Orbital myositis in a patient with Crohn's disease: A case report of two episodes.

PURPOSE: To describe a case report of a 34 years-old patient with Crohn's Disease and two episodes of Ocular Myositis. METHODS: The research methodology employed in this study consisted of a retrospective review of the patient's complete medical history. RESULTS: Crohn's Disease is a chronic inflammatory bowel disorder known to be associated with a wide range of extraintestinal manifestations. Ocular abnormalities, such as episcleritis and uveitis, are commonly observed. However, orbital myositis is an extremely rare ocular extraintestinal manifestations characterized by acute ocular pain that worsens with eye movements and is often accompanied by diplopia. In this case report, we present the case of a 34-year-old woman with a confirmed diagnosis of Crohn's Disease, who experienced two episodes of acute orbital pain exacerbated by ocular movements and diplopia. The diagnosis was established through clinical evaluation and radiologic imaging, with confirmation after a good response to systemic corticosteroids. She responded favorably to systemic corticosteroid therapy on both episodes, and no additional treatment was required. As of now, she remains stable without any ocular sequelae. CONCLUSION: It is important to note that orbital myositis is an uncommon ocular manifestation associated with Crohn's Disease, and prompt recognition and management are crucial to achieve successful outcomes.

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.

Myositis of the superior oblique muscle in a patient with suspected superior oblique muscle palsy.

Orbital myositis comprises a subtype of idiopathic orbital inflammation. Symptoms and clinical signs include orbital pain, eyelid swelling, ptosis, and conjunctival chemosis, sometimes concurrent with diplopia. Orbital myositis typically occurs in an idiopathic acute form and affects one or more extraocular muscles. It mainly involves the rectus muscles; cases involving the superior oblique muscle have been rarely reported. We report the case of a 57-year-old man with sudden-onset diplopia. Initial examination was suggestive of right superior oblique muscle palsy; however, myositis of the right superior oblique muscle was confirmed on magnetic resonance imaging (MRI). The patient was started on intravenous steroid pulse treatment. The steroid was tapered for 4 months. Diplopia, exotropia, and excyclotorsion of the right eye disappeared after 3 weeks of treatment. Cranial MRI obtained 2 months after starting treatment showed a normal superior oblique muscle. There has been no recurrence with 8 months of follow-up after completing the steroid taper.

Efficacy of Infliximab in Disease Control of Refractory Orbital Myositis.

PURPOSE: Orbital myositis is a common cause of orbital inflammation with localized involvement of the extra ocular muscles. This study aimed to assess the value of infliximab in controlling orbital myositis and reducing its relapse rate. METHODS: We conducted a retrospective review of the medical records of all consecutive patients with orbital myositis treated with infliximab between 2007 and 2016. We examined change in corticosteroid and immunomodulatory doses as well as relapse rates following treatment with infliximab. RESULTS: The study included seven patients with an average follow up of 19 ± 13.4 months. The mean dose of systemic corticosteroid was reduced from 28.57 ± 14.35 mg/day at the time of infliximab initiation to 7.00 ± 6.83 mg/day at final follow-up (p = .003). Long-term remission was achieved in 85.7% (n = 6). CONCLUSION: This study supports the role of infliximab in treating refractory orbital myositis and this was associated with clinical improvement, decreasing relapse rate with dose reduction of conventional treatment.

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.

Diagnostics and treatment of orbital myositis.

