Acute Bilateral Orbital Myositis Following Covid 19 Vaccination.

The authors present a case of acute bilateral orbital myositis occurring 24 hours after the administration of the mRNA1273 vaccination for COVID 19. The patient was presented with right proptosis, with orbital imaging demonstrating bilateral enlargement of all the extraocular muscles. Serological investigation did not reveal a precipitating cause or underlying disease process. The presenting features resolved entirely following treatment with methylprednisolone and the patient remains asymptomatic.

Orbital inflammation following COVID-19 vaccination: A case series and literature review.

PURPOSE: The purpose of the study was to report three cases of orbital inflammation following administration of the COVID-19 vaccination, manifesting as Tolosa-Hunt syndrome (THS) and orbital myositis. METHOD: A retrospective case series and literature review of patients who developed orbital inflammation following a COVID-19 vaccination. RESULTS: One patient presented with Tolosa-Hunt syndrome (THS) 14 days following her third (booster) COVID-19 vaccination, one patient developed orbital myositis 10 days following his first COVID-19 vaccination and one patient developed recurrent orbital myositis 1 and 7 days following her second and fourth COVID-19 vaccination. All patients received the Comirnaty vaccine (Pfizer-BioNTech). A thorough systemic autoimmune disease workup in both patients was unremarkable. Two patients had a prior history of orbital inflammation, with previous involvement of other different orbital structures. Characteristic MRI features for each pathology were observed, supporting the clinical presentation of THS and orbital myositis. There was complete resolution of THS following corticosteroids, with no recurrence at 2 months. Meanwhile, one case of orbital myositis self-resolved at 2 months without use of systemic corticosteroids, while the other patient with orbital myositis required treatment with intra-orbital steroid injections and oral corticosteroids. CONCLUSION: Orbital inflammation has been recognised as a rare adverse effect following COVID-19 vaccination. We present a case series of THS and orbital myositis as varied presentations of this entity.

Orbital myositis in systemic lupus erythematosus: a case-based review.

Ocular complications occur in up to one-third of patients with systemic lupus erythematosus (SLE). Among them, orbital myositis (OM) is considered a rare manifestation that affects the extraocular muscles and causes pain and restriction with eye movement. We report a case of OM in a 48-year-old female with SLE and secondary Sjogren's Syndrome, who presented headache, periorbital edema, and painful ocular movements in both eyes, with no other systemic manifestations. An orbital magnetic resonance image revealed thickening of the right medial rectus and left lateral rectus muscles. Laboratory tests were normal and there was no further disease activity. The patient was treated with prednisone 1 mg/Kg/day with a resolution of symptoms. We found 13 additional cases of OM from our literature review (11 SLE patients and 2 with discoid lupus erythematosus). There was a female predominance in these cases with a mean age of 43.6 years (SD ± 16.9). Their main clinical features included eye pain, swelling, proptosis, diplopia, and limitations in extraocular muscles, while in most of them, there was no other active systemic manifestation. Treatment with steroids led to the complete resolution of symptoms in most of these patients. The available evidence suggests that it is essential to have a high index of suspicion for OM in SLE patients even when there is no systemic disease activity so that proper treatment is initiated early.

Bilateral Paraneoplastic Orbital Myositis Associated With Malignant Melanoma and Multiple Myeloma.

Paraneoplastic extraocular muscle enlargement has been reported in a small number of patients with cancers including breast or lymphoma, usually presenting with bilateral multiple muscle involvement. Such myositis may be autoimmune. Furthermore, orbital inflammation is a recognized complication of immune-modulation therapy used to treat melanoma, such as ipilimumab. Extraorbital myositis has been described in myeloma, and polymyositis in melanoma. We present a case of bilateral, asymmetrical extraocular muscle enlargement with spontaneous resolution in a patient with simultaneous new diagnoses of metastatic malignant melanoma and multiple myeloma. A similar episode 7 months before diagnosis also resolved spontaneously. The authors believe this to be the first reported case of paraneoplastic orbital myositis associated with multiple myeloma or untreated malignant melanoma.

Orbital inflammatory disorders: new knowledge, future challenges.

