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.

Enfermedad inflamatoria orbitaria idiopática. Presentación de un caso / Idiopathic orbital inflammatory disease. Case presentation

RESUMEN El pseudotumor orbitario es la tercera causa de oftalmoplejía dolorosa; constituye un desafío diagnóstico pues obliga a descartar enfermedades de etiología muy diversa que lo provocan. Se presenta el caso de una paciente femenina, de color de piel blanca, de procedencia urbana, ama de casa, fumadora, que no practica ejercicios ni lleva dieta, con antecedentes de hipertensión arterial y artritis reumatoidea, que acudió al Servicio de Oftalmología por presentar síntomas inflamatorios anexiales: edema palpebral, quemosis conjuntival, vasos conjuntivales dilatados, que se acompañaban de proptosis, oftalmoplejía dolorosa con diplopía y de una masa tumoral palpable a nivel de la porción supero-externa de la órbita. Se ingresó y se le realizaron estudios imagenológicos e histológicos, que solo aportaron signos inflamatorios y una pansinusitis. Se concluyó como un pseudotumor orbitario en su forma aguda de aparición, asociada a la artritis reumatoidea, cuyo diagnóstico se realizó por exclusión sobre la base de los resultados negativos de los estudios imagenológicos y de la biopsia. Llevó tratamiento con antibióticos parenterales y altas dosis de esteroides orales con regresión del cuadro y mejoría clínica. Se decidió la presentación del caso porque en la provincia no existen reportes sobre esta entidad, y por el énfasis en cómo llegar al diagnóstico y su correcto manejo, evitando con ello secuelas en el órgano visual.

ABSTRACT Orbital pseudotumor is the third cause of painful ophthalmoplegia; it constitutes a diagnostic challenge as it forces us to rule out diseases of very diverse etiology that cause it. A female patient, of white skin color, of urban origin, housewife, smoker, who does not practice exercises or is not on a diet, with a history of high blood pressure and rheumatoid arthritis, who attended the Ophthalmology service for presenting Adnexal inflammatory symptoms: eyelid edema, conjunctival chemosis, dilated conjunctival vessels, which were accompanied by proptosis, painful ophthalmoplegia with diplopia and a palpable tumor mass at the level of the superior-external portion of the orbit was presented. She was admitted and imaging and histological studies were performed, which only revealed inflammatory signs and pansinusitis. It was concluded as an orbital pseudotumor in its acute onset form, associated with rheumatoid arthritis, the diagnosis of which was made by exclusion on the basis of the negative results of imaging studies and biopsy. She was treated with parenteral antibiotics and high doses of oral steroids with regression of the symptoms and clinical improvement. The presentation of the case was decided because in the province there are no reports on this entity, and because of the emphasis on how to reach the diagnosis and its correct management, thereby avoiding effects in the visual organ.

Uso terapéutico de la hialuronidasa y la triamcinolona en el pseudotumor orbitario / Therapeutic use of hyaluronidase and triamcinolone in orbital pseudotumor

RESUMEN El origen del pseudotumor orbitario no es del todo conocido. Se admite su naturaleza inflamatoria granulomatosa e inespecífica en diferentes localizaciones. El pseudotumor orbitario se define como una respuesta inflamatoria celular pleomórfica, que está usualmente confinado a estructuras de la órbita y tiene una evolución limitada. En este trabajo se presenta una paciente femenina de 16 años, con diagnóstico de pseudotumor orbitario corroborado por biopsia y tomografía axial computarizada, refractaria al tratamiento con esteroides sistémicos, por lo que se decide iniciar con la aplicación de hialuronidasa y triamcinolona en el espacio peribulbar. Los casos agudos casi siempre responden rápidamente al tratamiento con cortocoesteroides, como prednisona, pero debemos tener en cuenta que existen pacientes que son refractarios al tratamiento, por lo que es necesario buscar procedimientos alternativos. Una opción es el uso de hialuronidasa para destruir las uniones extracelulares, y difundir un esteroide de manera local, como la triamcinolona, más efectiva dentro del tejido inflamatorio para provocar un efecto localizado de este. A los tres meses del tratamiento hubo una regresión total del cuadro en esta paciente(AU)

