A retrospective analysis of the management and surgical treatment of orbital lesions: Outcomes and rationale.

The orbital cavity is a subject of interest for various specialists, and achieving optimal outcomes requires comprehensive, multidisciplinary management. This study aims to report 10 years of experience in the preoperative, surgical, and postoperative care of patients with orbital lesions, examining their clinical, radiological, and anatomopathological features and outcomes. A retrospective review of 125 patients who underwent surgical treatment for intraorbital masses between January 2012 and December 2021 was performed. Outcome measures included postoperative diplopia, exophthalmos, decimal visual acuity, eyeball position, ocular motility, operative time, complications, and aesthetic results. A total of 107 patients were included. All cases were discussed with a neuroradiologist to determine the best therapeutic approach based on preoperative imaging. Preoperative diplopia was linked to extraconal (p = 0.03) and anterior (p = 0.001) lesions, and exophthalmos and visual acuity deterioration were associated with intraconal (p = 0.02; p = 0.03) and retrobulbar (p = 0.001; p = 0.02) lesions. Complications (11.2%) included diplopia, worsened visual acuity, postoperative blepharoptosis, and postoperative ectropion. Of the patients, 80.4% reported an "excellent" aesthetic outcome. This study underscores the importance of a multidisciplinary approach based on a thorough analysis of preoperative imaging. Periorbital approaches tailored to the lesion's three-dimensional location enables safe access to most intraorbital lesions, resulting in minimal complications and good aesthetic results.

Lateral micro-orbitotomy as the technique of choice for orbital pseudotumor in IgG4-RD. Case report and review of the literature.

Immunoglobulin G4-related disease (IgG4-RD) is characterized by a systemic fibroinflammatory infiltrate that often involves the orbit in addition to other tissues. Thus it has to be considered in the differential diagnosis of orbital tumors. We report the clinical case of a 64-year-old woman who presented with right mydriasis, progressive proptosis and paralysis of the third cranial nerve of 1 year of evolution. Cranial MRI identified an intraconal lesion of the right orbit, located between the external and inferior rectus muscles and the optic nerve, and she was scheduled for surgery by transcranial approach with lateral micro-orbitomy. A satisfactory macroscopic excision was achieved with no remarkable complications and a definitive deferred histological result of pseudotumor by IgG4-RD. Follow-up for 24 months showed no tumor recurrence, and the patient clinically improved from ophthalmoplegia. This case highlights the efficacy of lateral orbitotomy in the etiologic diagnosis and successful therapeutic outcome of complex orbital lesions associated with IgG4-RD pseudotumor.

Central serous chorioretinopathy associated with nonspecific orbital inflammation: a case report.

We herein present a rare case of acute central serous chorioretinopathy (CSC) associated with nonspecific orbital inflammation (NSOI). A 38-year-old woman presented with a 3-day history of ocular pain, reduced vision, periorbital swelling, proptosis, conjunctival chemosis, and restricted eye movements. Optical coherence tomography of the affected eye confirmed signs of CSC. Additionally, a computed tomography scan revealed enlargement of intraconal soft tissues and the lacrimal gland. Ocular ultrasonography detected posterior sclera thickening, indicating posterior scleritis. Following the diagnosis of NSOI, the patient received treatment with systemic corticosteroids, resulting in gradual regression of both the orbital inflammation and CSC. This is the first reported case of localized posterior pole CSC documented in a patient with NSOI. Vigilant monitoring for any ocular disorders is important in patients with orbital inflammation.

Idiopathic Orbital Pseudotumor: A Rare Case Report.

Orbital pseudotumor is a rare inflammatory condition affecting the orbit of the eye. It has diverse clinical manifestations. Although its exact etiology remains unknown, it is believed to involve an immune-mediated response. A 42-year-old male presented to the ophthalmology clinic with progressively worsening symptoms in his left eye, including pain, swelling, and blurry vision. He had no history of trauma or recent illness. Initial discomfort had escalated over three weeks. The patient had controlled hypertension but no known allergies. Examination showed eyelid edema, erythema, and mild anterior chamber cell, and flare in the left eye. Magnetic resonance imaging revealed orbital soft tissue enhancement, extraocular muscle thickening, and optic nerve involvement. Laboratory results showed elevated inflammatory markers. A diagnosis of orbital pseudotumor was made. The patient was treated with oral corticosteroids, resulting in symptom improvement and regression of inflammatory changes on follow-up. Orbital pseudotumor is a complex condition with diverse clinical manifestations. Its diagnosis requires a comprehensive approach involving clinical evaluation, imaging studies, and laboratory investigations.

