Bilateral Proptosis Research Articles

Abstract #1405991: Graves’ Disease Presenting as Thymic Hyperplasia

Thymic hyperplasia associated with Graves' disease (GD) is well known to thyroidologist, and reported up to 38% of GD worldwide; however, it remains unrecognized in routine clinical practice. Here we report two cases of GD presenting as anterior mediastinum mass, consistent with thymic hyperplasia. I - A 41-year-old female presented with shortness of breath, chest pain, and nausea. She reported palpitations and unintentional weight loss over the last six months. There was no history of thyroid disease. Blood pressure was 162/86 mmHg, heart rate 94/minute, respiratory rate 26 breaths/minute, temperature 36.7 F, and body mass index 23.5 kg/m2. The thyroid was enlarged with mild tenderness. Laboratory showed TSH < 0.005 (0.358 to 3.74 mc IU/mL), free thyroxine 5.54 (0.76 to 1.46 ng/dL), thyroid-stimulating immunoglobulins 407% (0 to 140%). Computed tomography of the chest with contrast revealed an anterior mediastinal mass. GD was diagnosed, and she was started on standard therapy. II - A 54-year-old female presented with worsening shortness of breath, palpitations, and leg swelling for a several weeks. She reported progressive exertional shortness of breath for ten months, intermittent palpitations, fatigue, and weight loss for the last five months. She had a history of hypertension but no history of thyroid disease. At presentation blood pressure was 159/72 mm Hg, heart rate 102 beats/minute, respiratory rate 20/minute, temperature 37.1 F, and body mass index 32.7 kg/m2. She had atrial fibrillation with a rapid ventricular rate. Her examination showed mild bilateral proptosis and non-tender enlarged thyroid. Results showed TSH < 0.005 (0.358 to 3.74 mc IU/mL), free thyroxine 7.23 (0.76 to 1.46 ng/dL), free triiodothyronine 13.2 (2.18 to 3.98 pg/mL), anti-microsomal antibody (TPO) 315 (0 to 60 IU/mL), and thyroid-stimulating immunoglobulins (TSI) 429 (0 to 140%). Computed tomography of the chest with contrast revealed an anterior mediastinal mass. GD was diagnosed, and standard therapy was started. Discussion: Our two cases demonstrate GD-associated thymic hyperplasia later confirmed during hospitalization with MRI imaging. CT-imaging results were available at the same time or earlier than thyroid function tests but did not mention the differential diagnosis of GD or other autoimmune diseases which would help to avoid unnecessary interventions and guide therapy. Both patients were schedule to closely follow up with endocrinology and repeat imaging in about six months to one year is expected to show reduction in thymic size which would support the diagnosis of GD-associated hyperplasia.

PFEIFFER SYNDROME TYPE II : A GENETICALLY PROVEN CASE

Pfeiffer syndrome is a rare genetic disorder in western population .This condition is very rare in the Asian population. This syndrome was first described by Rudolf Pfeiffer in 1964. We are presenting here is a case of newborn with pfeiffer syndrome type II demonstrating a cloverleaf skull with craniosynostosis, hypertelorism, bilateral proptosis, low set ears, syndactyly, broad thumb broad great toe, elbow synostosis.

Therapeutic outcomes of mycophenolate mofetil and glucocorticoid in thyroid-associated ophthalmopathy patients.

