Tocilizumab use for optic nerve compression in thyroid eye disease: a prospective longitudinal cohort.

PURPOSE: To assess the effectiveness of tocilizumab in reverting the signs and symptoms of dysthyroid optic neuropathy (DON) in thyroid eye disease and the need for emergency orbital decompression. The secondary outcomes are to identify the optimal number of tocilizumab cycles to achieve the primary outcome, to analyze the association between thyroid stimulating immunoglobulin (TSI), clinical activity score (CAS) and proptosis in response to the treatment and the need for rehabilitative orbital decompression. METHODS: Prospective longitudinal cohort study that included 13 patients who had unilateral or bilateral dysthyroid optic neuropathy (DON) due to severe and progressive sight-threatening thyroid eye disease based on the CAS system. Patients were seen in this facility starting from July 2017, and all had received intravenous tocilizumab. RESULTS: Initial visual acuity mean was 0.52 ± 0.38 and the final were 0.93 ± 0.11 with a mean difference of 0.41 and P < 0.00245. The mean CAS prior to the initiation of the treatment was 7.92 ± 0.66 and the final was 2.85 ± 1.03 with mean difference of 5.07 and P < 0.00001. Initial mean proptosis was 24.85 ± 2.31 and the final was 21.78 ± 2.18 with a mean difference of 3.07 and P < 0.000497. No emergency orbital decompression was performed. TSI was high initially in all cases with a wide range of 2.4 to 40 IU/L and with a mean of 10.70 ± 13.40. The final TSI mean was 2.90 ± 3.90 with a mean difference of 7.81 and significant P value (P < 0.0272). CONCLUSION: Tocilizumab use in optic nerve compression showed promising results as it can be the primary or an alternative treatment option.

Long-term surgical outcome and impact on daily life activities of strabismus surgery in thyroid-associated ophthalmopathy with and without previous orbital decompression.

BACKGROUNDS: To report the long-term surgical outcomes and the impact on daily life activities of strabismus surgery in patients with Thyroid Associated Orbitopathy (TAO) with and without previous orbital decompression. METHODS: Patients who underwent strabismus surgery for TAO were retrospectively reviewed. The primary outcome was to evaluate the influence of orbital decompression on the outcomes of TAO related strabismus surgery. Surgical success was defined by the resolution of diplopia and a post-operative deviation < 10 prism diopters (PD). The secondary outcomes were the clinical features, surgical approaches, and impact on daily life activities. RESULTS: A total of 45 patients were included in the study. The decompression surgery group (DS) included 21 patients (46.7%), whereas the non-decompression surgery group (NDS) patients were 24 (53.3%). The mean follow-up time from the last strabismus surgery was 2,8 years (range 8-200 months). Successful surgical outcome was achieved in 57,1% of patients in the DS, and 75% of patients in the NDS (p = 0,226). DS patients required almost twice the number of surgical interventions for strabismus compared to the NDS (1,95 vs. 1,16 respectively, p = 0,006), a higher number of extraocular muscles recessed in the first surgery (2,67 vs. 1,08 respectively, p < 0.001), and a lower rate of unidirectional surgery compared to NDS (23% vs. 95%, p < 0,001). At the pre-operative assessment, 71.4% of DS patients had eso-hypotropia, while no patients had this type of strabismus in the NDS group (p < 0.001). On the other hand, the hypotropia rate was 79.2% in NDS patients and only 4.8% in DS patients (p < 0.001). Moreover, 21,8% of NDS patients used prism lenses in daily life activities, compared to 42.9% of patients that used prism lenses to reduce the impairment in their daily life activities (p = 0.016). CONCLUSIONS: The results of our study showed that DS patients required almost twice the number of strabismus surgical procedures, a higher number of extraocular muscles recessed in the first surgery, and an increased need for prism lenses to correct the residual deviation compared to the NDS, but with similar long-term surgical outcomes.

Asymmetric proptosis in thyroid eye disease.