PURPOSE OF REVIEW: This review aims to bring together recent advances in basic, translational and clinical research on the pathogenesis and treatment of orbital inflammatory conditions. RECENT FINDINGS: Basic science studies provide mechanistic insights into why the orbit is targeted for inflammation by autoimmune inflammatory disorders. Using Graves' disease as a test case reveals that endocrine pathways, such as the TSH and IGF1 receptor pathways play important roles in stimulating orbital inflammation. Furthermore, orbital tissues contain high concentrations of retinoids - byproducts of the visual pathway that diffuse across the sclera and can activate de novo transcription of inflammatory cytokines. Such cytokine expression places the orbit in a hyper-inflammatory 'resting' state, prone to respond to any additional systemic or local pro-inflammatory signals. The HIF2A--LOX pathway appears important for orbital tissue fibrosis. Lastly, bench-to-bedside studies of the IGF1R pathway have led to an FDA-approved drug, teprotumumab that represents a novel treatment approach for Graves' orbitopathy. Unfortunately, high drug costs and misplaced insurance company 'step-therapy' policies may block patients from receiving therapy that can protect vision and improve quality of life. SUMMARY: Improved understanding of orbital inflammatory conditions has led to a new drug and promises additional breakthroughs. Translational research is successful, but requires time, resources, and patience.

Unilateral Ptosis, Proptosis, and Orbital Myositis After Shingles Vaccination.

Vaccinations for shingles are recommended for most adults over 60 years of age and are typically well tolerated. The present case describes acute onset of unilateral ptosis, proptosis, and orbital myositis developing within days after administration of shingles vaccination. The episode resolved to baseline after 1 week of treatment with steroids. To the authors' knowledge, this is first reported case of orbital inflammation following shingles vaccination. Given the temporal relationship and rapid response to treatment, this may represent an autoimmune reaction to the shingles vaccine.

Neuro-ophthalmic complications of IgG4-related disease.

PURPOSE OF REVIEW: IgG4-related disease (IgG4-RD) is increasingly recognized as a fibroinflammatory disease with a plethora of organ-specific manifestations but a particular predilection for head and neck tissues, including the nervous system. This review discusses general features and organ-specific presentations of IgG4-RD as well as treatment considerations, particularly emphasizing features of neuro-ophthalmic interest. RECENT FINDINGS: IgG4-RD is emerging as a common cause of several fibroinflammatory disorders in the head and neck that were previously considered idiopathic, such as sclerosing orbital pseudotumor, orbital myositis, hypophysitis, and hypertrophic pachymeningitis. New and unusual presentations continue to be described, including a number of vascular manifestations. Substantial progress has been made in elucidating the cell types involved in IgG4-RD, and new pathogenic models are being proposed. Although clinicopathologic correlation remains the cornerstone of diagnosis, ancillary tests such as flow cytometry for circulating plasmablasts and PET-computed tomography have high sensitivity, and certain radiologic features are recognized to be particularly suggestive, such as infraorbital nerve enlargement in IgG4-RD orbitopathy. IgG4-RD often responds to steroids but incomplete responses and relapses are common. Rituximab is emerging as a promising new therapy. SUMMARY: The current review summarizes manifestations of IgG4RD that are of particular relevance to neuro-ophthalmic practice.

Herpes Zoster Ophthalmicus With Orbital Findings Preceding Skin Rash.

The authors describe 2 patients who presented with orbital findings and later developed vesicular lesions that were positive for varicella zoster virus and consistent with Herpes Zoster ophthalmicus. One case is the first to involve dacryoadenitis and orbital myositis preceding disseminated Herpes Zoster. In the other case, a patient developed zoster orbital syndrome leading to elevated intraocular pressure, loss of vision, and afferent pupillary defect. Canthotomy and cantholysis were required to restore vision. In both cases, the orbital syndrome developed prior to the vesicular rash. These cases highlight the need to consider Herpes Zoster ophthalmicus in patients with orbital syndrome not responding to conventional treatment.

Orbital Myositis as Both a Presenting and Associated Extraintestinal Sign of Crohn's Disease.

Orbital myositis is a rare extraintestinal manifestation of inflammatory bowel disease and has been reported to occur at variable times relative to gastrointestinal symptoms, including years before, concurrently to, and years after the gastrointestinal diagnosis. Here, the authors report 3 cases of Crohn's disease associated orbital myositis, adding to the 18 previously reported cases. The authors describe 2 cases of middle-aged females presenting with orbital myositis during clinical remission of Crohn's disease. The authors also describe the first reported case of orbital myositis-associated Crohn's disease diagnosed prior to any gastrointestinal symptoms in a teenage male presenting with corticosteroid responsive intermittent eyelid swelling. These cases underscore the importance of the Ophthalmic Plastic Surgeon in initiating a thorough systemic workup for cases of orbital inflammatory disease.

Orbital myositis revealing a vegetal intraorbital foreign body: a case report.

INTRODUCTION: Foreign intraorbital bodies are rare. Their clinical manifestation is usually late. CLINICAL CASE:     We report a case of 32 years-old man presenting an old vegetal foreign intraorbital body. The orbitotomy allowed its extraction with favorable result. DISCUSSION: Vegetal foreign intraorbital bodies have clinical aspects. The latency between the trauma and the onset of symptoms is variable. Radiological examination can identify exactly the size and location. Surgical removal is often necessary but can expose to major risks. CONCLUSION: The diagnosis of ignored intraorbital foreign body remains difficult. The management should be early in order to avoid severe functional consequences.

