decompression, as vision is successfully preserved in most cases with a reduction in proptosis.[6] The advent of teprotumumab, a targeted insulin-like growth factor 1 (IGF-1) receptor-based therapy, is slowly establishing itself as “medical orbital decompression” therapy. It reduces the levels of thyroid stimulating hormone (TSH) receptors and IGF-1 receptors on fibroblasts and reduces the release of pro-inflammatory cytokines like interleukin (IL)-6, IL-8, and tumor necrosis factor alpha (TNF-α) responsible for severe TED.[26] Hence, an ophthalmologist should be aware of the clinical features of subclinical DON and maintain a low threshold for aggressive treatment. For the holistic management of DON, a multidisciplinary team involving an ophthalmologist, endocrinologist, and otolaryngologist should be working in coordination to avoid blindness.

ODP655 Apathetic Hyperthyroidism

Abstract Introduction Apathetic hyperthyroidism is a rare presentation of hyperthyroidism described in the elderly population. It is insidious in onset and symptoms are depression, lethargy, and weight loss. The adrenergic symptoms of hyperthyroidism are often absent. Furthermore, orbital disease associated apathetic hyperthyroidism has not been reported widely in literature. We present a clinical scenario of apathetic hyperthyroidism associated with orbitopathy. A 64-year-old lady presented with acute onset bilateral periorbital swelling that developed over 2 days. About 3 weeks prior, she began experiencing symptoms of brain fog, lethargy, intermittent nausea, anorexia, and headache. She experienced palpitations with exertion. Review of other systems was negative. Clinical exam: HR 70. Thyroid gland was enlarged, symmetric with no nodules and nontender, bilateral periorbital puffiness and mild conjunctival redness was noted. Lab data was significant for TSH at 0. 03 uIU/mL, elevated FT4 2.5ng/dL, total T3 of 222ng/dL, TSI 4.2 and sed rate of 10. Her thyroid ultrasound indicated hypervascular and heterogenous gland. Our patient was diagnosed with apathetic hyperthyroidism and thyroid eye disease. She was started on propranolol 60mg ER one tablet a day and methimazole 10mg a day. CT scan of the orbit indicated symmetric enlargement of extraocular muscles. Evaluation by the ophthalmologist confirmed the presence of thyroid eye disease with a clinical activity score of 6 out of 7 indicating severe thyroid eye disease and she was a candidate for treatment with teprotumumab. Discussion Apathetic hyperthyroidism was first described by Lahey in 1931, as a state of non-activated hyperthyroidism. It is manifested with gradual onset of apathy, depressed mood, lethargy, nausea, lack of appetite, weight loss, proximal muscle weakness, goiter, atrial fibrillation, and congestive heart failure. It lacks the typical presentation of a hyperadrenergic state of hyperthyroidism which include palpitations, anxiety, tremor, heat intolerance, and diaphoresis. Due to the lack of typical presentation of hyperthyroidism, timely diagnosis and treatment may be challenging. Concomitant occurrence of occult malignancy and hyperglycemia have been reported in case reports. Patients can also transition from apathetic state to hyperadrenergic state of hyperthyroidism and vice versa. Lahey emphasized the risk of unexpected death following surgery in apathetic hyperthyroidism. The absence of adrenergic symptoms could be supported by the theory that these patients are in a relative state of catecholamine deficiency or have a state of end organ catecholamine resistance. Significant orbital involvement in apathetic hyperthyroidism is not widely reported in the literature. Blepharoptosis has been mentioned in association with this form of hyperthyroidism. Our patient presented with acute onset eye symptoms which prompted testing of thyroid function. Further review of symptoms led to a diagnosis of apathetic hyperthyroidism with thyroid eye disease.