PURPOSE: Patients with thyroid eye disease (TED) can present with asymmetric disease. The purpose of this study was to evaluate the prevalence of asymmetric TED in an Australian cohort and investigate clinical, biochemical, and radiological associations of asymmetric TED. METHODS: This was a retrospective study of patients with TED who underwent Hertel exophthalmometry and orbital computed tomography (CT) scans. Asymmetry was defined as a difference in the globe protrusion of ≥ 3 mm using Hertel exophthalmometry. Data was collected on patient demographics, clinical disease presentation, thyroid function tests and antibody levels. Muscles volumes were determined by manually segmenting the extraocular muscles on CT scans using a commercially available software. RESULTS: 172 orbits from 86 patients were included in the study. 28 (33%) patients had asymmetric TED. No significant differences were observed in age, gender, thyroid hormone status, anti-thyroid peroxidase levels, thyroid stimulating hormone receptor antibodies, disease activity status, or dysthyroid optic neuropathy between the asymmetric and non-asymmetric groups. The extraocular muscle volumes were significantly higher in the asymmetric orbit compared to its contralateral orbit. CONCLUSION: Asymmetric TED is a reasonably common occurrence in the course of TED. It is associated with higher extraocular muscle volumes, suggesting muscle enlargement as one of the underlying contributors to asymmetric proptosis. Thyroid eye disease should be considered in the differential of asymmetric orbital inflammatory disease.

Pearls & Oy-sters: Thyroid-Associated Ophthalmopathy and Transient Neuromuscular Junction Disorder Due to Graves Disease.

The concomitant presentation of thyroid-associated ophthalmopathy (TAO) and ocular myasthenia gravis is well documented. In the course of Graves disease (GD), symptomatic transient neuromuscular junction disorder may occur due to the effect of thyroid hormones at the neuromuscular synapse. Diagnostic clues are the clinical and electrophysiologic remission synchronous with restoration of euthyroidism. Furthermore, the occurrence of thymic hyperplasia in GD poses further diagnostic and therapeutic considerations. These points are discussed in the case report of a 43-year-old male patient suffering from TAO and transient neuromuscular junction disorder due to GD.

Treatment of Thyroid Eye Disease-Associated Ophthalmopathy and Myopathy With Intraorbital Injection of 5-Fluorouracil and Triamcinolone Acetonide.

PURPOSE: The authors report a technique of local application of anti-metabolite and corticosteroid mixture in the orbit for treatment of thyroid orbitopathy with moderate-severe inflammation and muscle involvement. METHODS: Patients of one orbital surgeon seen between March 2019 and May 2020 with active thyroid eye disease and restrictive strabismus were considered for local treatment of the myopathic component of the disease. A mixture of 1 ml 5-FU 50 mg/ml, 0.25 ml triamcinolone 40 mg/ml, and 1 ml lidocaine 2% is injected through the skin using a 25-gauge, 1.5-inch needle into the orbit adjacent to the affected extraocular muscle. Six patients were treated in the outpatient setting and 3 patients have been treated with this intervention intraoperatively at the time of orbital decompression. One was treated with the mixture reconstituted with hyaluronic acid (Healon GV) to address postoperative medial rectus fibrosis to the medial wall, this mixture was applied topically in the operative field and not injected. RESULTS: All patients had subjective improvement in the eye movement limitation and 2 patients had a change in motility on exam that was temporally correlated to injections. One patient did not disclose high-dose aspirin intake before injection and experienced a retrobulbar hemorrhage immediately following injection which was successfully treated. No complications were noted as a result of the medication itself. DISCUSSION: The combination of 5-fluorouracil and triamcinolone acetonide for orbital treatment may be a useful adjunct in treating patients with ongoing inflammatory activity, both in the office and in the operating room. The novel combination may optimize ophthalmic outcomes, modifying disease course in some patients.

Objective Diplopia Outcomes for Patients Treated With Teprotumumab for Thyroid Eye Disease.