To analyze the efficacy of mycophenolate mofetil (MMF) and glucocorticoid administration in patients with thyroid-associated ophthalmopathy (TAO). Sixty patients with moderate to severe TAO treated in Jingzhou Central Hospital from January 2022 to June 2022 were selected and enrtolled in this study. The subjects were divided into experimental group (n=30) and control group (n=30) based on the random number table method. Glucocorticoid pulse therapy was provided in the control group, while MMF was given in the experimental group on the basis of Control group. Clinical activity score (CAS), quality of life (QOL), visual acuity, eyelid fissure width, intraocular pressure, and degree of exophthalmos were observed at the time of admission and at the 12th week and 24th post-treatment weeks. We compared the immune function (TRAb, IL-6, and CD4+/CD8+) of the two groups pre-treatment and 24 weeks post-treatment, and evaluated the clinical therapeutic effect. The clinical effective rates at 12 and 24 weeks in the experimental group were higher (73.3% and 83.3%) than those in the control group (46.7% and 60.0%) (P <0.05). After 12 weeks of treatment, patients' CAS scores, and bilateral lid fissure width decreased and right eye visual acuity increased in the control group compared with those before treatment (P < 0.05); further, after 24 weeks of treatment, patients' QOL scores and bilateral visual acuity increased and CAS scores, bilateral lid fissure width and proptosis decreased compared with those before treatment, and patients' QOL scores, CAS scores and bilateral proptosis improved more than those at 12 weeks of treatment (P <0.05). Additionally, greater improvements were observed in the patients' QOL and CAS scores, and proptosis after 24-week treatment than after 12-week treatment (P<0.05). In the experimental group, the QOL score and binocular visual acuity increased, whereas the CAS score, intraocular pressure, lid width, and proptosis decreased after 12 weeks of treatment as compared to the values of these parameters in the pre-treatment period (P < 0.05); after 24 weeks of treatment, greater improvements were established in the ocular-related indexes improved compared to the pre-treatment period and after 12 weeks of treatment (P < 0.05). After 12 weeks of treatment, the patients in the experimental group had more considerable improvements in the right visual acuity, right intraocular pressure, and left lid fissure width than the control group (P < 0.05); at 24 weeks of treatment, patients in the experimental group had greater improvements in the QOL score, bilateral visual acuity, intraocular pressure, bilateral lid fissure width, and bilateral proptosis than the control group (P < 0.05). No significant differences were found in the values of TRAb, IL-6, and CD4+/CD8+ between the two groups before treatment (P>0.05); the values of TRAb, IL-6, and CD4+/CD8+ in the experimental group was significantly lower than those before treatment and in the control group after 24weeks of treatment. (P>0.05). No statistically significant difference was observed in the incidence of liver damage and menstrual disorders between the two groups during the 24 weeks of treatment (P>0.05). The combination of oral MMF and glucocorticoid shock therapy is an effective drug for the treatment of patients with moderately active TAO.

Wegener’s Granulomatosis Revealed by Bilateral Orbital Inflammatory Pseudotumor: a Case Report and Literature Review

Purpose: To describe the chronology and the extent of orbital involvement in a case of granulomatosis with polyangiitis (GPA). Methods: Descriptive case report and literature review. Results: A thirty-two years old male consulted with the following complaints: A retro-orbital discomfort in both eyes, a bilateral fast progressive proptosis and a decrease in visual acuity in the left eye. Eye examination revealed a proptosis, ptosis, epiphora and a motility restriction and peripheral corneal ulcer infiltrates with nodular scleritis in the left eye. A palpable orbital mass was located in the sub temporal orbit in both eyes. Orbit magnetic resonance imaging (MRI) unveiled a diffuse and elongated enlargement of both lacrimal glands with enhancing mass in the superior lateral aspect of both eyes globe. The diagnosis of an orbital pseudotumor was suspected. The biological work-up revealed a high blood levels of inflammation markers and antineutrophil cytoplasmic antibodies (c-ANCA). The diagnostic of active systemic granulomatosis with polyangiitis was then made. Conclusion: As seen in this case, clinicians should consider a differential diagnosis of granulomatosis with polyangiitis in patients with an orbital pseudotumor mimic aspect.

Hand-Schüller-Christian syndrome combined with empty sella syndrome: A case report and literature review.

Hand-Schüller-Christian syndrome (HCS) is a rare disease with little clinical awareness, but the condition is more dangerous, and it combines with empty sella syndrome (ESS) which is extremely rare. A 26-year-old male patient who had proptosis, headaches, and diabetes insipidus for more than 10 years, and chronic cough and wheeze for 8 years presented to our hospital due to an abrupt onset of chest pain for 2 days. Hand-Schüller-Christian syndrome is diagnosed based on the typical clinical manifestations of diabetes insipidus and bilateral proptosis, magnetic resonance imaging (MRI) pituitary imaging and pathology. Empty sella syndrome is diagnosed based on hormonal indicators, clinical manifestations and MRI pituitary scan results. Type 1 respiratory failure and severe pneumonia can be diagnosed based on the results of clinical examination, chest imaging (including chest x-ray and computed tomography), pathology and blood gas analysis. Left pneumothorax can be diagnosed with chest imaging. "Meropenem and Cefdinir" were given for antimicrobrial coverage, "Desmopressin acetate" for anti-diuretic treatment, "Forcodine" for cough relief, "Ambroxol and acetylcysteine" for phlegm reduction, and continuous closed chest drainage was performed. The patient discharged after cough, wheezing, headache and other symptoms improved, and vital signs were stable. The patient has been followed up once a month for 17 months ongoing after discharge. At present, symptoms such as cough, sputum, and wheezing have improved considerably, and the mMRC score of dyspnea is 2 points. The reexamination of the chest X-ray shows that the absorption of lung exudates is better than before, and there is no recurrence of pneumothorax. Consider whether isolated diabetic insipidus is related to HSC, and if so, conduct an MRI, a biopsy, and other examinations as soon as possible.

