Miositis orbitaria, una causa de oftalmoplejia dolorosa en pediatría / Orbital myositis, a cause of pediatric painful ophthalmoplegia

INTRODUCCIÓN: La miositis orbitaria (MO) es un proceso inflamatorio grave de etiología desconocida que compro mete los músculos extraoculares. La presentación en edad pediátrica es rara y con frecuencia afecta a más de un individuo de una familia, lo que sugiere algún grado de predisposición genética. OBJETIVO: Describir un caso de miositis orbitaria de presentación en edad pediátrica, sus características clínicas, y la utilidad de la imagen por resonancia magnética para la confirmación del diagnóstico. CASO CLÍNICO: Paciente femenina de 13 años que presenta cefalea aguda, dolor periorbitario derecho, exacerbado con los movimientos oculares y visión borrosa a quien se le realizaron estudios para miopatía tiroidea, enfermedades infecciosas, autoinmunidad y cáncer que fueron negativos. En la imagen por resonancia magnética se evidenció miositis del músculo recto medio derecho, sin evi dencia de neuritis óptica. Recibió tratamiento con glucocorticoides sistêmicos intravenosos seguido de esteroides orales con mejoría clínica completa. CONCLUSIONES: La MO tiene etiología desconocida, y puede tener un curso maligno. Dada su presentación clínica inespecífica, el estudio diagnóstico diferencial debe ser amplio, y su estudio debe considerar realizar resonanacia magnética. El inicio temprano del tratamiento con esteroides evita el daño permanente de los músculos extraoculares.

INTRODUCTION: Orbital myositis (OM) is a serious inflammation of extraocular muscles with unknown etiology. Pe diatric presentation is rare and often affects more than one individual in a family, suggesting a genetic predisposition. OBJECTIVE: To describe a pediatric case of orbital myositis, its clinical characteristics, and the usefulness of MRI for confirming the diagnosis. CLINICAL CASE: A 13-year-old female patient presenting with acute headache, right periorbital pain, exacerbated by eye movements, and blurred vision. We ruled out thyrotoxic myopathy, infectious diseases, autoimmunity, and malignancy. An MRI showed right medial rectus muscle myositis and no evidence of optic neuritis. She was treated with intravenous systemic glucocorticoids followed by oral steroids with complete clinical resolution. CONCLUSIONS: OM has unknown etiology and can present a malignant course. Due to its unspecific clinical presentation, a comprehensive differential diagnosis should be made and it should consider performing MRI. Early treatment avoids permanent damage of extraocular muscles.

Miositis orbitaria en adolescente / Adolescents´ orbital myositis

Introducción: La miositis orbitaria aguda es una afección inflamatoria de la musculatura extrínseca ocular muchas veces de causa desconocida. Forma parte de un grupo de afecciones inflamatorias orbitarias inespecíficas englobadas bajo el término de pseudo tumor orbitario. Afecta principalmente a las mujeres jóvenes. Objetivo: Describir el caso de una adolescente con miositis orbitaria aguda, afección inflamatoria poco frecuente en la edad pediátrica. Presentación del caso: Se trata de una adolescente de 14 años, mestiza, femenina, quien sufrió dengue por tres ocasiones poco tiempo antes de presentarse con dolor ocular del ojo izquierdo, fiebre elevada intermitente, protrusión ocular intermitente, vómitos, cefalea, decaimiento, pérdida de peso y de apetito. El ojo izquierdo presentaba edema del parpado superior y limitación a la abducción, musculatura ocular extrínseca: diplopía horizontal a mirada derecha a izquierda. El ultrasonido ocular informa presencia de imagen quística que no impresiona ser de contenido hemático. El resultado de la tomografía axial computarizada de órbita y cráneo simple fue compatible con diagnóstico de miositis orbitaria. Se indicó tratamiento inmediato con esteroides orales y se remitió al Instituto de Oftalmología Ramon Pando Ferrer para seguimiento. Conclusiones: La miositis orbitaria exige un alto índice de sospecha para un diagnóstico y tratamiento correcto. El dengue pudiera estar implicado en la etiología de la enfermedad(AU)