BACKGROUND: Thyroid eye disease (TED) is a condition caused by inflammatory damage to the periocular tissue that often leads to double vision. Teprotumumab is an insulin-like growth factor 1 receptor antibody that was FDA approved for the management of TED in 2020, although much is yet to be elucidated regarding its effects on diplopia outcomes among patients with TED. Diplopia is a significant and life-altering effect of TED. Previous studies have reported the effect of teprotumumab on double vision subjectively using the Gorman diplopia score. However, there is a gap in the literature addressing the effect of teprotumumab treatment on objective ocular alignment measures. The purpose of our study was to address this gap. METHODS: We performed a retrospective review of patients who were diagnosed with TED, presented with diplopia, and treated with teprotumumab in a single-center academic ophthalmology practice. The primary outcome was change in ocular alignment in primary gaze position at 6 months (completion of teprotumumab treatment). Secondary outcomes included change in ocular alignment in other gaze positions, proptosis, eyelid position, and clinical activity score (CAS) at 6 months compared with baseline. To determine what factors may predict ocular alignment response to teprotumumab, we analyzed baseline characteristics among 3 groups, divided based on whether ocular alignment was worsened, stable, or improved at 6 months. RESULTS: Seventeen patients met inclusion criteria, 3 (18%) worsened, 10 (59%) were stable, and 4 (24%) improved. CAS ( P = 0.02) was significantly different among the groups and was higher in those who worsened and those who improved compared with those who remained stable. Right gaze horizontal prism deviation ( P = 0.01) and left gaze horizontal prism deviation ( P = 0.03) were significantly different among the groups, with a greater degree of left gaze horizontal prism deviation in the worse group than the stable group ( P = 0.04). CONCLUSIONS: Our study demonstrated that most patients remained stable after teprotumumab treatment regarding ocular alignment in primary gaze and the number of patients who improved was slightly higher than the number of patients who worsened after teprotumumab treatment. There are some baseline measures, such as CAS and right and left gaze horizontal prism deviation that can help better predict how a patient will respond to teprotumumab treatment. Our results can better inform physicians of how to counsel patients with TED when considering teprotumumab therapy.

Real-World Experience With Teprotumumab in Patients With Dysthyroid Optic Neuropathy.

BACKGROUND: Teprotumumab, an insulin-like growth factor I receptor inhibitory antibody, improved proptosis, diplopia, inflammatory signs/symptoms, and quality of life in patients with active thyroid eye disease (TED) in clinical trials. The trials excluded patients with dysthyroid optic neuropathy (DON). Recently, many case reports and case series have reported the successful use of teprotumumab to treat DON. Here, we review the data from published cases and our clinical experience in treating patients having DON with teprotumumab. METHODS: A literature search was conducted of patients with DON treated with teprotumumab from January 2020 through September 2022. Data from DON patients from the authors' (M.A.T. and C.A.B.) clinical practice were included. Primary outcome measure was mean (SD) improvements for visual acuity, color vision, and visual fields. Improvements in proptosis and clinical activity score (CAS) and diplopia were compared before and after teprotumumab administration. RESULTS: Ten observational studies/case reports were identified along with 2 patients in our practice. In all, there were 24 active TED patients with DON (37 eyes) who were treated with teprotumumab. Mean (SD) age was 66.5 (13.6) years and 13 (54%) were females, disease duration ranged from 2 months to >15 years. 22/24 patients had none, minimal improvement or progression of visual loss with intravenous/oral corticosteroids, orbital decompression (n = 9), and orbital radiation (n = 2). There were 2 patients who received teprotumumab as the only therapy. Overall, 88% (21/24) reported improvement in visual acuity after teprotumumab and in 75% (18/24), improvement in vision was observed after just 2 infusions of teprotumumab. Three eyes had decompression surgery in close proximity to teprotumumab infusions and were excluded from analyses. Mean (SD) improvement in visual acuity was 3.73 lines (SD 3.74), range 2-15 lines in 33 eyes. The mean (SD) improvement in the mean deviation on visual field testing in 15 eyes was 5.6 db (3.0 db). Mean (SD) improvement in proptosis was 4.37 mm (SD: 2.11) (20 patients, 32 eyes); and clinical activity score: mean reduction of 5.1 (1.3) for 18 patients. Teprotumumab was well tolerated in all but one patient. Adverse events reported included fatigue, dysgeusia, hearing loss, nausea, hyperglycemia, and muscle spasms. CONCLUSIONS: Teprotumumab is an effective treatment for DON in our experience and in published cases in whom treatment with steroids, surgery, or orbital radiation was unsuccessful.