ABSTRACT The exact etiology of orbital pseudotumor is unknown, but its granulomatous unspecific inflammatory nature at various locations has been recognized. Orbital pseudotumor is defined as a cellular pleomorphic inflammatory response of limited evolution often confined to orbital structures. A case is presented of a female 16-year-old patient diagnosed with orbital pseudotumor confirmed by biopsy and computerized axial tomography, refractory to treatment with systemic steroids, due to which it is decided to start treatment with hyaluronidase and triamcinolone in the peribulbar space. Acute cases often respond fast to treatment with corticosteroids such as prednisone. It should be borne in mind that there are patients who are refractory to treatment for whom alternative treatments should be sought. An option is the use of hyaluronidase to destroy extracellular junctions and locally spread a steroid such as triamcinolone, most effectively within the inflammatory tissue to ensure its localized effect. Total regression of the patient's status was observed at three months of treatment(AU)

Neurofibromatose tipo 1 com acometimento do nervo infraorbital: relato de caso / Neurofibromatosis type 1 with infraorbital nerve involvement: a case report

A neurofibromatose tipo 1 é uma doença autossômica dominante rara, com manifestações clínicas diversas. Sua apresentação mais marcante é a presença de neurofibromas (tumores da bainha neural) cutâneos ou internos, que também podem ocorrer de forma esporádica, associados a outras manifestações sistêmicas, como manchas café com leite e lesões oculares. Por serem tumores da bainha de mielina, os neurofibromas podem acometer diversos nervos periféricos, incluindo nervos da face. Apresentamos o caso de um paciente de 1 ano, portador de neurofibromatose tipo 1, com neurofibroma em nervo infraorbital direito, com o acesso proposto para tratamento cirúrgico que fornecesse ampla visualização e acesso a lesão, sem comprometimento estético importante, permitindo preservação de partes moles e adequado crescimento facial.

Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disease with multiple clinical manifestations. Its most significant presentation is cutaneous or subcutaneous neurofibromas (myelin sheath tumors), which may be associated with other systemic manifestations such as caféau- lait spots and eye involvement. Neurofibromas can affect several peripheral nerves, including the facial nerves. This report presents a case of a 1-year-old patient with NF1 with right infraorbital nerve neurofibroma in which the proposed access for surgical treatment allowed adequate visualization of the tumor with good aesthetic results, preservation of the soft tissues, and normal facial growth.

Pseudotumor orbit-bilateral v/s unilateral: a case study of two orbital pseudotumor cases

Orbital pseudotumor is a non-granulomatous inflammation of orbital soft tissue for unknown etiology. In the first case, a 35-year-old female presented with bilateral painless upper eyelid swelling since 3years.No proptosis, no extra ocular movements restriction of both eyes was noted. Best corrected visual acuity both eye: 6/6, N6. USG B-Scan and CECT revealed bilateral lacrimal gland enlargement with bulky muscles and tendons. USG guided FNAC confirmed pseudotumor which showed prompt response to steroids but recurred after 1month of stopping of steroid therapy. In the second Case, a 15year old female presented with unilateral proptosis left eye for 3 months with painful extraocular movements and restricted in all gazes. Best corrected visual acuity in L/E - 6/12, N6, R/E-6/9, N6. USG B-scan and CECT revealed soft tissue lesion encasing Optic nerve. CEMR revealed bulky lacrimal gland, muscles and lesion extending up to orbital apex. Prompt response to steroid with no recurrence till date.

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)

Pseudotumor orbital bilateral recorrente / Recorrent bilateral orbits pseudo-tumor

Resumo O pseudotumor orbitário é uma doença inflamatória idiopática benigna. Os autores apresentam um caso manifestado em adolescente de 12 anos, diagnosticado por meio do exame clínico, laboratorial e radiológico. Houve boa resposta ao tratamento proposto com corticosteroides. O relato é seguido de breve retomada literária acerca do tema.

Abstract The orbital pseudotumor is a benign idiopathic inflammatory disease. The authors present a case manifested in 12 years old boy, diagnosed by clinical, laboratory and radiological examination. There was a good response to treatment with corticosteroids proposed. The report is followed by brief literary resume on the subject.

Infarto cerebral limítrofe secundario a enfermedad inflamatoria orbitaria idiopática / Watershed cerebral infarction secondary to idiopathic orbital inflammatory disease

Los infartos limítrofes son aquellos ubicados en regiones entre dos territorios vasculares contiguos y la enfermedad inflamatoria orbitaria idiopática es un proceso inflamatorio orbitario inespecífico. Presentamos el caso de un varón de 60 años con exoftalmos, ptosis palpebral, disminución de agudeza visual derecha y afección de los nervios oculomotores derechos. Los estudios de imágenes mostraron una masa retro ocular derecha con invasión de los senos cavernosos y esfenoidal. Durante la hospitalización, el paciente sufrió un infarto cerebral limítrofe por compresión de la arteria carótida interna derecha en su segmento intracavernoso. La exéresis y la anatomía patológica de la lesión orbitaria fueron compatibles con la enfermedad inflamatoria orbitaria idiopática. Nuestro caso muestra a la enfermedad inflamatoria orbitaria idiopática como una causa inhabitual de infarto cerebral limítrofe.