Septic Cavernous Sinus Thrombosis in a Young Male Presenting With Pneumothorax Secondary to Septic Pulmonary Emboli: A Case Report

Septic pulmonary embolism (SPE) is a rare complication that happens when infected thrombi from the original site of infection break off and travel to the pulmonary blood vessels, causing an infarction or an abscess. Cases were reported on SPE, with tricuspid or pulmonary valve endocarditis being the most common primary site, especially in intravenous drug abusers. There are, however, very few reports of SPE brought on by septic cavernous sinus thrombosis (CST). Here, we describe the case of an 18-year-old male who had a pustule on his left eyelid, after which he developed fever, spontaneous swelling of his left eye, followed by his right eye, along with bilateral proptosis and diplopia, and new-onset dyspnea. Auscultation revealed decreased breath sounds in the left lung fields. Magnetic resonance imaging (MRI) revealed cavernous sinus thrombosis. Blood cultures isolated Staphylococcus aureus species. High-resolution computed tomography (HRCT) revealed a left-sided pneumothorax with minimal pleural effusion and multiple nodules scattered among both lungs, suggesting septic pulmonary emboli. We report this case to convey how a minor lesion, that is, an eyelid pustule (stye), can get complicated and set off a spiral of events that takes an unexpected tangent, challenging physicians and necessitating a rigorous approach.

Etiological Pattern, Clinical Presentation and Outcome of Patients with Proptosis in a Tertiary Care Hospital

Objective: To determine the etiological pattern, clinical presentation and outcome of patients with proptosis in a tertiary care hospital.&#x0D; Study Design: Cross-sectional study.&#x0D; Place and Duration of Study: Ophthalmology Department, Khyber Teaching Hospital, Peshawar Pakistan, from Jan 2019 to Jun 2020.&#x0D; Methodology: This study was conducted on 60 patients having proptosis. Patients were treated either medically, surgically or both. The demographic profile included age, gender and type of proptosis. The outcome included recovery, re-treatment,referral to the relevant speciality and loss to follow-up, were measured.&#x0D; Results: Out of 60 patients with proptosis, 39 were males, and 21 were female. Fifty-two patients had unilateral, and 8 had bilateral proptosis, with the majority suffering from non-axial proptosis. On aetiology exhibited tumours (45%), infectious (25%), inflammatory (16.6%), vascular (6.66%) and injury (6.66%). The surgical procedure was indicated in 27(45%) patients,while medical treatment was given in 28(46.6%) patients. Five patients (8.4%) received both surgical and medical treatment. Out of 60 patients, 26 patients (46.33%) fully recovered and 11 patients (18.33%) did not recover, 17(28.33%) patients were referred to other specialities for management, and 5(8.3%) patients lost to follow-up.&#x0D; Conclusion: In our study, tumours were the main cause of proptosis, followed by infective and inflammatory causes, with the paediatric age group (&lt;18 years) at more risk.

Multisystemic langerhans cell histiocytosis with orbital involvement

A 3-year-old male initially presented with low-grade fever and scalp rash of 2 months' duration. Patient also showed macrocephaly with bilateral proptosis since birth. Work up revealed CD1a, S100, Langerin and BRAF mutation positive results. A diagnosis of multisystemic Langerhans Cell Histiocytosis (LCH) was confirmed. Multiple punched-out bony lytic lesions with geographic appearance predominantly in the frontal and parietal regions. These are classic radiographic finding for cranial vault lesions in LCH. A solitary lesion may already be suggestive. Langerhans cell histiocytosis is a rare disorder commonly affecting children with unknown etiology. It has multisystemic involvement (skin, lymph nodes, bones, central nervous system, abdominal organs especially the liver and/or spleen). The bones are commonly involved in this disease. That's why biopsy-proven LCH should include a skeletal survey. Langerhans cell histiocytosis can involve any bone in the human body but mainly in the axial skeleton. Over 50% occur in the skull, ribs and pelvis.