The effect of previous orbital decompression on outcomes of rectus muscle recession surgery in patients with thyroid ophthalmopathy.

PURPOSE: To evaluate the effect of previous orbital decompression on outcomes of rectus muscle recession surgery in patients with thyroid-associated ophthalmopathy. METHODS: This retrospective study enrolled 55 eyes of 33 patients treated in our hospital for restrictive strabismus caused by thyroid-associated ophthalmopathy. We performed muscle recession for the obviously restricted extraocular muscles, with 6 weeks of follow-up. Surgical outcomes were compared between the orbital decompression group (DG, n=15) and non-orbital decompression group (NDG, n=18). RESULTS: A total of 33 patients with Graves' ophthalmopathy who underwent rectus muscle recession surgery were included. Of these, 15 patients had undergone orbital decompression prior to strabismus surgery, and 18 had not. The two groups did not differ in terms of the preoperative horizontal or vertical ocular deviation, degree of restriction of eye movement, degree of diplopia, or mean number of muscles that underwent surgery (P>0.05). There was no significant difference in the preoperative horizontal or vertical ocular deviation, level of eye movement restriction, degree of diplopia and the success rate of the surgery (P>0.05). CONCLUSION: Rectus muscle recession surgery in patients with thyroid-associated ophthalmopathy during the quiescent period could improve the ocular deviation and diplopia, and orbital decompression performed before strabismus surgery had no significant effect on surgical technique or outcomes of rectus muscle recession surgery.

A case of rapidly declining glycemic control and diabetic ketoacidosis in a newly diagnosed diabetes patient after starting teprotumumab for thyroid eye disease.

PURPOSE: Teprotumumab for thyroid eye disease has a known hyperglycemic adverse effect through its impact on the insulin-like growth factor-1 receptor. While most cases are mild and easily managed by adjusting diabetes medications, it appears some patients have a more dramatic response. The purpose of this case report is to highlight an example of rapidly declining glycemic control and diabetic ketoacidosis (DKA) in a patient with newly diagnosed diabetes after starting teprotumumab for thyroid eye disease. METHODS: This was a single-patient case report assessing a severe episode of hyperglycemia leading to new-onset diabetes. The case report was approved by Atrium Health Wake Forest Baptist's IRB committee. The patient was closely monitored by a pharmacist-led pharmacotherapy clinic after initial diagnosis and periodically since then to adjust therapy and assess glucose and hemoglobin A1c (HbA1c) trends. RESULTS: After the acute episode of DKA was managed inpatient, the patient was discharged with insulin outpatient, but this was ultimately weaned off, and the patient's glucose and HbA1c are stable on metformin alone. This patient decided to not continue teprotumumab due to extensive side effects including but not limited to severe hyperglycemia. CONCLUSION: While additional research is needed as to the cause of severe hyperglycemia in select patients, providers should consider proactively monitoring glucose throughout treatment with teprotumumab by ensuring that patients have baseline labs and labs at every visit and access to a glucometer with education for its use.

Preoperative Risk Factors for Proptosis Recurrence After Rehabilitative Orbital Decompression in Graves' Orbitopathy Patients.