Watershed cerebral infarctions are those located in regions between two adjacent vascular territories and Idiopathic orbital inflammatory disease is a nonspecific inflammatory process of the orbit. We report the case of a 60-year-old man who presented suddenly in the right eye: exophthalmos, ptosis, reduced visual acuity and paresis of the extraocular muscles innervated by the III, IV and VI cranial nerves. Imaging studies showed a right retro-ocular mass with invasion of the ipsilateral cavernous and sphenoid sinus. During hospitalization, suddenly he presented a watershed infarction with narrowing of the right internal carotid artery in its intracavernous portion. Surgical excision of the lesion was performed and pathology showed a characteristic infiltration of idiopathic orbital inflammatory disease. Our case shows idiopathic orbital inflammatory disease as an uncommon cause of watershed cerebral infarction.

Pseudotumor esclerosante de órbita / Sclerosing orbital pseudotumor

RESUMO O pseudotumor esclerosante de órbita é um subtipo raro de pseudotumor inflamatório idiopático de órbita. É mais comum em adultos e apresenta diagnóstico de exclusão. A primeira linha de tratamento são os esteroides. O subtipo esclerosante apresenta resposta moderada aos esteroides devido à predominância de fibrose e colágeno na histologia. Relatamos o caso de um paciente com diagnóstico histológico de pseudotumor esclerosante de órbita que teve boa resposta ao tratamento com corticoide associado à azatioprina.

ABSTRACT Sclerosing orbital pseudotumor is a rare subtype of idiopathic orbital inflammatory pseudotumor. It's more common in adults and presents diagnosis of exclusion. Steroids represent the first option of treatment. The sclerosing orbital pseudotumor subtype shows moderate response to steroids due to the predominance of fibrosis and collagen in its histology. We report on a case of a patient with histologic diagnosis of sclerosing orbital pseudotumor successfully treated with corticosteroid associated with azathioprine.

Inhibitory effects of dexamethasone on proliferation of fibroblasts derived by idiopathic orbital inflammatory pseudotumor / 中华实验眼科杂志

Background Idiopathic orbital inflammatory pseudotumor (IOIP) is a common orbital disease, but its etiology is still unclear,so the effect of glucocorticoid treatment is unsatisfied.Objective This study was to investigate the effects of dexamethasone on orbital fibroblasts from IOIP patients and explore the action machanism.Methods Six pieces of IOIP tissues from 6 IOIP patients and 3 pieces of normal orbital connective tissues from lacrimal gland prolapse patients were obtained during the surgery in Beijing Tongren Hospital from November 2011 to January 2012.The orbital fibroblasts were cultured using explant culture method.The morphology of the cells were observed under the optical microscope,and biomarks of the cells were detected by immunochemistry.The growth and proliferation of the cells were assayed using WST-8.The expression of ICAM-1 in the cells in both the control group and the IOIP group was detected by immunochemistry.The fibroblasts were incubated in 96-well plates, and different concentrations of dexamethasone (0,1 × 10-3 , 1 × 10-4 , 1 × 10-5 and 1 × 10-6 mol/L) were respectively added into the medium for 24,48 and 72 hours,and then the proliferation of the cells was detected by WST-8 assay.The contents of ICAM-1 in different concentrations of dexamethasone groups were assayed by ELISA.Results The characteristics of the cells were similar between the control group and the IOIP group with the spindle shape and long protructions.The cells showed the positive response for vimentin and absent response for desmin, S-100, cytokeratin (CK).Compared with the control group,the growth speed of fibroblasts was fast in the IOIP group.The proliferative values of the cells (absorbancy) were gradually reduced with the increase of dexamethasone concentrations (F ion =36.27,P=0.00) and the lapse of acting time (Ftime =3.69 ,P=0.00).In cultured cells without dexamethasone for 24,48 and 72 hours,the mean expression levels of ICAM-1 were 0.298±0.008,0.312±0.003 and 0.319±0.011, showing a gradually increasing trend.However,the expression of ICAM-1 was gradually reduced with the increases of concentrations and the lapse of acting time of dexamethasone (Fconcentration =75.17,P=0.00;Ftime =3.11,P=0.00).Conclusions Occurrence and development of IOIP is probably associated with the over-expression of ICAM-1 in orbital fibroblasts.Dexamethasone plays anti-inflammation and treating effects on IOIP by down-regulating the expression of ICAM-1 and inhibiting the proliferation of orbital fibroblasts.

