Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

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.

Linfangioma orbitário: relato de caso / Orbital lymphangioma: case report

Descrevemos um caso de linfangioma orbitário em uma paciente de nove anos de idade que apresentava proptose à direita (Hertel= 29 mm), acompanhada de restrição da motilidade ocular, dor e perda visual decorrente de neuropatia óptica compressiva. A ressonância magnética demonstrou a lesão expansiva, localizada na órbita direita, de aspecto cístico, não infiltrativa, extraconal e com sinais sugestivos de hemorragia intralesional. Não houve melhora com corticoterapia oral. Foi, então, realizada punção via transconjuntival, com aspiração de 35 ml delíquido "cor-de-chocolate" (confirmado como hemorrágico pela citologia). Ocorreram exacerbações do quadro clínico, manifestadas por dor e piora da proptose, devido à hemorragia intralesional, optando-sepela exérese dos cistos orbitários, usando o acesso orbitário lateral (cantólise e retirada de parede orbitária lateral, a qual não foi recolocada para efeito de descompressão) e inferior (transconjuntival inferior), com resolução do quadro. CONCLUSÃO: Foi relatado um caso de linfangioma, uma doença desafiadora, de difícil tratamento, com potenciais complicações visuais e estéticas, além da possibilidade de recidivas frequentes.

A case of orbital lymphangioma in a 9 year-old female, with proptosis (Hertel= 29 mm), ocular motility restriction, pain and visual loss due to compressive optic neuropathy is described. A magnetic resonance imaging (MRI) exam showed an expansive cystic lesion in the extraconal space of the right orbit, non-infiltrating with intralesional hemorrhage. The patient had no improvement with steroid treatment. A transconjunctival puncture with 35 ml aspiration of a "chocolate color" fluid (which was confirmed by cytologic study as a hemorrhagic fluid) was performed. Exacerbations occurred after an intralesional hemorrhage (pain and increase of proptosis). The lesion was excised via lateral cutaneous approach (a lateral canthotomy and removal of the right orbital wall was performed done for decompression) and lower transconjunctival approach. CONCLUSION: Lynphangioma is a challenging disease and difficult to treat, with potential visual and cosmetic complications and the possibility of frequent recurrences.

Tumor orbitário como primeira manifestação clínica de mieloma múltiplo: relato de caso / Orbital tumor as the first clinical manifestation of multiple myeloma: case report

O acometimento da órbita pelo mieloma múltiplo é raro. Neste estudo, os autores descrevem um caso infrequente de proptose como primeiro sinal clínico do mieloma múltiplo. A presença de lesão orbitária expansiva associada a destruição do rebordo superior da órbita, notáveis à tomografia computadorizada, fez com que a diagnóstico de mieloma múltiplo fosse considerado. Aspectos diagnósticos e terapêuticos são discutidos.

Orbital involvement by multiple myeloma is rare. In this study, the authors report an unusual case of proptosis as the first clinical sign of multiple myeloma. The presence of an orbital lesion expanding and destroying the superior orbital rim, disclosed by computed tomography, lead us to consider multiple myeloma. Diagnostic and therapeutic aspects are discussed.

Orbital and para-orbital tumours--a 10 years retrospective study.

A total number of 631 cases of orbital and para-orbital tumours causing proptosis was analysed in a retrospective way in relation to age, anatomic location, histological and clinical profile. CT-scan was considered as the primary modality of investigation as it correctly delineates the extent of the tumour, integrity of bony structures and intracranial spread. Different modalities of surgery like lateral orbitotomy, medial orbitotomy, lateral rhinotomy and endoscopic excision, etc, had been performed depending upon the anatomical locations. Surgical approaches, histological types of various tumours are analysed in detail.

Case report of orbital lymphoma associated with polycystic disease: a coincidence?

The most common known extra renal malignancy associated with polycystic kidney disease (PKD) is of pancreatic origin. Here we report a case of orbital lymphoma in a patient with PKD. This might just be a co-incidence or there might be some genetic interaction.