Limited granulomatosis with polyangiitis in children with idiopathic orbital inflammation: a case series and literature review.

Idiopathic orbital inflammation (IOI) is a diagnosis of exclusion, but the exclusion of other inflammatory diseases of the orbit is broad and relies on clinician experience, response to corticosteroid, or biopsy. This study aimed to investigate the presence of granulomatosis with polyangiitis (GPA) in patients initially diagnosed as IOI and describe its clinicopathological features, ANCA status, treatment, and outcome. We performed a retrospective case series study of children diagnosed with limited GPA (L-GPA) in patients with IOI. A systematic review of the literature was performed in children with GPA and orbital mass. Eleven of 13 (85%) patients with IOI had L-GPA. Two additional patients with orbital mass and L-GPA were included in this analysis. The median age was 10 years, and 75% were female. Twelve cases were ANCA positive and 77% were MPO-pANCA positive. Most patients had a poor response to treatment and had a high relapse rate. Based on literature review, 28 cases were found. Most (78.6%) were female with a median age of 9 years. Three patients were misdiagnosed as IOI. Patients with L-GPA more frequently had MPO-pANCA positivity (35%) than children with systemic GPA (18%) and were less often PR3-cANCA positive than patients with systemic GPA (18% vs. 46%). L-GPA accounts for a high prevalence of children diagnosed as IOI. The high prevalence of MPO-pANCA observed in our study may be related to L-GPA rather than with the orbital mass. Long-term follow-up, orbital biopsy, and serial ANCA testing are necessary to exclude GPA in patients with IOI.

Orbital pseudotumor in pediatrics: A single tertiary center experience.

Orbital pseudotumor is a rare condition characterized by an idiopathic inflammatory process of the orbit with a polymorphous lymphoid infiltrate. It is misdiagnosed as orbital cellulitis or orbital mass with conjunctivitis in children.

A single-institution review of lacrimal gland biopsies between 1962 and 2017.

PURPOSE: To examine the clinical and histopathologic characteristics of lacrimal gland biopsies at a tertiary academic center. METHODS: A retrospective chart review of patients undergoing lacrimal gland biopsy or excision between 1962 and 2017 was performed via the ocular pathology specimen log. All cases were reviewed for demographics, clinical presentation, and histopathologic diagnoses. RESULTS: Four hundred and two eyes in 356 patients were included in the analysis. Median age was 49 (range 5-91) with a female predominance (255, 72%, p < .001). Most had unilateral involvement (308, 86.5%) and visual acuity of 20/50 or better (332 eyes, 83%). Limitation in extraocular motility was present in 71 eyes (18%), relative afferent pupillary defect in 10 eyes (2.5%), and intraocular pressure 20 mmHg or above in 80 eyes (20%). The pre-operative radiology report commented on the enlargement of the lacrimal gland in 236 eyes (58.7%), and lack thereof in 73 eyes (18.2%). The most common histopathologic diagnoses were nonspecific inflammation or orbital pseudotumor (170, 42%), lymphoma (65, 16%), pleomorphic adenoma (22, 5.5%), adenoid cystic carcinoma (19, 4.7%), granulomatous inflammation (19, 4.7%), and normal lacrimal gland (16, 4%). Three hundred and seven cases were benign (76%) and 95 malignant (24%). The biopsy specimen was diagnostic in 343 (85%), and non-diagnostic in 59 (15%). CONCLUSIONS: This is a comprehensive review of one of the largest ocular pathology databases of lacrimal gland lesions. This study confirms the wide range of inflammatory and neoplastic conditions affecting the lacrimal gland and highlights the nuances of histopathologic diagnoses and diagnostic yield of biopsies in clinical practice.

Orbital Inflammatory Pseudotumor following mRNA COVID-19 Vaccination.

The authors present a case of orbital pseudotumor after mRNA COVID-19 vaccination. A 40-year-old otherwise healthy woman was referred to our oculoplastics unit because of left blepharoptosis of 2 months duration starting 1 week after she received her first Pfizer-BioNTech mRNA vaccination. On presentation, her best-corrected visual acuity was 20/20 in each eye. The external examination revealed left blepharoptosis and mild upper eyelid swelling. Orbital magnetic resonance imaging revealed left lacrimal gland enlargement with homogeneous contrast enhancement and diffuse mild enlargement of the left lateral and superior rectus muscles. The results of the extended workup for autoimmune and infectious etiologies and the systemic examination findings were normal. Systemic corticosteroids were started for the orbital pseudotumor. The presented case of orbital pseudotumor development after the mRNA vaccine may be considered to be an immunological process targeting the orbital tissue following immunization, although the cause-effect relationship remains uncertain.