Somatostatin receptor scintigraphy of orbital inflammatory pseudotumor associated with systemic vasculitis / 中华核医学与分子影像杂志

Objective To explore the value of somatostatin receptor scintigraphy(SRS) in evaluating the immune activity of orbital inflammatory pseudotumor associated with systemic vasculitis.Methods Twenty-five patients with orbital inflammatory pseudotumor associated with systemic vasculitis (10 males,15 females,average age:(51.2± 14.2) years) underwent SRS.The uptake ratio (UR) of orbital inflammatory pseudotumor was obtained.(1) Patients were divided into group A (with immune activity) and group B (without immune activity) according to Birmingham vasculitis activity score (BVAS).The difference of UR between the 2 groups was compared by two-sample t test.The difference of UR before and after treatment in 12 patients was also compared.(2) Based on the results by BVAS,ROC curve was used to obtain the cut-off value of UR,as well as the diagnostic efficiency and Youden index.The consistency between SRS and BVAS was calculated.(3)Patients were divided into two groups according to the cut-off value of UR and the prognosis difference between them was compared by Fisher exact test.(4)The expression of SSTR2 and SSTR5 was observed by immunohistochemistry.Results (1) UR in group A was significantly higher than that in group B (2.09±0.44 vs 1.32±0.46,t =5.94,P＜0.01).After glucocorticoids treatment,the UR in group A reduced significantly (t=4.07,P＜0.01),but not in group B (t=1.76,P＞0.05).(2)ROC curve analysis identified UR cut-off value as 1.66,with the sensitivity of 87.5％,specificity of 95.7％,positive predictive value of 95.2％,negative predictive value of 88.0％,accuracy of 91.3％ and Youden index of 83.2％.The consistency between SRS and BVAS was strong (Kappa =0.840).(3) The prognosis was significantly different between patients with UR≥ 1.66 and UR＜1.66 (P＜0.05).(4) The immunohistochemical results revealed high expression of SSTR2 and SSTR5 in inflammatory cells in patients with immune activity.Conclusion SRS has potential value in evaluating the immune activity of orbital inflammatory pseudotumor associated with systemic vasculitis.

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

No abstract available.

Clinical Characteristics of Pediatric Orbital Pseudotumors

PURPOSE: To describe the clinical manifestations, radiologic findings, and treatment outcomes of pediatric pseudotumors. METHODS: A retrospective chart review of patients diagnosed with pediatric pseudotumor (age under 20 years old) from August 2008 to February 2012 was performed. RESULTS: Thirteen patients (16 eyes) were included in this study. The mean age of the subjects was 14.2 years (5-20 years). Swollen eyelid (56.3%), ptosis (43.8%), conjunctival injection (18.8%), localized mass (18.8%), limitation of ocular movement (12.5%), proptosis (6.3%), and decreased visual acuity (6.3%) were found initially. Dacryoadenitis (62.5%), myositis (18.8%), anterior orbital inflammation (18.8%), and diffuse type (6.3%) were observed on orbital computed tomography (CT). Among the 13 patients (16 eyes), 8 patients (10 eyes) were administered oral systemic corticosteroids, 2 patients (2 eyes) received IV systemic corticosteroid, 1 patient (1 eye) received systemic corticosteroids combined with NSAID, and 2 patients (3 eyes) were prescribed NSAIDs only. Symptoms improved 4.1 days after initiation of treatment. CT scans revealed that one patient experienced diffuse-type disease recurrence twice. CONCLUSIONS: Orbital pseudotumors, which are rare in younger people, are likely to respond very well to corticosteroid treatment, and the recurrence of orbital inflammation is rare.

