Differential Diagnoses of Diseases Involving the Extrinsic Ocular Musculature ­ A Pictorial Essay

Introduction There are some inflammatory, infectious, and neoplastic diseases affecting the extrinsic orbital musculature (EOM) that present with pain, decreased visual acuity, and proptosis. Imaging is fundamental to the differential diagnoses of these diseases with similar clinical presentations. The present case series report has as main objective to illustrate and discuss the main pathologies that affect the orbit. Material and Methods The present series of cases discusses the main pathologies that can affect the extraocular musculature that can be characterized by computed tomography (CT) or magnetic resonance imaging (MRI) using cases from our institution. Results and Discussion The present study compiled several cases of ophthalmopathy from our institution to illustrate and address some of these pathologies, such as orbital lymphoma, Grave disease, metastases, periorbital cellulitis, and idiopathic orbital inflammatory syndrome. The diseases are discussed according to the presentation of clinical cases with emphasis on the main imaging findings of each pathology. Conclusion Computed tomography and MRI can help in the diagnosis and follow-up of the diseases that affect the EOM. We must be conversant with the main characteristics of the pathologies presented in the present case series report, since such findings together with clinical data can confirm the diagnosis of these diseases or at least help to narrow the differential diagnoses.

Expanding the limits of endoscopic intraorbital tumor resection using 3-dimensional reconstruction / Expandindo os limites da ressecção endoscópica de tumor intraorbital utilizando reconstrução tridimensional

Abstract Introduction: Endoscopic orbital surgery is a nascent field and new tools are required to assist with surgical planning and to ascertain the limits of the tumor resectability. Objective: We purpose to utilize three-dimensional radiographic reconstruction to define the theoretical lateral limit of endoscopic resectability of primary orbital tumors and to apply these boundary conditions to surgical cases. Methods: A three-dimensional orbital model was rendered in 4 representative patients presenting with primary orbital tumors using OsiriX open source imaging software. A 2-Dimensional plane was propagated between the contralateral nare and a line tangential to the long axis of the optic nerve reflecting the trajectory of a trans-septal approach. Any tumor volume falling medial to the optic nerve and/or within the space inferior to this plane of resectability was considered theoretically resectable regardless of how far it extended lateral to the optic nerve as nerve retraction would be unnecessary. Actual tumor volumes were then superimposed over this plan and correlated with surgical outcomes. Results: Among the 4 lesions analyzed, two were fully medial to the optic nerve, one extended lateral to the optic nerve but remained inferior to the plane of resectability, and one extended both lateral to the optic nerve and superior to the plane of resectability. As predicted by the three-dimensional modeling, a complete resection was achieved in all lesions except one that transgressed the plane of resectability. No new diplopia or vision loss was observed in any patient. Conclusion: Three-dimensional reconstruction enhances preoperative planning for endoscopic orbital surgery. Tumors that extend lateral to the optic nerve may still be candidates for a purely endoscopic resection as long as they do not extend above the plane of resectability described herein.