Bilateral proptosis in Marshall-Smith syndrome

ABSTRACT Marshall-Smith syndrome (MSS) is an extremely rare developmental disorder characterized by accelerated skeletal maturation, failure to thrive, dysmorphic facies, respiratory difficulties, and neurological abnormalities. Herein, the authors report bilateral proptosis in a 10-year-old child with MSS. At present, there are limited reports on ophthalmologic considerations in MSS; therefore, this article subserves to broaden the clinical spectrum seen in this syndrome.

The Role of Thermal Camera in the Assessment of Thyroid Eye Disease Activity.

Thyroid eye disease (TED; also known as thyroid - associated orbitopathy, Graves ophthalmopathy) is an autoimmune inflammatory disease which presents in typical signs and symptoms such as deep orbital pain, chemosis with or without caruncular edema, unilateral or bilateral proptosis, eyelid retraction, eyelid edema or erythema, restrictive strabismus and compressive optic neuropathy. The aim of this study was to investigate the role of thermal camera in the assessment of thyroid eye disease (TED) activity compared to the Clinical Activity Score (CAS) scale, exophthalmometry values, and thyroid hormone and antibody levels. A total of 50 patients participated in this cross-sectional study of whom 29 were in the active phase of TED according to the sum on CAS scale and 21 patients in the inactive phase. The Flir E8® thermal camera was used to measure the temperature of the orbital area and the values were compared with the CAS scale, exophthalmometry values and thyroid hormone and antibody levels. Higher values of temperature (p>0.0001), CAS score (p>0.0001), exophthalmometry (p=0.022), FT4 (p=0.0176) and TRAb (p=0.0091) were found in patients in the active phase of TED. Temperature of orbital area showed statistically significant positive correlation with CAS scale (p=0.0001), exophthalmometry values (p=0.0022) and anti-TPO levels (p=0.019). Thermal camera showed higher values of the temperature of the orbital area in patients in the active phase of the disease and positively correlated with the CAS scale, exophthalmometry findings and anti-TPO levels.

A case of thyroid associated ophthalmopathy

Thyroid-associated Ophthalmopathy (TAO) is an ocular condition that frequently manifests with thyroid disfunction, and is the most common extrathyroidal manifestation of Graves’ disease. It involves an organ specific autoimmune reaction that can affect the thyroid gland, orbital and periorbital tissue. The condition primarily affects women and it is the commonest cause of unilateral and bilateral axial proptosis in young and middle -aged adults. The clinical presentation may vary from mild disease to severe irreversible sight threatening complications. Here we report a case of 35-years old female who presented to our OPD with complaints of protrusion of both eye, diminution of vision in both eye, pain, redness and unable to move eye in the last 2 months. She was known case of hyperthyroidism in the last one year and on regular antihyperthyroid medication.

Transconjunctival internal decompression of the orbit in patients with endocrine ophthalmopathy: a retrospective analysis

BACKGROUND: The most characteristic manifestation of endocrine ophthalmopathy (EO) is the proptosis development in a patient. It is possible to correct the displacement of eyeballs by performing an orbital decompression surgery.&#x0D; AIM: To evaluate the clinical efficacy of treating patients with endocrine proptosis using transconjunctival internal decompression of the orbit.&#x0D; MATERIALS AND METHODS: The study included 86 orbits of 43 patients with bilateral proptosis. All patients underwent MSCT examination, proptosis was detected due to an increase in the soft tissue component. Transconjunctival decompression of both eye sockets was performed according to the described method, and the amount of eyeball displacement after surgery was investigated.&#x0D; RESULTS: Patients complaints about constant pressure behind the eye, observed in 39 patients, disappeared during the first day after surgery in 21 patients, in the remaining patients they gradually disappeared within a week. In 32 patients with preoperative diplopia after surgery, in 27 it completely disappeared, in the remaining 5 it remained in the extreme positions. 6 months after surgery, a decrease in proptosis amount from 21.1 1.5 mm to 20.6 1.5 mm was noted, visual acuity was 1.0 0.02, decreased visual acuity remained in 3 cases due to the incipient cataract, IOP decreased from 20 1.2 mm Hg to 19 1.3 mm Hg. There were no eyeball movement restrictions at the control examination.&#x0D; CONCLUSIONS: Internal (soft tissue) decompression of the orbit is an effective method for proptosis correction exophthalmos in patients with lipid EO form. Carrying out the decompression surgery through the conjunctival access allows to constantly monitor the shape and size of the pupil in the operated orbit, to conduct controlled hemostasis of the orbital tissues. The use of preliminary calculations of the volume of soft tissues to be removed (according to MSCT) makes it possible to obtain a predictable effect of the surgery in variable degrees of proptosis. Transconjunctival decompression helps avoiding cicatricial processes of the eyelid skin.