A deep learning model combining multimodal radiomics, clinical and imaging features for differentiating ocular adnexal lymphoma from idiopathic orbital inflammation.

OBJECTIVES: To evaluate the value of deep learning (DL) combining multimodal radiomics and clinical and imaging features for differentiating ocular adnexal lymphoma (OAL) from idiopathic orbital inflammation (IOI). METHODS: Eighty-nine patients with histopathologically confirmed OAL (n = 39) and IOI (n = 50) were divided into training and validation groups. Convolutional neural networks and multimodal fusion layers were used to extract multimodal radiomics features from the T1-weighted image (T1WI), T2-weighted image, and contrast-enhanced T1WI. These multimodal radiomics features were then combined with clinical and imaging features and used together to differentiate between OAL and IOI. The area under the curve (AUC) was used to evaluate DL models with different features under five-fold cross-validation. The Student t-test, chi-squared, or Fisher exact test was used for comparison of different groups. RESULTS: In the validation group, the diagnostic AUC of the DL model using combined features was 0.953 (95% CI, 0.895-1.000), higher than that of the DL model using multimodal radiomics features (0.843, 95% CI, 0.786-0.898, p < 0.01) or clinical and imaging features only (0.882, 95% CI, 0.782-0.982, p = 0.13). The DL model built on multimodal radiomics features outperformed those built on most bimodalities and unimodalities (p < 0.05). In addition, the DL-based analysis with the orbital cone area (covering both the orbital mass and surrounding tissues) was superior to that with the region of interest (ROI) covering only the mass area, although the difference was not significant (p = 0.33). CONCLUSIONS: DL-based analysis that combines multimodal radiomics features with clinical and imaging features may help to differentiate between OAL and IOI. KEY POINTS: • It is difficult to differentiate OAL from IOI due to the overlap in clinical and imaging manifestations. • Radiomics has shown potential for noninvasive diagnosis of different orbital lymphoproliferative disorders. • DL-based analysis combining radiomics and imaging and clinical features may help the differentiation between OAL and IOI.

Canine idiopathic orbital inflammation: A case series of four affected dogs (five orbits).

PURPOSE: In humans, idiopathic orbital inflammation (IOI) is a diagnosis attributed to benign, inflammatory orbital conditions without identifiable local or systemic cause. We describe the clinical signs, imaging and histopathological findings, management and outcome of four dogs diagnosed with IOI. METHODS: Multicentric retrospective study. RESULTS: A total of four dogs (five orbits) of three different breeds (three cases were English Springer Spaniels [ESS] or ESS-cross) and ages ranging from 3 to 12 years were included. Initial presenting signs were unilateral and included exophthalmos, enophthalmos, globe deviation, thickening and protrusion of the third eyelid and conjunctival hyperemia. Computed tomography and magnetic resonance imaging identified heterogeneous space-occupying, contrast-enhancing orbital lesions in all cases. Sparing of the retrobulbar space was detected in four of five orbits. Histopathology revealed mixed inflammatory infiltrates of lymphocytes, plasma cells, and histiocytes. Immunohistochemistry was performed in two cases highlighting the presence of histiocytes and lymphocytes, predominantly T cells. Resolution of clinical signs was achieved in two cases managed with oral immunosuppressant medication (corticosteroids alone or combined with cyclosporine or azathioprine), one went into spontaneous remission, one resolved with topical corticosteroids, and one underwent exenteration. Recurrence occurred in two cases within 15 months of initial diagnosis and required further immunosuppressant medication. One case developed signs in the contralateral orbit within 8 months of presentation. CONCLUSIONS: IOI is an uncommon condition in dogs. Its diagnosis relies on the combination of advanced imaging and histology. As in humans, it appears that spontaneous remission and recurrence may occur requiring long-term immunosuppressant medication.

Features of ophthalmic, magnetic resonance imaging, and histopathology of a feline case of idiopathic sclerosing orbital pseudotumor.

In the present report, we describe a case of sclerosing orbital pseudotumor in an 11-year-old castrated male American Shorthair cat. Ophthalmic exam showed lagophthalmos, retracted right upper eyelid, and resistant to retropulsion in his right eye. Under magnetic resonance imaging (MRI) scans, increased volume of the extraocular muscles (EOMs) of the right eye was prominent. Immunosuppressive dosage of prednisolone partially ameliorated the clinical signs, but some clinical signs were still gradually progressive or persistent. In the second MRI scan, decreased diameter of the thickened right extraocular muscles was found. After the third MRI scan, enucleation of the right eye was performed due to substantial adverse effects of systemic steroid therapy. Histopathological examination revealed no evidence of neoplastic transformation nor infection. Feline restrictive orbital myofibroblastic sarcoma (FROMS) was therefore excluded, suggesting unknown causes of extensive fibrotic changes in the right orbit of the affected cat.