Objective: Orbital myositis is a rare clinical condition that involves idiopathic inflammation mostly of extraocular muscles. The purpose of this study was to present a diagnostic and treatment plan of orbital myositis. Methods: A 60-year-old female presented with decreased visual acuity on her left eye, ocular hypertension, restricted and painful left abduction, diplopia, swollen eyelids, and orbital discomfort. MRI, as well as ultrasound, showed enlargement in width of medial rectus muscle. After other diagnoses were excluded, the diagnosis of left orbital myositis was established. Results: She was started on systemic corticosteroid treatment, but each time the steroid dose was tapered she experienced a relapse. Immunosuppressive therapy was introduced and the steroid dose was gradually tapered and excluded. One year after immunosuppressive therapy, the clinical findings improved. Conclusion: The diagnosis of orbital myositis requires detailed examination, laboratory testing and MRI scans of the orbits in order to exclude other diseases with similar clinical findings. The first line treatment option is systemic corticosteroid therapy with additional immunosuppressive therapy if needed. Abbreviations: MRI = magnetic resonance imaging, BCVA = best corrected visual acuity, ENT = ear, nose, throat specialist, CBC = complete blood count, WBC = white blood cell, ESR = erythrocyte sedimentation rate, CRP = C reactive protein, HM = hand motion, TED = thyroid eye disease, SLE = systemic lupus erythematosus, ECG = electrocardiogram, CT = computed tomography.

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.

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.

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.

Myositis of an Extraocular Muscle, a Possible Drug Reaction: Histopathologic and Immunopathologic Analysis.

A 58-year-old man presented with left-sided orbital inflammation, including chemosis and a lateral rectus abduction defect. Initially presumed to represent cellulitis, the condition responded poorly to oral and intravenous antibiotics. CT showed the epicenter of an infiltrate to involve the lateral rectus. The patient improved dramatically when oral prednisone was added. Lateral rectus biopsy displayed intramuscular polyclonal lymphoid infiltrates, rich with eosinophils. Complete resolution of the inflammatory process was confirmed by a follow-up CT. The presumptive diagnosis was idiopathic orbital myositis, an uncommon condition of unknown etiology. However, the patient had taken rosuvastatin, which has been rarely associated with diplopia and ophthalmoplegia, raising the question of whether this case was truly idiopathic.

Atypical Cogan Syndrome Featuring Orbital Myositis and Dacryoadenitis.

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.

Histopathologic Findings in Idiopathic Orbital Myositis.

PURPOSE: To report the histologic and clinical features of idiopathic orbital myositis (IOM) patients who underwent extraocular muscle (EOM) biopsy over 20 years, to provide the first methodical reference to the expected histopathologic findings, and to discuss the histopathologic differences from common differential diagnoses. DESIGN: Cohort study. PARTICIPANTS: All patients with a diagnosis of IOM who underwent EOM biopsy from 2000 through 2019 were included. Patients who had a different final diagnosis were excluded. METHODS: Tissue samples of EOM and medical records of all participants were reviewed. MAIN OUTCOME MEASURES: Histopathologic features of muscle biopsy, including tissue morphologic features and cellular composition. RESULTS: Thirteen patients met both inclusion and exclusion criteria, and their tissue samples were revisited. Nine patients showed histopathologic findings that suggested a conclusive diagnosis of IOM, and the study focused on them. The average age at presentation was 49 years, and 66.7% of patients were women. The most commonly biopsied EOM was the medial rectus (44.4%). The most common indications for biopsy were nonresolving orbital disease with inadequate response to corticosteroids (44.4%) or a high suspicion of malignancy because of known pre-existing systemic malignancy or the presence of an atypical orbital mass in addition to enlarged muscles (44.4%). The histopathologic findings that suggested a diagnosis of IOM were splaying of muscle fibers by inflammatory infiltrates (n = 9) and mild fibrosis (n = 8) in the endomysium or replacing muscle fibers, with no granulomas or vasculitis. The inflammatory infiltrates identified were of chronic inflammatory cells, consisting of lymphocytes (n = 9), plasma cells (n = 6), and histiocytes (n = 6). Other less commonly identified cells were eosinophils (n = 4), polymorphonuclears (n = 1), and giant cells (n = 1). Muscle fiber degeneration or regeneration was evident in 5 patients. Four patients from the initial cohort showed inconclusive histologic findings on revision and were reassigned as suspected IOM. CONCLUSIONS: The histopathologic features of involved muscles in IOM resemble those seen in idiopathic orbital inflammation and differ from those seen in common differential diagnoses. Extraocular muscle biopsy should be strongly considered whenever the presentation of orbital myositis is not typical or when significant underlying conditions are a possibility.