ODP306 Diplopia as Presenting Manifestation of Acromegaly

Abstract Introduction Acromegaly is a chronic disorder characterized by excess growth hormone production, withclinical manifestations ranging from subtle signs to significant systemic complications. We describe a patient who presented with diplopia as initial symptom of acromegaly. Clinical case: A 72-year-old female patient was referred for diplopia. Upon questioning, she reported headache, muscle aches and heavy sweating. On physical assessment, she had coarse facial features, thickened tongue and hand enlargement. Orbital CT images revealed a mild to moderate symmetric enlargement of the extraocular muscle which spared myotendinous junction. MRI sella showed a 12 mm pituitary adenoma. The diagnosis of acromegaly was confirmed with high IGF-1 and oral glucose tolerance test. The patient underwent endoscopic debulking of the pituitary tumor and pathology showed a densely granulated GH producing adenoma. Upon follow up, she reported improvement of the diplopia which was completely corrected with prism glasses. Remission of acromegaly was confirmed biochemically and repeated MRI sella showed no residual tumor. Conclusion Acromegaly is the most likely cause of extraocular muscle enlargement with subsequent diplopia in this patient. Ocular manifestations in patients with acromegaly include visual field defect, excessive lacrimation, proptosis and double vision. This case adds to previous literature that diplopia could be the presenting manifestation of acromegaly. This is the first case reporting of improvement of diplopia following remission of acromegaly. Comprehensive ophthalmic assessment of patients presenting with eye symptoms and features suggestive of acromegaly is of value as it helps with early diagnosis as well as improving the outcome of this condition. Presentation: No date and time listed

Automated detection of enlarged extraocular muscle in Graves' ophthalmopathy with computed tomography and deep neural network.

This study aimed to develop a diagnostic software system to evaluate the enlarged extraocular muscles (EEM) in patients with Graves’ ophthalmopathy (GO) by a deep neural network.This prospective observational study involved 371 participants (199 EEM patients with GO and 172 controls with normal extraocular muscles) whose extraocular muscles were examined with orbital coronal computed tomography. When at least one rectus muscle (right or left superior, inferior, medial, or lateral) in the patients was 4.0 mm or larger, it was classified as an EEM patient with GO. We used 222 images of the data from patients as the training data, 74 images as the validation test data, and 75 images as the test data to “train” the deep neural network to judge the thickness of the extraocular muscles on computed tomography. We then validated the performance of the network. In the test data, the area under the curve was 0.946 (95% confidence interval (CI) 0.894–0.998), and receiver operating characteristic analysis demonstrated 92.5% (95% CI 0.796–0.984) sensitivity and 88.6% (95% CI 0.733–0.968) specificity. The results suggest that the deep learning system with the deep neural network can detect EEM in patients with GO.

Clinical Characteristics of Patients with Euthyroid Thyroid-associated Ophthalmopathy

Purpose: To compare clinical features between thyroid-associated ophthalmopathy (TAO) patients with hyperthyroidism and euthyroidism.Methods: Between September 2015 and January 2021, we enrolled 96 TAO patients with euthyroidism (n = 16) or hyperthyroidism (n = 80). We recorded the age, sex, smoking status, presence of hypertension and diabetes, total follow-up period, unilateral involvement, eyelid edema, lid lag and retraction, clinical activity score (CAS), modified NOSPECS score, exophthalmometry, Hess test, area of extraocular muscles on orbital computed tomography (CT), treatment with systemic and subconjunctival steroids and radiotherapy, and serum levels of free thyroxine, thyroid-stimulating hormone (TSH), anti-thyroglobulin antibody (anti-Tg Ab), anti-thyroid peroxidase antibody (anti-TPO Ab), TSH receptor antibody (TSHR Ab), and thyroid stimulating antibody.Results: Unilateral involvement was significantly more common in the euthyroid group, while conjunctival injection, CAS, extraocular muscle movement restriction within 10° on the Hess chart, maximum cross-sectional area of the inferior rectus on orbital CT, and treatment with intravenous high-dose steroid were significantly more common in the hyperthyroid group. Furthermore, the modified NOSPECS score and levels of free thyroxine, anti-Tg Ab, anti-TPO Ab, and TSHR Ab were significantly higher in the hyperthyroid than euthyroid group.Conclusions: Compared to hyperthyroid TAO patients, those with euthyroid TAO were more likely to have unilateral involvement. They also had lower clinical activity and severity, milder enlargement of extraocular muscles (EOMs), and lower rates of EOM movement restriction and treatment with intravenous high-dose steroid. Euthyroid TAO patients had lower levels of free thyroxine, anti-Tg Ab, anti-TPO Ab, and TSHR Ab compared to hyperthyroid TAO patients.