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.

F-18 Fluorodeoxyglucose PET/CT as a Diagnostic Tool in Orbital Inflammatory Disorders.

PURPOSE: To assess the usefulness of FDG-PET/CT as a potential diagnostic tool for detecting underlying systemic diseases (SD) in patients with orbital inflammatory disorders (OID). METHODS: All consecutive patients managed for new-onset OID between 2011 and 2018 in a tertiary referral center, who underwent FDG-PET/CT as part of the etiological diagnostic workup were evaluated. To quantify the incremental value of FDG-PET/CT over standard diagnostic workup, the Net Reclassification Index (NRI) and Integrated Discrimination Index (IDI) were used. RESULTS: Among the 22 patients enrolled, 11 (50%) had a positive FDG-PET/CT. After clinicobiological evaluation, FDG-PET/CT correctly reclassified 4(29%) of 14 patients with SD (p = .04) and 1(13%) of 8 with idiopathic orbital inflammation syndrome (p = .32). NRI and IDI were 0.41 ± 0.17 (p = .03) and 0.38 ± 0.08 (p < .001), respectively. FDG-PET/CT successfully detected asymptomatic lesions in all (n = 4) patients with lymphoma. CONCLUSION: FDG-PET/CT enabled accurate reclassification of more than one-quarter of patients with SD, especially extraorbital lymphomas.

The Role of Radiotherapy in Orbital Pseudotumor: A Systematic Review of Literature.

PURPOSE: Orbital pseudotumor (or pseudotumor orbitae) is a benign entity, also known as idiopathic orbital inflammatory syndrome (IOIS), which encompasses a wide range of non-tumoral, space occupying lesions of the orbit. In selected cases of refractory disease or presence of side effects or even comorbidities that limit systemic therapies, radiotherapy could play a role in the management because it has been demonstrated that irradiation is effective in suppressing the inflammatory process. METHODS: A systematic review of the literature about the main scientific databases was launched and the time interval included all published articles present in the databases from their inception until September 2020. RESULTS: We were able to identify 19 studies eligible for inclusion in this review from 1978 to 2018. Overall the data of 241 patients were collected and are presented in this systematic review. The response rate varied between 74% and 100% with a median recurrence rate of 10%. The median total dose was 20 Gy whereas the mean total dose was 21 Gy with a range from 4 to 36 Gy. Regarding the fractionation, 2 Gy/fraction daily was the most widely used. CONCLUSIONS: Radiotherapy seemed to achieve good response rates however, in most of the studies, inclusion criteria and outcome parameters are not uniform and therefore the results are difficult to compare. Often important parameters such as chronic pain and permanent functional deficits are not assessed in the outcome. Therefore, prospective studies, with good cohort characteristics and a clear definition of the outcome, are required.

Long-term follow up of systemic rituximab therapy as first-line and salvage therapy for idiopathic orbital inflammation and review of the literature.

PURPOSE: To report long-term outcomes of systemic rituximab therapy for idiopathic orbital inflammation (IOI) as both primary and salvage therapy and to review the English literature. METHODS: A retrospective review of four consecutive biopsy-proven IOI cases managed with systemic rituximab including demographics, management, and outcomes, and review of English literature, were performed. Primary outcome measures included resolution of symptoms, recurrence, and length of follow up. RESULTS: Of four cases, systemic rituximab was the first-line therapy in two cases and salvage therapy in two cases. The mean age of the patients was 62 years (range, 50-68 years). The orbit was involved in three cases and extraocular muscle in one case. Systemic rituximab (1 g weekly for 4 weeks) was given for one session in three patients and for 12 sessions in 1 patient. All four patients responded with the resolution of all symptoms without recurrence after at least 5 years of follow up. Review of the literature showed systemic rituximab had provided clinical improvement at shorter follow up in 14 of 15 cases when used as a salvage therapy. CONCLUSIONS: Systemic rituximab therapy seems to be an effective therapy for IOI as salvage or first-line therapy with long-term clinical durability.

Disease modifying drugs in idiopathic sclerosing orbital inflammatory syndrome.