PURPOSE: Rehabilitative orbital decompression treats disfiguring exophthalmos in patients with Graves' orbitopathy (GO). This study aimed to identify risk factors associated with the postoperative recurrence of proptosis after orbital decompression. DESIGN: Retrospective, case-control study. METHODS: This retrospective review included patients with GO who underwent rehabilitative orbital decompression for disfiguring proptosis in an inactive state with a low clinical activity score (0-2) between January 2017 and December 2020 by a single surgeon. Exophthalmos was measured using a Hertel exophthalmometer, and recurrence was defined as an increase of 2 mm or more after decompression during the follow-up period. The association between preoperative variables and proptosis recurrence was analyzed using multivariable logistic regression. RESULTS: Of the total 217 patients, 11 (5.1%) developed recurrence of proptosis during the follow-up period (range, 3-30; mean, 15.6 months). Univariate logistic regression analysis identified thyroid-stimulating hormone receptor antibody (TRAb) and thyroid-stimulating immunoglobulin (TSI) as significant factors for recurrence, with age, sex, smoking, disease duration, orbital radiotherapy, and total thyroidectomy history being nonsignificant. TRAb remained significant in a multivariate logistic regression analysis (odds ratio, 1.06; P = .014). Receiver operating characteristic curve analysis revealed an area under the curve of 0.86 with a sensitivity of 90.9% and specificity of 82.0% at a TRAb level of 7.96 IU/L. CONCLUSION: Preoperative TRAb and TSI are valuable markers to predict proptosis recurrence after orbital decompression. These results may help surgeons to decide the optimal timing for orbital decompression to lessen the risk of postoperative recurrence of proptosis.

Comparison of surgical effect in active and inactive Dysthyroid Optic Neuropathy after Endoscopic Transnasal Medial Orbital Decompression.

PURPOSE: To evaluate and compare the changes in orbital soft tissue volume and visual function after endoscopic transnasal medial orbital decompression in patients with active and inactive dysthyroid optic neuropathy (DON). METHODS: This prospective, cohort study recruited 112 patients (112 eyes) with DON who were divided into an active and inactive DON group (56 eyes each) by clinical activity scores. All patients underwent endoscopic transnasal medial orbital decompression. The pre- and post-operative orbital soft tissue volumes were measured with high-resolution computed tomography (CT) using Mimics software. Visual function, including best-corrected visual acuity (BCVA), visual field (VF), and visual evoked potential (VEP), was recorded before and after surgery. RESULTS: Preoperatively, compared with the inactive DON group, the active DON group had greater extraocular muscle volume (EMV) and EMV/orbital volume (OV) ratio, but worse BCVA, VF, and exophthalmos. Postoperatively, although the EMV slightly increased, with the enlarged medial rectus muscle contributing dramatically, the EMV/OV ratio decreased in patients with DON. Besides, visual function including BCVA, VF, VEP and exophthalmos was also improved in both groups after surgery. There were no significant differences in postoperative OV; EMV; EMV/OV ratio; and the BCVA, VF, and VEP parameters between both groups (all P > 0.05). CONCLUSION: Patients with DON who did not respond well to steroids, regardless of disease activity, may benefit from orbital decompression via the decrease in the proportion of EMV in OV, especially patients with active DON, who showed more improved visual function than patients with inactive DON.

Thyroid Inflammation and Immunity During the COVID-19 Pandemic: A Comprehensive Review and Case Study.

The coronavirus disease 2019 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has led to the development of various vaccines. Reports have emerged suggesting a possible association between SARS-CoV-2 vaccination and the onset of thyroid diseases. This review explores the clinical aspects of thyroid disorders following SARS-CoV-2 vaccination, including a case report of a patient with concomitant subacute thyroiditis (SAT) and Graves' disease (GD) with blocking thyrotropin receptor autoantibodies (TSH-R-Ab) following SARS-CoV-2 vaccination. SAT, characterized by transient inflammation of the thyroid gland, has been reported after SARS-CoV-2 vaccination. GD, an autoimmune hyperthyroidism, has also been observed post-vaccination, often with stimulating TSH-R-Ab. Graves' orbitopathy (GO) has been associated with SARS-CoV-2 vaccination in patients with a history of immune thyroid disease. The unique case underscores a very rare thyroid condition of functional hypothyroidism in possible relation to SARS-CoV-2 vaccination and the usefulness of functional analysis of TSH-R-Ab that can provide valuable insights into disease pathogenesis and help to guide treatment. This review highlights the need for continued monitoring and awareness of potential thyroid-related complications following SARS-CoV-2 vaccination.

