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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A 62-year-old Chinese woman, with a history of immunoglobulin A nephropathy, presented with an ulcerative conjunctival lesion and anterior orbital inflammation. No infective foci were found, and her symptoms were not alleviated by antibiotic treatment or drainage of the lesion. She was diagnosed with idiopathic orbital inflammatory disease, also known as orbital pseudotumor, which completely resolved with oral corticosteroid treatment. To the best of our knowledge, this is the first documented case of idiopathic orbital inflammatory disease that initially presented with an ulcerative conjunctival lesion. We illustrate our stepwise diagnostic approach and management of the disease. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

PurposeThis report will describe a case of orbital pseudotumor that is associated with underlying Crohn's disease in a pediatric patient.ObservationsAn 8-year-old male with a past medical history of chronic constipation who presented to the ophthalmologist in July 2017 with a 7-month history double vision, left upper lid ptosis, left abducens nerve palsy, and an abnormal thyroid test. The patient's family history was negative for any autoimmune disease including, juvenile idiopathic arthritis, rheumatoid arthritis, thyroid disease, type 1 diabetes mellitus or inflammatory bowel disease. Diagnosis of orbital pseudotumor of the left eye was made based on CT scan findings and he was then treated with a one-week course of oral prednisone. After resolution of his initial symptoms, he presented a month later with swelling in his left eye and was treated with a 6-month steroid taper with resolution of symptoms. In June 2018, the patient presented with swelling in his right eye and was treated with prednisone plus steroid sparing agents. Extraocular muscle biopsy was negative for IgG4 related disease, fungal infection, or malignant lymphoma and workup for sarcoidosis and granulomatosis with polyangiitis was unremarkable. In September 2018, the patient presented with bloody stools, diagnosed and treated for a perirectal abscess. Subsequent colonoscopy performed in January 2019 confirmed Crohn's disease. He is currently undergoing treatment with adalimumab and is in remission in terms of orbital pseudotumor.Conclusion and importanceIn conclusion, although the association between orbital pseudotumor and Crohn's disease is very rare, medical professionals should remember this connection when a patient presents with idiopathic orbital pseudotumor. To rule out this possibility, we recommend a thorough history of GI findings should be taken on the initial patient encounter. Crohn's disease may be an underlying cause of certain cases of orbital pseudotumor, and treatment and control of the underlying Crohn's disease may help to reduce recurrence rates of orbital pseudotumor. Additional studies need to be performed to better understand the association between the two diseases.

To examine the clinical and histopathologic characteristics of lacrimal gland biopsies at a tertiary academic center. 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. 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%). 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract 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.

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

Background: The coexistence of thyroid-associated orbitopathy (TAO) and idiopathic orbital inflammatory diseases (IOID) is rare. They mimic each other clinically, and it is challenging to differentiate the two. We are presenting a case of bilateral proptosis resulting from concomitant TAO and IOID. Case Report: A 44-year-old female presented with swelling, pain, and protrusion of both eyes for one month. She had a past history of trauma in the right eye (RE) 15 years ago. Her best-corrected visual acuity in RE was hand movements and 20/30 in the left eye (LE). There was lid edema, conjunctival congestion, chemosis with axial proptosis, and restricted extraocular movements in both eyes (BE). The anterior segment of RE revealed sequels of old anterior traumatic uveitis and it was normal in the LE. The B scan done in BE showed enlargement of all extraocular (EOM). CT scan orbit showed enlargement of all the EOM belly and tendon insertions. Thyroid profile found to have high anti-TPO (thyroid peroxidase) suggestive of Hashimoto's thyroiditis. The patient was started on intravenous methylprednisolone and showed significant improvement. Conclusion: TAO and IOID can present together. This case report highlights the need for clinicians to be aware of this condition.

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. [ABSTRACT FROM AUTHOR]

Abstract 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.

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. 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. 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. Systemic rituximab therapy seems to be an effective therapy for IOI as salvage or first-line therapy with long-term clinical durability. [ABSTRACT FROM AUTHOR]

Nonspecific orbital inflammation (NSOI), also known as orbital pseudotumor, is a condition characterized by inflammation in the tissues around the eye socket (orbit) without a clearly identifiable cause. This inflammatory disorder can affect various structures within the orbit, including muscles, fat, and connective tissues, leading to symptoms such as pain, swelling, and changes in vision. A 74-year-old man with a history of previous orbital trauma presented with acute-onset head and orbital pain, followed by restricted left eye movements in all directions, left ptosis, and a dilated left pupil. Orbital imaging revealed bilateral inflammation of the lateral rectus muscles and orbital fat, suggestive of bilateral NSOI, while brain and laboratory studies ruled out other differential diagnoses. The presence of left ptosis, a dilated pupil, and limited upward, downward, and inward movements in the left eye suggested intraorbital involvement of both the superior and inferior divisions of the left third nerve. The complete resolution of orbital symptoms and third nerve function after systemic corticosteroid therapy supported the inflammatory nature of the nerve involvement in this case. The case is notable in terms of bilateral involvement in adult-onset NSOI, the possible role of previous orbital trauma in the development of the disease, and the inflammatory involvement of third nerve divisions following the extension of inflammation into the orbital tissues. NSOI can mimic other, more serious conditions, making accurate diagnosis crucial for effective management and treatment. Understanding its presentation, potential causes, and appropriate diagnostic approaches is essential in providing optimal care for patients affected by this complex inflammatory condition., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Ghumman et al.)