Introduction: Acute orbitary myositis is an inflammatory condition of the extrinsic ocular musculature, often of unknown cause. It is part of a group of non-specific orbital inflammatory conditions encompassed by the term of orbital pseudotumors. It mainly affects young women. Objective: To describe the case of a teenager with acute orbital myositis which an inflammatory condition not frequent in the pediatric ages. Case presentation: This is a 14-year-old, mixed-race, female patient, who suffered dengue three times before presenting ocular pain in the left eye, intermittent high fever, intermittent ocular protrusion, vomiting, headache, weakness, loss of weight and appetite. The left eye presents edema of the upper eyelid and limitation in abduction. The extrinsic ocular musculature presented: horizontal diplopia in right to left gaze. The ocular ultrasound informs: cystic image that does not impress to be of hematic content. A computerized axial tomography of the orbit and simple skull was performed, and the result was compatible with the diagnosis of orbital myositis. Immediate treatment with oral steroids was indicated and the patient was sent to the Ramon Pando Ferrer Ophthalmology Institute for follow-up. Conclusion: Orbital myositis requires a high index of suspicion for a correct diagnosis and treatment. Dengue seems to be involved in the etiology of the disease(AU)

Orbital myosits in a patient with Behçet's disease / Miosite orbitária em paciente com doença de Behçet

Abstract The etiological diagnosis of orbital myositis (OM) is a challenge. Although it has been associated with previous infectious disease, diseases with autoimmune background, thyroidopathies or with paraneoplasic syndrome, most of them still remain as idiopathic. We describe here a case of OM uncovering a Behçet disease that is considered a rare cause for this kind of eye involvement.

Resumo Trata-se de um caso de ceratite bilateral e simultânea por Acremonium relacionada ao uso intermitente e sem respeitar a lateralidade O diagnóstico etiológico da miosite orbitária (MO) é desafiador. Embora ela tenha sido relacionada com doenças infecciosas prévias, doenças associadas à autoimunidade, tireoidopatias ou síndromes para neoplásicas, a maioria delas ainda permanece como idiopática. Descreve-se aqui, um caso de MO revelando o diagnóstico de doença de Behçet, doença considerada como uma causa pouco comum para este tipo de envolvimento ocular.

A Case of Surgical Diagnosis and Treatment of Idiopathic Orbital Myositis with Sudden Vision Loss

PURPOSE: We report a case of idiopathic orbital myositis with sudden onset of unilateral visual loss and hypertrophy of the lateral rectus muscle at the apex of the orbit that was diagnosed using orbital biopsy and treated with intravenous corticosteroids. CASE SUMMARY: We examined a 20-year-old woman who complained of decreased visual acuity and binocular diplopia for 2 weeks. She showed 40-prism diopter left esotropia at the primary position. Her visual acuity with eyeglasses was 0.7 and 0.2 in the right and left eyes, respectively. Brain magnetic resonance imaging (MRI) revealed a round mass on the left lateral rectus muscle at the apex of the orbit with optic nerve compression. We planned orbital biopsy to exclude orbital lymphoma and other biopsy-requiring diseases. After 3 weeks, the visual acuity of her left eye decreased to 0.02. Repeated MRI revealed enlargement of all extraocular muscles on the left orbit. With biopsy showing several lymphocytes infiltrating muscle fibers, we made a diagnosis of idiopathic orbital myositis. Intravenous injection of corticosteroids was administered during hospitalization. After 2 weeks of corticosteroid therapy, the visual acuity of her left eye was improved to 0.63, the esotropia disappeared, and the enlargement of the left lateral rectus muscle was improved on orbital MRI. CONCLUSIONS: For non-specific idiopathic orbital myositis located at the apex of the orbit and enlargement of the extraocular muscle in a short period with decreased visual acuity, administration of intravenous injection of corticosteroids before orbital biopsy could help restore vision.