LBODP030 A Rare Case Of Partial Lipodystrophy From Mpdl (mandibular Hypoplasia, Deafness, Progeroid, Lipodystrophy Syndrome) Due To Pold1 Gene Mutation

Abstract MDPL (Mandibular hypoplasia, Deafness, Progeroid, Lipodystrophy syndrome) caused by a mutation in the polymerase delta 1 (POLD1) gene is an extremely rare progeroid syndrome associated with partial lipodystrophy. Currently, there are about 24 cases reported worldwide(1), mostly in people of European descent. We are reporting one such rare case in a Hispanic patient. The patient is a 37 year old male from Honduras who was referred to us by genetics for diabetes management. Family members noted extremely thin limbs and he did not walk till 2 years of age. Family history was negative with 8 full siblings, all reportedly healthy. On examination, his BMI was 15.19 kg/m2. He was noted to have birdlike facies with mid-face hypoplasia, beaked nose, retrognathia, dental malocclusion, and protruding maxillary teeth. He also had bilateral proptosis. His extremities were thin, lacking subcutaneous fat and muscle bulk with distal joint contractures in elbow, hands and feet. Genitourinary showed low testicular volumes (&amp;lt;3 ml). Genetic testing revealed a recurring mutation in the active site of POLD1 gene, which is diagnostic for MDPL. His clinical presentation was classic with dysmorphism, deafness, lipodystrophy and metabolic abnormalities. Due to the rarity of the disorder, there is very limited data on management of these patients. A symptom-based management was recommended. In terms of his diabetes, it was diagnosed at age 33 when his HbA1c was 11.2 and he was started on insulin. Patients with this syndrome tend to have insulin resistance. C-peptide was 2.88 ng/ml (ref. range: 0.78 - 5.19 ng/mL) with glucose 160 mg/dL indicating adequate beta-cell reserve; GAD-65 and anti-pancreatic islet cell antibodies were negative. Insulin was weaned off and currently, his diabetes is being managed with diet and lifestyle changes. He was found to have elevated total cholesterol and triglycerides. Hepatic steatosis was seen on liver ultrasonography. He was started on fenofibrate and low-fat diet; lipid panel has normalized. His echocardiogram was normal. In addition, males are known to have hypogonadotropic hypogonadism however this patient had a low testosterone and high FSH and LH, indicative of primary hypogonadism. A scrotal ultrasound was ordered. DXA scan showed low bone mineral density with the lowest Z-score of -3.6 at the lumbar spine. He was started on testosterone replacement with Vitamin D. Testosterone therapy will help replace the testosterone deficiency in addition to slowing down bone loss. MDPL syndrome is an exceedingly rare clinical entity associated with multiple endocrinopathies. We plan to periodically monitor labs and imaging to continue managing this patient with a symptom-based approach. (1) Wang, Xiu-Wen; Lu, Ling-Yun; Xie, Ying1; Yu, Xi-Jie1 A Chinese girl with (MDPL) diagnosed via POLD1 mutation detection, Chinese Medical Journal: August 20, 2020 - Volume 133 - Issue 16 - p 2009-2011 Presentation: No date and time listed

RF35 | PSAT299 Thyroid Eye Disease Activation After mRNA SARS-CoV-2 Vaccination in Patients With Autoimmune Thyroid Disease