Surgical Outcomes of Standardized Endoscopical Deep Medial Orbital Decompression in Dysthyroid Optic Neuropathy.

INTRODUCTION: The aim of the study was to standardize the endoscopic deep medial orbital decompression surgery for better relief of optic nerve compression in dysthyroid optic neuropathy (DON). METHODS: A total of 128 eyes from patients received the standardized endoscopic deep medial orbital decompression surgery were recruited in this study. The efficacy of the procedure was assessed at a 1-month follow-up by the best-corrected visual acuity (VA), visual field (VF), and visual evoked potential (VEP). Clinical data were collected to explore the factors that affected visual recovery. Oxygen saturation of retinal blood vessels, retinal thickness, and vessel density were measured to demonstrate the potential recovery mechanisms. RESULTS: After surgery, the ratio of extraocular muscle volume in the orbital apex to orbital apex volume significantly decreased from 44.32 ± 22.31% to 36.82 ± 12.02% (p < 0.001). 96.87% of eyes' final VA improved; average VA improved from 0.93 ± 0.73 to 0.50 ± 0.60 at 1 week (p < 0.001) and 0.40 ± 0.53 at 1 month (p < 0.001). Postoperatively, VF and VEP also improved, the oxygen saturation of retinal arteries increased, and the retinal thickness was reduced. Preoperative VA, visual impairment duration, and clinical activity score evaluation were associated with visual recovery. CONCLUSION: In this study, we standardized the endoscopic deep medial orbital decompression, of which key point was to relieve pressure in the orbital apex and achieved satisfactory visual recovery in DON patients.

Unusual onset of Graves' disease associated with thymic hyperplasia in a 5-year-old girl with congenital bilateral clinical anophthalmia: diagnostic and therapeutic challenges.

OBJECTIVES: Graves' disease (GD) is a rare auto-immune disorder in pediatric population. The association between GD and thymic hyperplasia was rarely reported in children. Diagnosis and management of GD are challenging in children. CASE PRESENTATION: This report presents the case of a 5-year-old girl with a personal history of asthma and congenital bilateral isolated clinical anophthalmia who presented with acute congestive heart failure, sinus tachycardia and atypical signs of orbitopathy with edema and erythema of the lower right eyelid and excessive tearing. The diagnosis of GD was based on detecting a suppression of serum TSH level and the presence of high titers of TRAbs. Relapse occurred after 10 months of antithyroid drugs with chief complaints of palpitations, dyspnea and dysphagia. Computed tomography showed heterogeneous anterior mediastinal mass with no invasion into the surrounding tissue. The marked shrinkage of the mass after radioiodine therapy supported the diagnosis of thymic hyperplasia associated with GD. CONCLUSIONS: The presence of clinical anophthalmia may be a confusing factor for the diagnosis of Graves' ophthalmopathy. Recognition of the association between GD and thymic hyperplasia would avoid invasive diagnostic procedures and unnecessary surgical resection. Radioiodine therapy may be used in young children with repeated relapses of GD.

Thyroid Eye Disease and Its Association With Diabetes Mellitus: A Major Review.