Orbital myositis associated with celiac disease.

A 26-year-old female patient presented with redness, lid edema on the right eye and diplopia on left gaze. There was a minimal limitation of adduction of the right eye, accompanied with pain and diplopia. Orbital magnetic resonance imaging confirmed the diagnosis of orbital myositis. Anti-endomysial immunoglobulin (Ig) A, anti-endomysial IgG, anti-gliadin IgA and anti-tissue transglutaminase IgG antibodies were positive, while other tests for autoimmune diseases were unremarkable. The patient stated that her diarrhea had been relieved by a gluten-free diet. Methylprednisolone therapy (1 mg/kg) was started and on her next visit her complaints were relieved. Later, methotrexate (15 mg/week) was added to the therapy as the patient became steroid-dependent, and she has been attack-free for the last 3 months under methotrexate and low-dose methylprednisolone (4 mg/day) treatment. Associations between orbital myositis and various autoimmune diseases have previously been reported. We report here the first case of associated orbital myositis and celiac disease.

Infliximab Reverses Symptoms and May Protect from Developing Chronic Restrictive Ophthalmopathy in Children with Familial Orbital Myositis: A Case Report.

BACKGROUND: Orbital myositis is a rare sporadic eye disease associated with extraocular eye muscle inflammation. To date, there have been two reports of familial orbital myositis (FOM), which demonstrate partially penetrant autosomal dominant inheritance. CASES: We report six new Australian cases of FOM, four of whom extend one of the reported pedigrees, as well as a separate mother and daughter manifesting orbital myositis, which constitutes a third report of familial occurrence. We can confirm that the disease has onset in childhood, appearing to go into remission in adult life, and that the inflammation is corticosteroid-responsive. However, one patient went on to develop permanent diplopia in upgaze. We also report two children suffering chronic pain and diplopia who demonstrated complete resolution of symptoms with the anti-TNF-α monoclonal infliximab. CONCLUSION: Uncontrolled FOM in childhood may result in permanent extraocular eye muscle damage, while TNF-α blockade provides an excellent steroid-sparing effect.

Orbital myositis and scleritis after anti-SARS-CoV-2 mRNA vaccines: A report of three cases.

PURPOSE: To report three cases of ocular myositis and scleritis, bilateral scleritis and unilateral single muscle myositis after mRNA COVID-19 vaccination. METHODS: Case series of three patients who presented to the Orbit Outpatient Service of Fondazione Policlinico Universitario A. Gemelli with a history of unilateral proptosis, diplopia and pain, bilateral red eye and pain during eye movements and unilateral proptosis and inconstant diplopia respectively with onset 5-10 days after m-RNA COVID-19 vaccine. A thorough hematologic work up and orbital contrast enhanced magnetic resonance imaging (MRI) in patients with proptosis was performed. RESULTS: Patients were females, 64, 58 and 45 years old respectively. MRI showed enlargement of all right rectus muscles, with both muscle belly and insertion involvement in the first case associated to right scleritis. A bilateral scleritis was diagnosed in the second patient and a single muscle myositis in the third patient. Serological tests excluded thyroid diseases. The first and second patient were treated respectively with oral and topical glucorticoids with a complete clinical response. Two 2 cycles of oral non-steroidal anti-inflammatory drugs were administered to the third patient with a partial response. CONCLUSION: As far as we know these are the first report of orbital myositis and scleritis presenting after mRNA BNT162b2 vaccine (Pfizer/BioNTech) and mRNA-1273-(Moderna) vaccine, an uncommon effect of a likely autoimmune reaction triggered by the virus antigen.

Bilateral diffuse orbital myositis in a patient with relapsing ulcerative colitis.

Ophthalmic manifestations of inflammatory bowel disease are common, but orbital inflammatory disease is rare with most cases associated with Crohn disease. We describe the first case of bilateral, diffuse, orbital myositis associated with ulcerative colitis in a patient taking infliximab. Within 72 hours of intravenous methylprednisolone treatment, the orbital inflammation dramatically improved. After 8 weeks of prednisone, it completely resolved and has remained quiet for 13 months. This is only the third report of ulcerative colitis-associated orbital inflammatory disease.

A unique case of foreign-body associated orbital myositis.