Abstract Background Occurrence of Graves’ disease (GD) has been reported following SARS-CoV-2 vaccine administration, but little is known about thyroid eye disease (TED) after SARS-CoV-2 vaccination. Case 1 A 50-year-old non-smoker male was diagnosed with GD in March 2019 and treated with methimazole. In April 2020, he developed TED with bilateral proptosis, pain, erythema and diplopia and clinical activity score (CAS) 6/7. Following poor response to 3-month oral steroids, he underwent total thyroidectomy in November 2020. In six weeks, his TED improved (CAS 4/7) and thyroid stimulated immunoglobulin (TSI) levels normalized from 4.13 IU/l (preoperatively) to 0.70 IU/l (reference&amp;lt; 1.75 IU/l). In January 2021, he received 2 doses of the mRNA BNT162b2-SARS-CoV-2 vaccine (Pfizer-BioNTech). Three weeks after the second dose, TED deteriorated significantly with severe eye pain, lid swelling, conjunctival erythema, and worsening proptosis and diplopia (CAS 6/7). TSI levels rose to 4.45 IU/l, while patient was euthyroid on levothyroxine (TSH 2.3 mIU/l). He received 12 weekly methylprednisolone infusions (cumulative dose of 4.5 g) with limited response. He was then treated with three monthly cycles of intravenous tocilizumab with partial response (CAS 5/7). Case 2 A 71-year-old non-smoker female had a 40-year history of hypothyroidism, controlled on levothyroxine. In March 2021, three days after her second dose of the mRNA-1273 SARS-CoV-2 vaccine (Moderna), she developed bilateral eye swelling and burning. She initially received anti-histamines and steroid eye drops without improvement. By August 2021, her eyes further deteriorated with eye pain, redness, lid edema and erythema, diplopia, and worsening proptosis (CAS 4/7). She also experienced 20 Ib weight loss, palpitations, tremors, and heat intolerance. TSI index was 5.5 (reference ≤1.3), TSH was undetectable, and FT4 1.4 ng/dl (reference 0.93-1.70 ng/dl). Levothyroxine was discontinued. Two weeks later she was admitted to the hospital with loss of color vision and decreased visual acuity in the right eye. She received 2 daily doses of 1 g intravenous methylprednisolone and intravenous teprotumumab on hospital day 3. She responded well to teprotumumab infusions every 3 weeks, with return of color vision and improvement of proptosis and periorbital edema (CAS 1/7). Discussion Activation of TED has so far only been described in four patients following SARS-CoV-2 vaccination. Here we report two more cases: one of TED deterioration in a GD patient with prior TED, and one of de novo TED progressing to dysthyroid optic neuropathy in a patient with a long history of Hashimoto's hypothyroidism. Possible etiologies include a maladaptive autoimmune/inflammatory syndrome from polyclonal B cell stimulation by adjuvants or activation of preexisting autoimmune disease. The possibility of new onset or deterioration of TED after SARS-CoV-2 vaccination in patients with underlying autoimmune thyroid disease and its mechanism should be further studied. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m., Monday, June 13, 2022 1:18 p.m. - 1:23 p.m.

PSAT357 Preventing Progression of Ophthalmopathy Using a 'Block-Replace' Treatment Strategy in Labile Graves’ Disease

Abstract Introduction Graves’ disease is an autoimmune disorder that most commonly causes hyperthyroidism. Pathogenesis usually involves the stimulation of TSH receptors by antibodies, but in rare cases can alternate between hyper- and hypothyroidism. In this case report, we describe a patient who had labile thyroid function with persistent ophthalmopathy, requiring a temporizing "block and replace" strategy for treatment. Case Description A 53-year-old woman with a history of GERD and fibroids presented to her primary care physician with a month-long history of hand and leg tremors, weakness, heat intolerance, palpitations and worsened anxiety. She also developed eye irritation and enlargement. Thyromegaly was noted on examination. Thyroid function tests showed that TSH was &amp;lt;0.01 (0.45-5.33 uIU/mL) and Free T4 was 2.48 (0.61-1.12 ng/dL). TRab was 21.2 (&amp;lt;1.75 IU/L). Thyroid ultrasound revealed heterogeneous enlargement with increased vascularity representing thyroiditis. CT orbit revealed mild bilateral ocular proptosis with normal extraocular muscle. She was diagnosed with Graves’ disease complicated by ophthalmopathy and she was started on methimazole and metoprolol. Repeat labs six weeks later improved to TSH of 0.01 and total T4 of 6.5 (4.50-11.70 ug/dL). Unfortunately, follow up labs 2 months later revealed overt hypothyroidism, with TSH of 134 with total T4 0.9. Methimazole and metoprolol were discontinued and 75mcg of levothyroxine was started. One month later, TSH was suppressed again to 0.11 and Total T4 was 17.8, so methimazole was again added with the intent of suppressing hyperthyroidism while simultaneously replacing levothyroxine to avoid hypothyroidism. Given the difficulty of maintaining a euthyroid state and ongoing symptomatic ophthalmopathy, she eventually underwent total thyroidectomy and started levothyroxine post operatively. Pathology of the thyroid gland was consistent with Graves’ disease. Discussion Graves’ disease is typically treated with anti-thyroid medical therapy (thioamides such as methimazole), which is titrated to a euthyroid state. In our case, the patient's thyroid function varied widely on methimazole, potentially representing variable stimulating and blocking functions of TRab. As periods of hypothyroidism can worsen ophthalmopathy, a "block and replace" treatment strategy was adopted, which involves a thioamide and levothyroxine concurrently to achieve a biochemically euthyroid state. While a Cochrane review has suggested there is no improvement in relevant outcomes using this approach, it was useful in our case as a bridge to surgical management1.