PURPOSE: Thyroid eye disease (TED) associated with diabetes mellitus (DM) presents unique challenges. DM is a risk factor for TED. Standard management of TED with glucocorticoids (GC), orbital radiation, or teprotumumab can cause adverse events in poor glycemic control. The authors reviewed the literature on the relationship between TED and DM and the management of co-existing diseases. METHODS: The authors searched PubMed with keywords "thyroid eye disease," "diabetes mellitus," and similar terms from 2013 to 2022. The authors included relevant studies after screening the abstracts. Additional references to the selected studies were included where applicable. Data were extracted from the final articles according to the preplanned outline of the review. RESULTS: The initial search yielded 279 abstracts. The final review included 93 articles. TED and DM interact at multiple levels-genetic, immunologic, cellular, nutritional, and metabolic. Both DM and thyroid dysfunction exacerbate the morbidity caused by the other. Metabolic factors also affect the inflammatory pathway for TED. Patients with DM develop TED with greater frequency and severity, necessitating interventions for vision salvage. Agents (GC, teprotumumab, or radiation) used for TED are often unsuitable for treatment with DM, especially if there is poor glycemic control or diabetic retinopathy. There were no studies on using steroid-sparing agents in TED with DM. CONCLUSION: TED and DM co-exist because of multiple intersections in the pathophysiology. Challenges in the treatment include increased TED severity and risk of hyperglycemia and retinopathy. Multidisciplinary teams best undertake treatment of TED with DM.

Dysthyroid Optic Neuropathy.

PURPOSE: Dysthyroid optic neuropathy (DON) is a sight-threatening complication of thyroid eye disease (TED). This review provides an overview of the epidemiology, pathogenesis, diagnosis, and current therapeutic options for DON. METHODS: A literature review. RESULTS: DON occurs in about 5% to 8% of TED patients. Compression of the optic nerve at the apex is the most widely accepted pathogenic mechanism. Excessive stretching of the nerve might play a role in a minority of cases. Increasing age, male gender, smoking, and diabetes mellitus have been identified as risk factors. Diagnosis of DON is based on a combination of ≥2 clinical findings, including decreased visual acuity, decreased color vision, relative afferent pupillary defect, visual field defects, or optic disc edema. Orbital imaging supports the diagnosis by confirming apical crowding or optic nerve stretching. DON should be promptly treated with high-dose intravenous glucocorticoids. Decompression surgery should be performed, but the response is incomplete. Radiotherapy might play a role in the prevention of DON development and may delay or avoid the need for surgery. The advent of new biologic-targeted agents provides an exciting new array of therapeutic options, though more research is needed to clarify the role of these medications in the management of DON. CONCLUSIONS: Even with appropriate management, DON can result in irreversible loss of visual function. Prompt diagnosis and management are pivotal and require a multidisciplinary approach. Methylprednisolone infusions still represent first-line therapy, and surgical decompression is performed in cases of treatment failure. Biologics may play a role in the future.

Comparison of Three Methods of Tonometry in Patients with Inactive Thyroid-Associated Orbitopathy.