Congenital unilateral ocular fibrosis syndrome secondary to benign congenital tumor.

Congenital unilateral fibrosis, blepharoptosis and enophthalmos syndrome is one of the rare forms of congenital fibrosis of extraocular muscles. The nature and cause of such unilateral inflammation leading to fibrosis is unknown. Prenatal orbital penetration has been proposed as a cause of this syndrome. We report a case of this rare syndrome associated with an adjacent sinus tumor.

T-Cell Lymphoma Presenting as Painful Ophthalmoplegia

PURPOSE: To present a case of peripheral T-cell lymphoma presenting as painful ophthalmoplegia. METHODS: A 61-year-old woman presented with a 2-week history of headache and eyeball pain. Examination showed mild exophthalmos, complete ophthalmoplegia, and ptosis of the left eye. Under the impression of nonspecific orbital inflammation, she was treated with oral prednisone with initial response. Two months later, she revisited the clinic with exacerbated symptoms. Anterior orbitotomy and incisional biopsy was performed for the inferior rectus muscle lesion. RESULTS: Histopathologic examination revealed an infiltrate of atypical lymphoid cells between degenerative muscle bundles. It was consistent with peripheral T-cell lymphoma. A metastatic workup was performed without any evidence of extraorbital tumor. The patient was recommended to be treated with chemotherapy, however, refused to take the treatment. The patient died of progression of the disease in a month. CONCLUSIONS: T-cell lymphoma in the orbit can present as painful ophthalmoplegia and take a rapid clinical course. The disease should be regarded as one of the differential diagnosis for painful ophthalmoplegia refractory to corticosteroid therapy.

Orbital neurofibrosarcoma: a case report.

Malignant peripheral nerve sheath tumours of orbit is rare entity. Only sixteen cases are reported in world literature. Bengin schwannomas of the orbit are found in 1.5 to 18 percent of patients with von Recklinghausen's disease (VRD) and form 1 percent of all orbital tumours1. The incidence of malignant transformation in VRD is varies from 5-15 percent of all the cases1.

Tumor benigno intraorbitario de la vaina nerviosa: neurilemoma intraorbitario con exoftalmos: presentación de un caso / Bening neural sheet neoplasm: neurilemoma with exoftalmos. Report of a case

Las causas de exoftalmos son múltiples. Este puede ser unilateral o bilateral. La etiología puede estar relacionada con alteraciones endocrinológicas, alteraciones reactivas o inflamatorias y neoplasias benignas o malignas. Entre las neoplasias benignas se encuentran las originadas en la vaina nerviosa y pueden ser neuromas, neurilemomas o neurofibromas. Estos últimos pueden ser compomentes de la enfermedad de Von-Recklinghausen. En el presente artículo presentamos un caso de tumor benigno de la vaina nerviosa, intraorbitario, unilateral, productor de exoftalmos.

Malformación vascular como causa de proptosis a propósito de un caso: revisión de laliteratura / Vascularmalfotmations as cause of proptosis, apropos of a case: review of the literature

Se presenta un caso de malformaciones vascular como causa de proptosis. Se detalla el caso clínico con resumen de la historia, ultrasonido, seguimiento y resultado anatomopatológico. Se discuten los tipos de tumores orbitarios

Bilateral proptosis due to metastatic Ewing's sarcoma of the orbit: fine needle aspiration cytology (FNAC) and histopathology of a case.

Bilateral proptosis due to metastatic Ewing's sarcoma is an extremely rare presentation and thus merits reporting. The role of fine needle aspiration cytology in the diagnosis is highlighted.

Superior orbital fissure syndrome as a presenting symptom in hepatocellular carcinoma.

A patient with superior orbital fissure syndrome caused by metastatic hepatocellular carcinoma is reported. She had painful ophthalmoplegia and decreased sensation along the first branch of the trigeminal nerve. Histological confirmation was done by biopsy of sternal metastases. The awareness of various neurological presentations apart from hepatic failure in this malignancy is emphasized.