ODP533 Graves’ Ophthalmopathy in the Setting of Primary Hypothyroidism

Abstract Background Graves’ ophthalmopathy (GO) is commonly associated with hyperthyroidism secondary to Graves’ disease (GD). Although rare, there have been case reports of it occurring in patients who are hypothyroid with underlying Hashimoto's thyroiditis (HT), as well as in euthyroid patients. It is thought that the mechanism behind developing GO in these patients is due to the presence of stimulating thyrotropin receptor antibodies (TRAB). Below we describe a case of GO developing in a patient who has hypothyroidism secondary to HT. Clinical Case A 53 years-old female non-smoker, known to have primary hypothyroidism (Hashimoto's thyroiditis) diagnosed at the age of 39 years, on levothyroxine 75 mcg/day. Presented to our endocrine clinic complaining of diplopia and periorbital swelling for the past 5 months. The diplopia occurs on left gaze while both eyes are open, it resolves with closure of either eye. No history of ocular surgeries, injections, or trauma. No previous hyperthyroid state or radioactive iodine therapy. On physical examination there was no goiter, bruit, palpable lymphadenopathy, tremor, or pretibial myxedema. Ophthalmic examination showed left sided upper and lower eyelid swelling, limited abduction with diplopia and mild punctate keratopathy. Laboratory investigation revealed TSH 3.6 mIU/L (0.250 - 5), T4 14.6 pmol/L (11.5 - 22.7), positive thyroid peroxidase antibody 173.9 units (&amp;gt; 100 units positive) and positive thyroid stimulating immunoglobulin 500IU/ml (Normal &amp;lt; 140). On ultrasound the thyroid gland was small and heterogeneous. MRI orbits demonstrated bilateral ocular proptosis with intraocular muscles enlargement particularly the medial, inferior, superior rectus as well as the levator palpebrae superioris muscles. Our impression was active moderate-severe isolated GO with aBackground of HT, clinically and biochemically euthyroid on levothyroxine. The patient was referred to an ophthalmologist and was started on a course of high dose oral prednisone tapered over 3 months. She reported a 50% improvement in her symptoms after the first month of therapy. Patient was then started and maintained on selenium 100 mcg oral twice daily with lubricating eyedrops. During her last clinic visit she had stable thyroid eye disease and there was no need for further medical or surgical intervention. Conclusion GO can occur in patients with HT due to the development of stimulating TRAB targeting thyrotropin receptors expressed on orbital fibroblasts. The incidence varies between studies from 2-7.5%. Awareness of this clinical presentation is important, as early detection and treatment can prevent visual complications. To date there are no clear guidelines on how to treat GO with underlying HT. Treating our patient with high dose steroids extrapolated from treating GO secondary to GD showed significant improvement in her symptoms. Presentation: No date and time listed

RF30 | PSAT135 Rare Case of Hypercalcemia Mediated by an Ectopic Parathyroid Hormone (PTH), and Parathyroid Hormone-Related Peptide (PTHrP) Co-Secretion Secondary to Metastatic Cervical Carcinoma