A 50-year-old woman presented with a 2-week history of diplopia and right-sided orbital pain with eye movement. Examination revealed an edematous, ptotic right upper eyelid with conjunctival hyperemia, proptosis, and significant limitation to upward and downward ductions on the right. MRI was significant for a homogeneously enhancing lesion within the superior rectus muscle. A laboratory evaluation seeking an infectious, inflammatory, or autoimmune process was nonrevealing. A diagnosis of orbital myositis was made, and the patient experienced significant improvement with oral corticosteroids. The patient's symptoms, however, recurred after attempts at a slow taper of the corticosteroids. An orbital biopsy of the lesion revealed fibroadipose tissue containing irregularly shaped yellow-white deposits birefringent under polarized light, suggestive of silica crystals. The patient denied any history of trauma or prior surgery. An intraorbital triamcinolone injection to the superior orbit allowed resolution of symptoms and a successful taper off systemic corticosteroids.

[A case of pulmonary tuberculosis associated with orbital myositis].

Ocular tuberculosis is rare. We report a case of orbital myositis suspected to be infected with tuberculosis. In January 2008, a 34-year-old man experienced discomfort in the right eye. In May 2008, this patient developed right exophthalmos, diplopia, and pain in the right eye, and he was diagnosed with idiopathic orbital myositis. The patient underwent 2 courses of steroid pulse therapy; after which, the dosage of steroids was reduced. The steroid treatment reduced the eye pain, but his diplopia and exophthalmos persisted. By November of the same year, his general malaise had increased, and chest X-ray radiography and computed tomography were performed on 3rd December. On the basis of the imaging results, we suspected active pulmonary tuberculosis of the right upper lobe. The smear made by using the sample obtained after bronchial brushing was negative for acid-fast bacilli, but a Mycobacterium tuberculosis nucleic acid amplification test of the post-bronchoscopic sputum yielded positive results. Therefore, the patient was diagnosed with pulmonary tuberculosis. After the 2HREZ/7HR regimen of treatment, the extent of the tuberculosis lesions of the lung was reduced and the exophthalmos and eye pain were alleviated. Orbital myositis is inflammation of the extraocular muscles and can be either idiopathic, without a known etiology, or secondary to conditions such as tuberculosis, sarcoidosis, or hyperthyroidism. Our patient was not definitively diagnosed with tuberculosis of the eye. A definitive diagnosis of tuberculosis of the eye would require detection of granulomatous lesions in the eye or isolation of Mycobacterium tuberculosis by puncturing the eye muscles; however, our findings suggested the possibility that it was secondary to tuberculosis. We think that a careful examination of the chest should be performed for patients with ocular abnormalities.

Pseudo-Duane retraction syndrome after orbital myositis.

Orbital myositis is a rare, commonly idiopathic, inflammatory condition that affects one or more extraocular muscles. We present a case of unilateral orbital myositis affecting the lateral rectus muscle presenting with gaze-evoked amaurosis, pain, and diplopia, with restrictive limitation of adduction. With improvement in adduction after initiating treatment, we noted narrowing of the palpebral fissure on attempted adduction, mimicking Duane retraction syndrome (DRS). Reported cases of "pseudo-DRS" are associated with multiple etiologies and are characterized by retraction on attempted abduction rather than adduction, as occurs in true DRS. In this case, pseudo-DRS occurred in the setting of idiopathic orbital inflammatory syndrome (orbital myositis) with a motility pattern more consistent with true DRS.

New Onset of Unilateral Orbital Myositis following Mild COVID-19 Infection.

Purpose: The authors present a case of unilateral orbital myositis of new onset following COVID-19 without a severe course.Methods: The patient had been received topical treatment with a preliminary diagnosis of conjunctivitis but no recovery had been noticed. The history revealed that the ocular signs had started 1 week after the COVID-19.Results: The examination revealed sectoral hyperemia of the temporal region in the bulbar conjunctiva together with marked limitation of right inward gaze. MRI of the orbits demonstrated diffuse fusiform enhancing enlargement of the right lateral rectus and superior rectus. The results of the laboratory tests and examination findings were normal. Systemic corticosteroids were started for the orbital myositis.Conclusions: Although conjunctivitis is the more common ocular disease following COVID-19, the possibility of orbital myositis should be considered in cases with resistance to topical treatment and/or gaze limitation. The possible role of orbital myositis as a trigger for COVID-19 could be explained with an immune-mediated mechanism.

Orbital compartment syndrome as the first manifestation of SLE.

The occurrence of orbital compartment syndrome is a rare and unusual complication of systemic lupus erythematosus (SLE). Patients will present with symptoms of ocular pain and diplopia and visible signs of proptosis. The condition is considered an ophthalmological emergency because the myositis involving the ocular muscles can cause irrevocable damage to the retina and optic disk, potentially leading to blindness. We report a case of a young African American male who developed orbital myositis with compartment syndrome as his initial manifestation of SLE. The patient underwent an emergent orbital decompression, followed by prompt initiation of immunosuppressant medications. To our knowledge, this is the first report of a case of myositis secondary to SLE complicated by compartment syndrome of the orbit.