Resumo Introdução: A cirurgia orbital endoscópica é um campo emergente e são necessárias novas ferramentas para auxiliar no planejamento cirúrgico e determinar os limites da ressecabilidade tumoral. Objetivo: Usar a reconstrução radiográfica tridimensional para definir o limite lateral teórico de ressecabilidade endoscópica de tumores orbitais primários e aplicar essas condições de limites a casos cirúrgicos. Método: Um modelo orbital tridimensional foi aplicado a quatro pacientes representativos com tumores orbitais primários utilizando o software de imagem de fonte aberta OsiriX. Um plano bidimensional foi propagado entre a narina contralateral e uma linha tangencial ao eixo longo do nervo óptico que reflete a trajetória de uma abordagem transeptal. Qualquer volume de tumor situado medialmente ao nervo óptico e/ou dentro do espaço inferior a esse plano de ressecabilidade foi teoricamente considerado ressecável, independentemente de quão longe ele se estendia até o nervo óptico, pois a retração do nervo seria desnecessária. Os volumes reais do tumor foram então sobrepostos sobre esse plano e correlacionados com os resultados cirúrgicos. Resultados: Entre as quatro lesões analisadas, duas eram totalmente mediais ao nervo óptico, uma se estendia lateralmente ao nervo óptico, mas permaneceu inferior ao plano de ressecabilidade, e uma se estendia lateralmente ao nervo óptico e superior ao plano de ressecabilidade. Conforme previsto pelo modelo tridimensional, uma ressecção completa foi obtida em todas as lesões, exceto uma, que transgrediu o plano de ressecabilidade. Nenhuma nova diplopia ou perda de visão foi observada em qualquer paciente. Conclusão: A reconstrução tridimensional melhora o planejamento pré-operatório para a cirurgia orbital endoscópica. Os tumores que se estendem lateralmente ao nervo óptico podem ainda ser candidatos à ressecção puramente endoscópica, desde que não se estendam além do plano de ressecabilidade aqui descrito.

Solitary fibrous tumor of the lacrimal gland mimicking pleomorphic adenoma / Tumor fibroso solitário de glândula lacrimal simulando adenoma pleomórfico

ABSTRACT Solitary fibrous tumor (SFT) is a rare mesenchymal spindle-cell neoplasm commonly found in the pleura; it is rare in the orbit and extremely rare in the lacrimal gland. We herein report a case of SFT of the lacrimal gland that mimicked a pleo morphic adenoma. We discuss the clinical, radiological, histopathological, and immunohistochemical findings that provided insight and rationale to accurately diagnose this case.

RESUMO Os autores relatam um caso de tumor fibroso solitário (TFS) de glândula lacrimal simulando um adenoma pleomórfico. O TFS é um raro tumor mesenquimal de células fusiformes, comumente encontrado na membrana pleural, raramente en contrado na órbita e extremamente raro na glândula lacrimal. Os autores discutem os achados clínicos, radiológicos, histológicos e imunohistoquímicos que são a chave para o correto diagnóstico desta rara entidade.

Neuronavegación en exéresis de linfangioma orbitario / Neuronavigation in exeresis of orbital lymphangioma

El linfangioma es un tumor benigno raro y predominante en la infancia, debido a su crecimiento puede comprometer al órgano donde se desarrolla, se han propuesto varias opciones de trata-miento, sin embargo, la cirugía continúa siendo la primera opción. La neuronavegación permite realizar exéresis con gran precisión y de utilidad en cirugía ocular, por lo que disminuye el riesgo de secuelas después de una exéresis de linfangioma orbitario.

Lymphangioma is a benign tumor predominantly in childhood, due to growth that can compromise the organ where it grows. Several treatment options, have been proposed however,surgery remains the first choice. Neuronavigation allows successful excision and use in eye surgery,which decreases the risk of sequels following excision of orbital lymphangioma.

Extraconal cystic schwannoma mimicking an orbital dermoid cyst / Schwannoma cístico extraconal simulando um cisto dermóide orbitário

ABSTRACT To our knowledge, we report the first case of an extraconal orbital cystic schwannoma in Brazilian literature. The tumor grew slowly and progressively and was associated with minimal eccentric proptosis and diplopia. The radiologic study (orbital computed tomography) revealed a rounded, encapsulated, and extraconal cystic mass in the superior medial region of the right orbit in the supraorbital and supratrochlear nerve topography. An anterior orbitotomy with full excision of the tumor was performed, and the histopathology examination revealed that the tumor was a schwannoma. After the surgery, the patient experienced total remission of his symptoms.