Abstract Introduction Hypercalcemia of malignancy occurs in ∼20-30% of cancer patients. This mostly occurs by three mechanisms: humoral mediated from excess production of PTHrP, osteoclast mediated bone resorption from osteolytic metastasis and calcitriol mediated in granulomatous diseases such as lymphoma. Very rarely, PTH mediated hypercalcemia can occur in the setting of ectopic PTH production from tumor cells. Case presentation A 57 year- old woman with a medical history of hypertension and hyperthyroidism presented to the emergency department with several days of fatigue, nausea, vomiting, constipation and abdominal pain. Exam was remarkable for a heart rate (HR) of 140, lethargy, dry mucous membranes and chronic bilateral proptosis. Labs were remarkable for corrected calcium level of 18.38 mg/dl, Phosphorous of 3.2 mg/dL, Mg level of 1.2 mEq/L, blood urea nitrogen (BUN) of 20 mg/dL, creatinine of 1.6 mg/dL alkaline phosphatase of 170 units/L, and Lactic acid was 3.0 mmol/L. TSH 0.291 (0.40-4.7), FT4 1.51 ng/dl (0.70-1.80), FT3 3.2 pg/ml (2.8-5.3). Patient was treated with IV fluids, calcitonin 4u/kg bid, zoledronic acid 4 mg and started on cinacalcet. Further workup revealed elevated PTH of 1351 pg/ml (16-80), PTH-rP of 28 (&amp;lt;2), 25-hydroxyvitamin D of 25.5 ng/ml (30-100), 1,25-dihydroxy vitamin D level of 59.8 pg/ml (19.9-79.3). Patient couldn't tolerate sestamibi scan. 4DCT couldn't be performed due to creatinine elevation Bedside US performed did not show any evidence of parathyroid adenoma. CT, abdomen and pelvis a large mass in the cervix and cecum. Cervical biopsy revealed necrotic tumor with vary rare nests of viable atypical cells. Given persistent hypercalcemia, despite additional treatment, CRRT was initiated on hospital day 15 with rapid normalization of calcium level. However, patient continued to rapidly deteriorate with multi-organ failure and was transitioned to comfort care before expiring. Autopsy revealed poorly differentiated necrotic cervical carcinoma measuring 12×6×8 cm with metastasis to the left ovary as well as multiple tumor lesions in the liver. Parathyroid glands were normal on gross exam. PTH staining could not be performed given degree of tumor necrosis Discussion Hypercalcemia with elevated PTH and negative localization studies, should raise suspicion for ectopic PTH production from underlying malignancy. In PTHrP mediated hypercalcemia, PTH as well as 1,25 (OH) D are expected to be suppressed. This patient had an elevated PTHrP but not enough to suppress PTH or 1,25 (OH) D level. Given that parathyroid glands were normal on gross exam; this is consistent with ectopic PTH mediated hypercalcemia with component of PTHrP co-secretion. Hypercalcemia of malignancy presents late and portends a poor prognosis and therefore appropriate diagnosis and management is critical. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m., Monday, June 13, 2022 12:42 p.m. - 12:47 p.m.

ODP590 Combined Orbital Irradiation and Systemic Steroids in the Management of Progressive Thyroid Eye Disease

Abstract We present a case of a 62-year-old female with Multinodular Toxic Goiter who later developed Grave's Ophthalmopathy. She had an elevated baseline TRAb with an orbital MRI that showed bilateral proptosis with enlarged bilateral medial and lateral recti and extraocular muscles of the superior complex. She was initially treated with oral steroids which led to resolution of her visual complaints. However, due to recurrence of an active ophthalmopathy, she was subsequently given pulsing corticosteroid therapy. This was followed by radiotherapy due to minimal improvement in double vision. After completion of treatment, inflammatory ophthalmologic signs and symptoms were significantly improved. Radiotherapy in combination with glucocorticoids, particularly in the presence of diplopia or restriction of extraocular motility, should be considered in the management of active thyroid associated orbitopathy. Presentation: No date and time listed

Infrequent Bilateral Orbital Tumors

A 44-year-old woman presented with a 1-year history of progressive, painless bilateral proptosis. Magnetic resonance imaging revealed well-circumscribed, enhancing, central masses (Fig A-B). Right mass resection was performed via an inferior fornix incision. Left mass resection was performed via a lateral orbitotomy. Histopathology showed the right-sided mass to be rich in vascular sinuses, lined with flat endothelial cells separated by fibrous intervals (characteristic of cavernous hemangioma, Fig C).

Bilateral Orbital Inflammation Associated with COVID-19 Infection: A Case Report and Brief Review of the Literature

A 31-year-old woman with no history of systemic autoimmune disease developed bilateral pain, redness, periorbital edema, and proptosis while being hospitalized for mild coronavirus disease 2019 (COVID-19) pneumonia. The onset of ocular symptoms occurred six days after systemic COVID-19 symptoms. Computed tomography examination of the orbit revealed diffuse inflammation involving multiple orbital structures. A complete systemic workup was performed to exclude other systemic inflammatory diseases. She was initially diagnosed with probable orbital cellulitis secondary to sinusitis. However, she did not improve with antibiotic therapy. Empirical administration of systemic corticosteroids led to a dramatic clinical response. The patient has been maintained on a gradual steroid taper without inflammation relapse. This article describes a potential causal association between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and orbital inflammation. Ophthalmologists should be aware of this presentation when assessing patients with COVID-19 who present with orbital inflammatory symptoms.