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.

Isolated primary amyloidosis of the inferior rectus muscle mimicking Graves’ orbitopathy / Amiloidose primária isolada do músculo reto inferior simulando orbitopatia de Graves

ABSTRACT The diagnosis of Graves’ orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves’ orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction. This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves’ orbitopathy. A 48-year-old man complained of painless progressive proptosis on the left side and intermittent vertical diplopia for 6 months. The diagnosis of Graves’ orbitopathy was entertained after magnetic resonance imaging revealing a markedly enlarged, tendon-sparing inferior rectus enlargement on the left side, and an autoimmune hypothyroidism was disclosed on systemic medical workup. After no clinical improvement with treatment, the patient was referred to an ophthalmologist and further investigation was performed. The presence of calcification in the inferior rectus muscle on computed tomography, associated with the clinical findings led to a diagnostic biopsy, which revealed amyloid deposition. This report emphasizes that a careful evaluation of atypical forms of Graves’ orbitopathy may be crucial and should include, yet with rare occurrence, amyloidosis in its differential diagnosis.

RESUMO O diagnóstico de orbitopatia de Graves usualmente é fácil de ser estabelecido. No entanto, doenças da órbita que simulam alguns sinais clínicos da orbitopatia de Graves podem levar à confusão diagnóstica, particularmente quando associada à alguma forma de disfunção tireoidiana. Relatamos a ocorrência rara de amiloidose localizada no músculo reto inferior em paciente com hipotireoidismo autoimune, que recebeu inicialmente o diagnóstico errôneo de orbitopatia de Graves. Paciente masculino, 48 anos, com queixa de proptose progressiva e indolor do lado esquerdo e diplopia vertical intermitente há 6 meses. O diagnóstico de orbitopatia de Graves foi considerado após a realização de ressonância magnética, que revelou aumento importante do músculo reto inferior esquerdo, sem acometimento do tendão, e uma propedêutica sistêmica detectou hipotireoidismo autoimune. Como não houve melhora com o tratamento clínico, o paciente foi encaminhado a um oftalmologista, que realizou nova investigação. A presença de calcificação no músculo reto inferior na tomografia computadorizada, associada aos achados clínicos, levou a uma biópsia da lesão, que demonstrou a deposição de material amiloide. Este relato enfatiza como uma avaliação minuciosa das formas atípicas de orbitopatia de Graves é essencial e deve incluir a ocorrência, embora rara, de amiloidose no diagnóstico diferencial da orbitopatia de Graves.

Espessamento do tendão muscular na oftalmopatia de Graves / Muscle tendon enlargment in Graves´ophthalmopathy

OBJETIVO: Estudar a frequência do espessamento do tendão muscular em pacientes com oftalmopatia de Graves buscando estabelecer correlações com as diferentes formas clínicas da doença. A diversidade clínica e laboratorial na oftalmopatia de Graves pode levar à confusão quanto ao diagnóstico, conduta e prognóstico. Os achados radiológicos variam desde o aumento isolado do tecido adiposo até o espessamento da musculatura extraocular, caracteristicamente poupando os tendões. Em 2004,no entanto, Ben Simon descreveu o espessamento do tendão muscular na oftalmopatia de Graves. MÉTODOS: Foram avaliados 20 pacientes, de ambos os sexos, com idades entre 20 e 80 anos, com formas clínicas designadas como :forma benigna (retração palpebral),forma intermediária (diplopia na posição primária do olhar) e forma maligna ou infiltrativa (sinais de comprometimento do nervo óptico).Todos os pacientes foram submetidos à tomografias computadorizadas de órbita. Os pacientes estavam eutiroideanos, há pelo menos um ano. Os padrões tomográficos foram estudados e divididos em dois grupos: com e sem espessamento dos tendões da musculatura extraocular. RESULTADOS: Uma relação estatisticamente significativa entre espessamento do tendão e a forma clínica intermediária foi encontrada (p <0,012). CONCLUSÃO: O espessamento do tendão extraocular, encontrado em 30 por cento dos pacientes com oftalmopatia de Graves, relaciona-se positivamente com a forma intermediária da doença, caracterizada pela presença de diplopia e que constitui um achado de grande valor clínico.

PURPOSE: The aim is therefore to study the frequency of tendon enlargment in Graves' ophthalmopathy, seeking to estabilish its clinical correlations. Clinical and laboratory diversity in Graves' Ophthalmopathy sometimes may mislead its diagnosis. Radiological findings are more reliable for the diagnosis of Graves' Ophthalmopathy. Since then, a number of patterns have been described. Extraocular muscle involvement in this pathology is considered as always sparing the tendons. In 2004, Ben Simon described extraocular muscles tendon enlargment in some patients with diplopia in Graves' orbitopathy. METHODS: 20 patients, aged between 20 and 80 years, of both sexes, designated as benign (eyelid retraction), intermediate(diplopia in primary sight position) and malignant or infiltrative(signs of optic nerve compromise) forms were evaluated by orbital tomography. All patients had already been euthyroidean for at least one year. Tomographic patterns were studied and divided into two groups: with or without extraocular muscle tendons enlargment. RESULTS: Statistically significant relationship was found between tendon enlargment and intermediate form (p<0.012). CONCLUSION: Extraocular tendon involvement present in 30 percent of the patients with Graves' ophthalmopathy, and is positively correlated to intermediary form of the disease, characterized by diplopia, a very important clinical landmark.

Lid swelling and diplopia as presenting features of orbital sarcoid.

Sarcoid is an idiopathic multisystem non-caseating granulomatous disease with protean clinical manifestations. In the eye, the common sites of involvement are the skin of eyelid, conjunctiva, uveal tract, retina, optic nerve and lacrimal gland.

A clinico-investigative profile in Graves' ophthalmopathy.

A clinico-investigative profile was studied in 30 patients with Graves' Ophthalmopathy (GO) (15 each with early and late). In accordance to the thyroid status 63.3% of patients were hyperthyroid and 36.7% euthyroid. There was slight female preponderence, with ratio being 1.5:1. Exophthalmometric readings were significantly high in GO patients as compared to controls. However, no significant diagnostic role of postural exophthalmometry was seen. Positional tonometery may have respectable place among the tests for early diagnosis of GO; however, it could not differentiate between hyperthyroid and euthyroid cases. Further the role of ultrasonography, if available could not be overemphasized.