Orbital Myositis with Rapid Successful Treatment with Corticosteroids.

A 41-year-old Japanese female was admitted to our hospital with a history of right abducens nerve palsy, right gaze diplopia, right eye pain and double vision. Thyroid function, thyroid autoantibody levels, and tests for other pathologies were normal. Orbital contrast-enhanced short-TI Inversion Recovery-magnetic resonance imaging before treatment showed contrast-enhanced, severe lateral rectus and rectus superior muscle swelling in the right eye. We therefore diagnosed this patient as orbital myositis. Intravenous glucocorticoid pulse therapy with methylprednisolone (mPSL) was initiated. After prescribing a daily dose of 1,000 mg of mPSL three times a week, all symptoms, including physical abnormalities, disappeared. The patient was discharged on day 14 after hospitalization and was prescribed oral PSL (30 mg/day). This report indicates that early and initial adequate treatment with a high dose of mPSL is very effective for orbital myositis treatment. However, the possibility of recurrences must be always considered at subsequent follow-up.

Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report / Miosite do músculo reto medial como forma atípica de apresentação de linfoma tipo MALT: relato de caso

Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT). Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

Descrevemos um raro caso de miosite do músculo reto medial como forma atípica de apresentação de linfoma não-Hodgkin de células B tipo MALT. A anatomia patológica e imuno-histoquímica do músculo afetado confirmaram o diagnóstico definitivo do caráter neoplásico da doença. As miosites orbitárias têm como principal etiologia a oftalmopatia de Graves, porém diversas outras causas podem apresentar-se dessa forma. Sendo assim, as causas neoplásicas devem ser descartadas. O linfoma não-Hodgkin de células B tipo MALT é o tipo histológico mais comum de linfoma orbitário, as regiões mais frequentemente acometidas são a conjuntiva e glândula lacrimal. No entanto, pode apresentar-se com formas clínicas atípicas, acometendo outras regiões e tecidos.

Superior Rectus-Levator Palpebrae Complex Myositis Presenting as Isolated Painless Ptosis

No abstract available.

Density of Orbital Fat and Extraocular Muscle in Thyroid-Associated Myopathy and Idiopathic Orbital Myositis

PURPOSE: To perform and compare differential diagnosis of patients with thyroid-associated myopathy, idiopathic orbital myositis and normal controls based on orbital computed tomography. Orbital fat and extraocular muscle densities were quantified using Hounsfield Unit (HU) and their characteristics were compared and analyzed. METHODS: From February 2005 to January 2013, orbital computed tomography was performed on 90 eyes of 47 thyroid-associated myopathy patients, 18 eyes of 14 idiopathic orbital myositis patients and 280 eyes of 140 normal subjects. The average values of orbital fat and extraocular muscle densities were measured and compared using HU. The density differences between the patients with thyroid-associated myopathy and the normal group were analyzed by age, clinical activity score, ocular protrusion and disease duration. RESULTS: In the thyroid-associated myopathy group, orbital fat and extraocular muscle densities were -87.8 +/- 12.5 HU and 48.7 +/- 7.1 HU, respectively. In the idiopathic orbital myositis group, the orbital fat and extraocular muscle densities were 79.9 +/- 9.9 HU and 49.2 +/- 9.1 HU, respectively. There was a statistically significant lower result of orbital fat in the thyroid-associated myopathy group (p = 0.002), however, the extraocular muscle density did not show a statistically significant difference (p = 0.775). The orbital fat and extraocular muscle densities of the normal group were -79.0 +/- 11.2 HU and 54.3 +/- 6.3 HU, respectively. There were significantly lower results in both orbital fat and extraocular muscle densities in the thyroid-associated myopathy group than normal group (p = 0.000). In active cases and those accompanied by ocular protrusion, there was no significant difference in orbital fat density (p = 0.345 and p = 0.952, respectively), while extraocular muscle density significantly decreased (p = 0.007 and p = 0.003, respectively). CONCLUSIONS: A difference between the orbital fat and extraocular muscle densities in thyroid-associated myopathy and idiopathic orbital myositis could be quantitatively found using HU and orbital computed tomography.