RESUMO Os autores relatam o primeiro caso de schwannoma orbitário extraconal cístico relatado na literatura brasileira. O tumor apresentou aparecimento lento e progressivo, associado a proptose excêntrica e diplopia leves. O exame de imagem (tomografia computadorizada de órbita) revelou uma lesão nodular, arredondada, capsulada, extracônica, cística, na região súpero-medial de órbita direita, na topografia do nervo supraorbitário e supra-troclear. O paciente foi submetido à orbitotomia por via anterior, com exérese integral da tumoração. O exame histopatológico revelou o diagnóstico de schwannoma. Após a cirurgia, o paciente teve regressão total dos sintomas.

Condrosarcoma del septum nasal: reporte de un caso y revisión de la literatura / Chondrosarcoma of the nasal septum: a case report and literature review

El condrosarcoma del tabique nasal es una neoplasia poco frecuente. Cuando esto ocurre, el diagnóstico precoz es difícil porque los pacientes generalmente presentan síntomas rinosinusales inespecíficos y en forma tardía. Se presenta un caso clínico de una mujer de 37 años de edad que se presentó con una historia de exoftalmo sin síntomas rinosinusales agregados. El estudio imagenológico mostró una masa de aspecto neoplásico en el tabique nasal con extensión al seno maxilar, celdillas etmoidales, cavidad orbitaria y hacia posterior a la fosa pterigopalatina. Se consideró un tumor naso-orbitario por lo que se complementó estudio con nasofibroscopía flexible que mostró una masa nasal de aspecto liso, brillante, rosada que ocupaba piso, tabique y pared lateral de fosa nasal izquierda que no era separable del tabique nasal. Se realizó biopsia que mostró una histopatología sugestiva de condrosarcoma. Se realizó cirugía por abordaje endoscópico y con apoyo neuroquirúrgico por vía transcraneal. La presentación clínica, diagnóstico y tratamiento de este caso, así como una revisión de la literatura son discutidos.

Chondrosarcoma of the nasal septum is a rare malignancy. When this occurs, early diagnosis is difficult since patients usually present non specific symptoms rhinosinusal and late. There is a clinic case of a 37-year-old women who presented with a history of exophthalmos without added symptoms rhinosinusal. The image study showed a neoplasic mass appearance in the nasal septum with extension to the maxillary sinus, ethmoid cells, orbital cavity and post to the pterygopalatine fossa. We have considered a naso-orbital tumor which was complemented nasofibroscopy flexible nasal showed a pink mass of smooth, shiny, occupied floor, septum and lateral wall of left nasal cavity that was not separable from the nasal septum was considered. We have performed a biopsy wich shows a suggestive chondrosarcoma. Surgery was performed by endoscopic approach and via transcranial neurosurgical support. The clinical presentation, diagnosis and treatment of this case and a review of the literature are discussed.

Solitary fibrous tumor of the orbit presenting in pregnancy.

A 32-year-old woman, three months pregnant, reported with the complaint of protrusion of the right eye for six months. She gave history of rapid protrusion of eyeball for the last two months along with the history of double vision for the last one month. Computer tomography (CT) scan revealed a well-defined mass lesion in the intraconal space of the right orbit which was excised through a lateral orbitotomy approach. Histological examination and immunohistochemistry revealed a solitary fibrous tumor, which showed a rapid progression in pregnancy.

Eyelid [and orbital] malignant fibrous histiocytoma

We report a case of an eyelid malignant fibrous histiocytoma in a 6 year old girl, because the eyelid is an unusual location for a fibrous histiocytoma [whereas it is common in the orbit], and the malignant variety of the tumor is the least frequent. The tumor presented as a painless, rapidly growing mass, causing complete ptosis of the right upper eyelid. Computerized tomography [CT-scan] showed a soft tissue mass in the right upper eyelid, in contact with the globe, and extending, superiorly to the globe, to the adjacent medial orbital wall, and posteriorly to the level of origin of the superior and medial recti muscles. The diagnosis of malignant fibrous histiocytoma was confirmed by histopathological examination. The tumor was treated by wide surgical excision