PURPOSE: Idiopathic sclerosing orbital inflammatory syndrome (ISOIS) is a rare, progressive and hard to control disease. There is a deep gap of evidence regarding application of disease-modifying drugs (DMD) regimen as a potentially effective treatment for orbital inflammatory diseases. We aimed to report the results of using DMDs and discuss the concept of applying this modality of treatment in patients with ISOIS. METHODS: This was a prospective interventional case series conducted in a tertiary university-based hospital. Biopsy proven patients with active ISOIS were included. Systematic criteria were developed to define and measure disease activity and monitor response to treatment. A DMD regimen including an anti-tumor necrosis factor alpha (anti-TNF alpha) agent plus azathioprine and low-dose corticosteroids were used. Comprehensive ophthalmic, orbital and systemic assessments were performed during each visit. RESULTS: Five eligible patients with primary ISOIS were included. Mean age was 34.20 (SD = 13.33, range 19-53) years. Three had unilateral and two had bilateral involvement. Four had diffuse orbital involvement pattern and progressive worsening of visual functions, reduced extraocular motility and proptosis. In one patient the disease was localized to extraocular muscle and lacrimal gland. Disease activity was decreased and stabilized after DMDs regimen in all patients. Mean follow up was 32.80 (SD = 30.80, range: 12-86) months. CONCLUSION: Biologic DMD (b-DMD) including anti-TNF alpha, corticosteroid and azathioprine were effective in decreasing disease activity and could change course of the disease. This study supports the concept of using b-DMD regimen in treatment of ISOIS.

Rituximab for treatment of non-infectious and non-malignant orbital inflammatory disease.

PURPOSE: To provide a comprehensive review of rituximab use for the treatment of non-infectious/non-malignant orbital inflammation. METHODS: Review of literature through January 2021. RESULTS: Individual data was available for 167 patients with refractory non-infectious/non-malignant orbital inflammation who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (108/149, 72.5%) at a mean of 44.6 months following the diagnosis of orbital inflammation (range = 0 to 360 months; median = 13.7 months). Patients with non-infectious/non-malignant orbital inflammation either received prior treatment with corticosteroids only (27/122, 22.1%), or with one (31/122, 25.4%), two (25/122, 20.5%), or three or more (25/122, 20.5%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (80/144, 55.6%), followed by the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; 51/144, 35.4%). Various other off-label regimens were used infrequently (13/144, 9.0%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with orbital inflammation (146/166, 88.0%). Commonly treated diagnoses included granulomatosis with polyangiitis (99/167, 59.3%), IgG-4 related disease (36/167, 21.6%), and orbital inflammation of indeterminate cause (25/167, 15.0%). No side effects were reported in 83.3% (55/66) of cases. The most common RTX-induced adverse event was an infusion-related temporary exacerbation of orbital disease (4/66, 6.1%), which occurred prior to the routine use of systemic corticosteroids as pre-conditioning. CONCLUSIONS: Overall, RTX appears to be both efficacious and well-tolerated as second- or third-line therapy for patients with non-infectious/non-malignant orbital inflammation.

[Idiopathic orbital inflammation]. / Idiopathische orbitale Entzündungen.

BACKGROUND: Orbital inflammatory disease encompasses a spectrum of disorders. Idiopathic orbital inflammation (IOI) is often a diagnosis of exclusion, which needs to be differentiated from infections, systemic inflammatory disease, and neoplasms. IOI includes anterior inflammation with dacryoadenitis, myositis, perineuritis of the optic nerve, periscleritis, diffuse sclerosing inflammation, and orbital apex inflammation. OBJECTIVE: A differential diagnostic overview of IOI is presented, including its subcategories, diagnosis, and treatment. CONCLUSION: The diagnosis of IOI is often made by exclusion with typical clinical findings, CT and MRI scans, and pathology. Treatment includes corticosteroids, immunomodulators, immunosuppressants, and radiotherapy.

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.

Not a Tumor-Nonspecific Orbital Inflammation.

Objective This study was aimed to illustrate the features and complexities of nonspecific orbital inflammation via discussion of two representative cases. Design Present study is a retrospective case review. Setting The study was conducted at a tertiary care medical center. Participants Two patients with nonspecific orbital inflammation were participants of this retrospective study. Main Outcome Measures Outcome of the study was disease-free patients and off all medications. Results At follow-up, both patients are disease free and off all medications. Conclusion Surgery plays a diagnostic and therapeutic role. While the clinical subtype is important for differential diagnosis and symptomatic treatment, the histologic subtype is similarly important. For inflammatory dacryoadenitis, surgery can be therapeutic. For extensive granulomatosis with polyangiitis, debulking surgery may allow better penetration of medications, especially rituximab.