Unilateral Ptosis Due to Isolated Levator Myositis

PURPOSE: To present a rare case of idiopathic orbital myositis involving levator palpebrae superioris. CASE SUMMARY: A 27-year-old male presented with a 1-week history of redness, discomfort, swelling, and drooping of his left upper eyelid. A computed tomography scan showed isolated enlargement of the right superior rectus/levator muscle complex. On examination, there was a left blepharoptosis, although eye movements were normal. The authors treated the patient with 3rd-generation cephalosporin; however, after 3 days, the symptoms did not improve. Subsequently, the patient was diagnosed with idiopathic orbital myositis and treated with oral corticosteroids for 1 month; the symptoms gradually resolved. CONCLUSIONS: Idiopathic orbital myositis is a subtype of nonspecific orbital inflammation primarily involving the extraocular muscles. Although the exact cause of orbital myositis is unknown, an immune-mediated pathophysiologic mechanism appears to be one of the causes. Medial rectus myositis is the most common, and isolated levator muscle myositis is very rare. The authors of the present study reported a case of orbital myositis involving the levator palpebrae superioris which should be considered a differential diagnosis of blepharoptosis with eyelid swelling.

A Case of Orbital Myositis Secondary to Systemic Lupus Erythematosus / 대한류마티스학회지

A 17-year-old girl with 12-year history of systemic lupus erythematosus (SLE) was presented with one month history of diplopia and headache. She had experienced acute cerebral infarction due to multiple cerebral arterial stenosis one year before, and fully recovered except right-side central facial nerve palsy. When she visited pediatric emergency room, ophthalmologic examination showed ophthalmoplegia of the left eye ball; limitation of medial gaze, supra-adduction and infra-adduction. Neurologic examination didn't show any newly developed neurologic defect. There was no newly developed intra-cranial lesion on the brain MRI. But, the brain MRI revealed irregularly enhanced thickened left medial rectus muscle, and that was compatible with orbital myositis. There was no definite evidence of infection or other autoimmune disease. Her condition responded to high dose intravenous methylprednisone therapy (1 g/day for 3 days) and continued oral prednisolone.

A Case of Idiopathic Orbital Myositis Involving All Extraocular Muscles of Both Eyes

PURPOSE: To report a case of idiopathic orbital myositis involving all extraocular muscles bilaterally after trauma and to review current literatures on the orbital myositis. METHODS: The idiopathic orbital myositis primarily involving the extraocular muscles is a subtype of nonspecific orbital inflammation. Pathophysiology of orbital myositis, although unknown, is likely to be an immune-mediated mechanism. A 17-year-old male presented with the orbital myositis involving all extraocular muscle after blunt head trauma. RESULTS: Treatment of the idiopathic orbital myositis with oral corticosteroid and radiotherapy has been reported to be ineffective in cases involving 4 extraocular muscles. We herein report an experience of treating a case of orbital myositis involving all extraocular muscles with intravenous corticosteroid and radiotherapy.