INTRODUCTION: Intraocular pressure (IOP) measurement in patients with thyroid-associated orbitopathy (TAO) can be difficult and misleading, particularly in patients with diplopia and eye deviation (esotropia or hypotropia). However, when measuring IOP, it is also necessary to pay sufficient attention to TAO patients without diplopia in primary gaze direction and without motility disorder that might not be readily apparent. PURPOSE: The aim of this study was to evaluate the accuracy of measurement of intraocular pressure (IOP) using three different types of tonometers: the rebound tonometer (iCARE), the Goldmann applanation tonometer (GAT) and the non-contact airpuff tonometer (NCT) in patients with inactive TAO.  Materials and Methods: A total of 98 eyes of 49 adult patients with TAO were examined. The study group included 36 females and 13 males, with an age range of 19-70 years and a median age of 55.0. All the patients had evidence of thyroid disease,  a history of mild to moderate TAO, no clinical signs or symptoms of active disease, and no diplopia in direct gaze direction. In addition to a comprehensive eye examination, all the patients underwent measurement of intraocular pressure with three tonometers: NCT, iCARE, and GAT. The measurements with these three devices were compared. RESULTS: The mean IOP was 18.1 ± 2.4 mmHg (range 13-25 mmHg) with GAT, 22.3 ±5.0 mmHg (range 13-35 mmHg) with NCT, and 18.0 ±2.4 mmHg (range 13.3-26 mmHg) with iCARE. The mean difference between the GAT and iCARE measurements (using the Bland-Altman analysis) was -0.1 ±1.16 mmHg (limits of agreement -2.4 to 2.1). The mean difference between the GAT and NCT measurements was 4.2 ±3.6 mmHg (limits of agreement -2.8 to 11.2). The mean difference between the iCARE and NCT measurements was -4.3 ±3.7 mmHg (limits of agreement -11.6 to 2.9). No significant difference was found between GAT and iCARE (p = 1.000). However, there was a significant difference between GAT and NCT (p < 0.0001), as well as between iCARE and NCT (p < 0.0001).  Conclusions: In patients with TAO, NCT significantly overestimates IOP values compared to the GAT and ICare. By contrast, the iCARE rebound tonometer provides IOP measurements comparable to the gold standard GAT in these patients.

Painless Orbital Bone Infarction in a Child with Sickle Cell Anemia: A Case of Misdiagnosed Periorbital Cellulitis.

BACKGROUND Sickle cell orbitopathy is a rare complication of sickle cell disease that closely mimics other conditions, such as orbital cellulitis and osteomyelitis. We report a case of painless orbital bone infarction masquerading as periorbital cellulitis in a child with sickle cell anemia. CASE REPORT A 4-year-old Saudi girl with sickle cell disease presented to our hospital with vaso-occlusive crisis characterized by bilateral lower limb pain and painless left orbital swelling. On examination, she had swelling of the left upper eyelid with redness and mild ptosis (margin reflex distance 1 was 2 mm) without proptosis. Magnetic resonance imaging with contrast showed bilateral sub-periosteal heterogeneous collections (2×0.8×2.1 cm in the superolateral wall of the left orbit and 1×0.6 cm in the inferolateral wall of the right orbit), with intermediate-to-high T1 signal intensity and high T2 signal, causing a mass effect on the adjacent superior and lateral rectus muscles. The patient was treated with systemic antibiotics and supportive treatment for vaso-occlusive crisis under the care of the pediatric team and was discharged without complications. CONCLUSIONS The diagnosis of sickle cell orbitopathy can be challenging, and an accurate diagnosis is essential to ensure appropriate management. Thus, we report the case of a 4-year-old child with painless sickle cell orbitopathy masquerading as pre-septal cellulitis.

Assessment of risk of obstructive sleep apnea with thyroid eye disease and its activity.

PURPOSE: To evaluate the association between obstructive sleep apnea (OSA) and thyroid eye disease (TED) and its effect on disease activity. METHODS: A prospective case-control study was conducted from January 2020 to March 2022. All TED patients (group A) were clinically evaluated. The activity of thyroid eye disease was calculated based on the clinical activity score (CAS), and grading of severity was done according to the EUGOGO classification. All TED patients (group A) were screened for OSA using the Snoring Tired Observed Pressure (STOP)-Bang survey. Age- and gender-matched control group patients (group B) without TED were screened for OSA. RESULTS: One hundred TED patients and 138 control patients without TED were included in the respective groups. Sixty-two (62%) patients in group A and 48 (34.78%) patients in group B were having high risk of OSA, and this difference was statistically significant (P = 0.001). Further, in group A patients, on univariate analysis, TED activity was significantly associated with a high risk of OSA (P = 0.009). On multivariate logistic regression analysis, OSA also showed significant association with TED activity (odds ratio [OR]: 4.14, 95% confidence interval [CI]: 1.11-18.85 at 10% level; P = 0.05). CONCLUSION: Our study showed that OSA is significantly associated with TED disease and its activity. However, no significant association was found between OSA and severity of the disease.