Radiological Determinants of Complicated Immunoglobulin G4-Related Ophthalmic Disease: A Territory-Wide Cohort Study.

To evaluate the presenting radiological features of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and their associations with IgG4-related optic neuropathy (IgG4-RON), and IgG4-related ocular adnexal lymphoma (IgG4-ROL). A territory-wide, biopsy-proven, Chinese cohort. Masked review of orbital images, medical records, and histopathology reports. A total of 115 (94%) of the 122 patients in our cohort had preoperative orbital images (computed tomography=105, magnetic resonance imaging=40). Among them, 103/115 (90%) showed enlarged lacrimal glands, and 91 (88%) were bilateral. Nerve enlargement was observed: infraorbital in 31/115 (27%) patients and frontal in 17/115 (15%), 10 and 9 being bilateral, respectively. At least 1 or more extraocular muscle (EOM) enlargement was found in 41/115 (37%) patients, bilaterally in 20. Lateral rectus occurred in 30 (73%) of these 41 EOM patients and inferior rectus in 28 (68%). Two adjacent EOMs (inferior and lateral recti in 11 patients, inferior and medial recti in 7 patients) or multiple EOMs (at least 3) were enlarged in 23/41 (56%) and 13/41 (32%) of the patients, respectively. Intraconal lesions (67% vs 11%, P<0.05), infraorbital (83% vs 23%, P<0.005), or frontal (50% vs 15%, P<0.05) nerve enlargement was significantly associated with IgG4-RON (6 patients) by univariate analyses. Asymmetric lacrimal gland enlargement and discrete orbital mass (both P<0.05) were associated with IgG4-ROL (9 patients) by multivariate analyses. In this IgG4-ROD cohort, most patients had bilateral enlarged lacrimal glands, and the lateral rectus is the most frequently involved EOM. For the first time, unique radiological patterns associated with the development of IgG4-RON and IgG4-ROL are found.

Role of muscle biopsy in diagnosis of extraocular muscles enlargement.

To study the role of muscle biopsy in patients with enlarged extraocular muscles. A retrospective review of 31 patients who underwent biopsy for extraocular muscle enlargement. Characteristics, including signs, symptoms, imaging findings, and histopathological assessment were examined. Chronic inflammatory disorders represented the most common cause of the muscle enlargement followed by malignancy/metastasis. Multiple muscle involvement was more consistent with benign diseases, whereas single muscle involvement was more consistent with malignant causes. Positive predictive value of muscle biopsy was 0.52 for determining a specific cause of the muscle enlargement. Muscle biopsy is the standard for diagnosis in patients with extraocular muscle enlargement. This diagnostic test should be performed in every case of non-resolving muscle enlargement, with a specific diagnosis being made in over half of cases.

A Case of Choroidal Folds in a Patient with Immunoglobulin G4-related Ophthalmic Disease

Purpose: To report a case of choroidal folds caused by enlargement of the extraocular muscles (EOM) and orbital fat in a patient with immunoglobulin (Ig) G4-related ophthalmic disease. A full recovery was achieved after steroid administration.Case summary: A 56-year-old male received intravenous high-dose steroids to treat IgG4-related ophthalmic disease presenting with bilateral eyelid edema, hypertrophy of all EOM, and mild exophthalmos. After 3 months, only slight improvement was observed and azathioprine was initiated. The patient exhibited only a minimal response over the next 2 months; all medications were therefore stopped. One month later, the patient presented with aggravation of the swelling (both eyelids), exophthalmos, and visual impairment of the left eye. The corrected vision was 1.0 in the right eye and 0.2 in the left eye; choroid folds were observed in the posterior pole of the left eye. Intravenous steroid (500 mg) was given once and oral steroids prescribed as maintenance therapy. After 2 weeks, the corrected visual acuity recovered to 1.0 in both eyes, the eyelid edema and exophthalmos improved and the choroidal folds resolved completely.Conclusions: Choroidal folds associated with enlargement of EOM and orbital fat tissue can cause visual impairment in patients with IgG4-related ophthalmic disease. Steroids are effective in the acute phases and prevent irreversible visual impairment.