Clinical Study of the Idiopathic Orbital Myositis

The purpose of this study is to evaluate the clinical features and efficacy of therapeutic method of 14 pseudotumor patients with radiologically suspected orbital myositis. Retrospective analysis was performed to elucidate correlation with clinical finding, ocular symptom and sign, involved muscles, therapeutic response, and combined ocular diseases. Patients enrolled in this study were 5 men(35.7%) and 9 women(64.3%) (mean age 38.9 years). Two(14.3%) of 14 patients had bilateral disease, 9(64.3%) in the left eye, and 3(21.4%) in the right. The characteristic signs and symptoms included proptosis(35.7%), eyelid swelling(28.6%), periocular pain(28.6%), diplopia(14.3%), and blepharoptosis(7.1%). Radiologically, the medial and superior rectus muscles were affected in equal frequency(64.3%) as were the lateral and inferior rectus muscles(42.9%). Therefore, 11(71.4%) of 14 patients had enlargement of at least 2 muscles. Drug therapy such as ibuprofen, indomethacin, and prednisolone or radiation therapy of pseudotumor with the orbital myositis was less effective in case of patients with the longer duration of myositis than 1 year or involved all the rectus muscles. These patients usually accompany optic neuropathy(35.8%), paranasal sinusitis(28.6%), ocular hypertension(28.6%), scleritis(14.3%), keratitis(14.3%), dry eye(14.3%), and dacryoadenitis(7.1%). In view of the results of this study, when a case presents clinically suspected pesudotumor with the inflammatory reaction or enlargement of extraocular muscle on CT or MRI, it is suggested that the early differential diagnosis, such as orbitoathy of Graves` disease, neoplastic disorder, ateriovenous malformation be made, and that proper treatment of the orbital myositis be institued for good prognosis of disease.

Clinical Study of the Idiopathic Orbital Myositis

The purpose of this study is to evaluate the clinical features and efficacy of therapeutic method of 14 pseudotumor patients with radiologically suspected orbital myositis. Retrospective analysis was performed to elucidate correlation with clinical finding, ocular symptom and sign, involved muscles, therapeutic response, and combined ocular diseases. Patients enrolled in this study were 5 men(35.7%) and 9 women(64.3%) (mean age 38.9 years). Two(14.3%) of 14 patients had bilateral disease, 9(64.3%) in the left eye, and 3(21.4%) in the right. The characteristic signs and symptoms included proptosis(35.7%), eyelid swelling(28.6%), periocular pain(28.6%), diplopia(14.3%), and blepharoptosis(7.1%). Radiologically, the medial and superior rectus muscles were affected in equal frequency(64.3%) as were the lateral and inferior rectus muscles(42.9%). Therefore, 11(71.4%) of 14 patients had enlargement of at least 2 muscles. Drug therapy such as ibuprofen, indomethacin, and prednisolone or radiation therapy of pseudotumor with the orbital myositis was less effective in case of patients with the longer duration of myositis than 1 year or involved all the rectus muscles. These patients usually accompany optic neuropathy(35.8%), paranasal sinusitis(28.6%), ocular hypertension(28.6%), scleritis(14.3%), keratitis(14.3%), dry eye(14.3%), and dacryoadenitis(7.1%). In view of the results of this study, when a case presents clinically suspected pesudotumor with the inflammatory reaction or enlargement of extraocular muscle on CT or MRI, it is suggested that the early differential diagnosis, such as orbitoathy of Graves` disease, neoplastic disorder, ateriovenous malformation be made, and that proper treatment of the orbital myositis be institued for good prognosis of disease.

A Case of Oribital Myositis / 대한류마티스학회지

No abstract available.

Effect of Corticosteroid on Orbital Pseudotumor Caused by Orbital Myositis

Idiopathic orbital myositis, a subgroup of inflammatory orbital pseudotumor may occur with acute-onset periorbital pain, diplopia, and, in most cases, eyelid swelling. Proptosis, extraocular muscle motility restrictions, and response to oral prednisolone administration were characteristic. Although the CT appearance of orbital myositis is often helpful, the findings are not pathognomonic: correlation with history, clinical finding, and therapeutic response must be considered in making the diagnosis. The results of corticosteroid therapy on the orbital pseudotumor caused by orbital myositis were as follows: 1. The effect of corticosteroid was more effective in single extraocular muscle involvement than in that of a multiple extraocular muscle. 2. Early age of onset and early treatment after symptom increased the effectiveness of corticosteroid therapy.