Atypical Presentation of Orbital Pseudotumor with Visual Loss as an Initial Manifestation

BACKGROUND: An orbital pseudotumor typically presents with periorbital pain, cranial nerve palsies and proptosis. Although visual deterioration is not unexpected in this pathology, its presentation solely with visual loss is unusual. CASE REPORT: In this short report, we summarize a case of orbital pseudotumor which presented solely with a decrease in visual acuity, and discuss the clinical and radiological findings. CONCLUSIONS: This atypical presentation likely resulted from the orbital pseudotumor originating in the optic foramen, leaving the neurovascular structures of the superior orbital fissure untouched initially. In the early clinical period, an orbital pseudotumor may manifest itself solely by visual loss. It should therefore be included in the differential diagnosis of visual pathologies-even in the absence of orbital pain and symptoms related to ocular movements.

Spontaneous Intraorbital Hemorrhage : A Case Report

Intraorbital hemorrhage is a rare clinical condition caused by orbital trauma, surgery around the orbit, intraorbital vascular abnormalities, and neoplasm. It was reported to occur spontaneously without any known causes and in association with orbital pseudotumor in a very few cases. A 59-year-old, female patient admitted with sudden onset of severe exophthalmos and pain on the left eye. Orbital CT and MR imaging suggested hemorrhage in the upper part of retrobulbar area of the left orbit. Cerebral angiography was taken to rule out any possible vascular abnormalities. On the left carotid cerebral angiography, the run-off of the distal ophthalmic artery was not seen and the engorgement of the supraophthalmic artery was noted. Systemic administration of corticosteroid did not improve the clinical status and craniectomy was done and retrobulbar hematoma was removed, and the clinical symptoms and signs were improved. Authors report a case of spontaneous intraorbital hemorrhage with the clinical features similar to those of orbital pseudotumor, requiring surgical decompression.

Inflammatory pseudotumor of an orbito-maxillary mass masquerading as a malignancy

Objective: To present a case of a benign orbito-maxillary tumor behaving as an invasive, expansive malignancy. Design: Case report Setting: A tertiary care hospital in Metro Manila Patient: A 4 1/2-year-old boy with a seven month history of right orbito-maxillary mass, proptosis and epiphora. Result: A series of biopsies were done before a definite diagnosis was made due to inconsistencies in the histopathologic findings, clinical picture, and the radiologic presentation of the case. After diagnosis, appropriate intervention resulted in a dramatic decrease in the size of the mass. At present, the patient is disease-free and asymptomatic. Conclusion: Histopathologic diagnosis of Inflammatory Pseudotumor is difficult and differentiating it from malignant tumors is often a concern for otolaryngologists and pathologists. In spite of an initial malignant biopsy result, the combination of clinical signs and symptoms and radiologic findings of an infiltrative mass lesion, should not discount the possibility of a benign entity such as Inflammatory Pseudotumor for which treatment is conservative. (Author)

A Case of Nonspecific Orbital Inflammation in Anophthalmia

PURPOSE: We report a case of orbital pseudotumor developed in an anophthalmic socket, presenting no typical symptoms or signs. METHODS: A 67-year-old woman was referred for treatment of necrotizing scleritis of her left eye and painful orbital pseudotumor of her right eye. The right eye had been removed 10 years previously. There was an irregular mass in superomedial portion of anterior orbit. Orbital MRI showed poorly defined T1 iso and low T2 signal intensity of a lesion in the medial anterior portion of the right orbit. Because systemic steroid administration was limited, 2 mg of betamethasone was injected locally, after which the size of the lesion was decreased and the orbital pain improved. CONCLUSIONS: The diagnosis of an orbital pseudotumor developed in an anophthalmic socket may be difficult because of the lack of typical eyeball-related signs or symptoms such as proptosis, red eye, or decreased vision. But, the easy access to the lesion can provide early and effective treatment through direct injection of glucocorticoid.

The Clincal Experiences of the Orbital Pseudotumor

Orbital pseudotumor, also known as idiopathic orbital inflammatory syndrome(IOIS), may have protean clinical manifestations. IOIS should be determined with a diagnosis of exclusion, with evaluation directed toward eliminating other causes of orbital disease. Orbital MRI and CT are the important diagnostic tests, but serologic studies are necessary to exclude a systemic causes. Biopsy is usually not performed currently, as the risk of producing damage to vital structures within the orbital outweighs the benefits. Patients with multiple recurrences, or those unresponsive to therapy, should be treated of biopsy sample extraction. Corticosteroids are the mainstay of therapy and administered for several months to ensure remission. Radiotherapy may be used in patients who fail to respond to steroids or who have a rapidly progressive course. We analysed the data of two patients. There were no specific complications related to this treatment. We discussed the radiologic findings, treatment procedures, and other orbital diseases.