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.

Background: The purpose of this study was to describe the radiologic and histopathologic features of lacrimal gland in patients presenting with lacrimal gland enlargement. Methods: We retrospectively retrieved the data of patients with lacrimal gland enlargement in Farabi Eye Hospital between 2012 and 2017. These data included demographics, the patients' facial photographs, orbital CT-scans, and histopathological findings of lacrimal gland biopsies. Results: Forty-seven patients (15 men and 32 women) were enrolled in this study with a median age of 37.9 years (range, 15-79 years). Histopathologic diagnoses were chronic dacryoadenitis in 26 cases (55.32%), IgG4-related disease in 6 patients (12.77%), two cases of acute dacryoadenitis, two cases of non-necrotizing granulomatous inflammation, two cases of Non-Hodgkin's B-cell lymphoma, two cases of adenoid cystic carcinoma and two cases of mixed tumor (4.26% each), as well as one case of conjunctival epithelial cyst, and one case of benign lymphoid tissue and fibrofatty tissue (2.13%). In two samples (4.26%), biopsy revealed normal lacrimal glands. Interestingly, in two cases with relapsing lacrimal gland enlargement, different histopathologic diagnoses were found in biopsies taken from each lacrimal gland at different times. The average size of enlarged lacrimal glands was 19.67 mm x 7.06 mm on axial CT scan and 19.44 mm x 6.20 mm on coronal CT scan. Conclusion: Tissue biopsy is needed for diagnosis of lacrimal gland enlargement because it is difficult to distinguish the type of the lacrimal gland pathology based solely on clinical or radiological presentation. [ABSTRACT FROM AUTHOR]

Biopsies and ANCA testing for limited forms of granulomatosis with polyangiitis (GPA) are frequently non-diagnostic. We characterized gene expression in GPA and other causes of orbital inflammation. We tested the hypothesis that a sub-set of patients with non-specific orbital inflammation (NSOI, also known as pseudotumor) mimics a limited form of GPA. Formalin-fixed, paraffin-embedded orbital biopsies were obtained from controls (n=20) and patients with GPA (n=6), NSOI (n=25), sarcoidosis (n=7), or thyroid eye disease (TED) (n=20) and were divided into discovery and validation sets. Transcripts in the tissues were quantified using Affymetrix U133 Plus 2.0 microarrays. Distinct gene expression profiles for controls and subjects with GPA, TED, or sarcoidosis were evident by principal coordinate analyses. Compared with healthy controls, 285 probe sets had elevated signals in subjects with GPA and 1472 were decreased (>1.5-fold difference, false discovery rate adjusted p

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. [ABSTRACT FROM AUTHOR]

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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.

Copyright of Journal of Pediatric Infection / Çocuk Enfeksiyon Dergisi is the property of Journal of Pediatric Infection / Cocuk Enfeksiyon Dergisi and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. [ABSTRACT FROM AUTHOR]

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To assess arterial spin-labeling (ASL) and diffusion-weighted imaging (DWI) and in combination for differentiating between idiopathic orbital inflammatory pseudotumor (IOIP) and orbital lymphoma. A retrospective study was done on 37 untreated patients with orbital masses, suspected to be IOIP or orbital lymphoma that underwent ASL and DWI of the orbit. Quantitative measurement of tumor blood flow (TBF) and apparent diffusion coefficient (ADC) of the orbital lesion was done. There was a significant difference (P = 0.001) in TBF between patients with IOIP (n = 21) (38.1 ± 6.2, 40.3 ± 7.1 ml/100 g/min) and orbital lymphoma (n = 16) (55.5 ± 7.1, 56.8 ± 7.9 ml/100 g/min) for both observers respectively. Thresholds of TBF used for differentiating IOIP from orbital lymphoma were 48, 46 ml/100 g/min revealed area under the curve (AUC) of (0.958 and 0.921), and accuracy of (86% and 83%) for both observers respectively. There was a significant difference (P = 0.001) in ADC between patients with IOIP (1.04 ± 0.19, 1.12 ± 0.23 × 10−3 mm2/s) and orbital lymphoma (0.69 ± 0.10, 0.72 ± 0.11 × 10−3 mm2/s) for both observers respectively. Thresholds of ADC used for differentiating IOIP from orbital lymphoma were 0.84 and 0.86 × 10−3 mm2/s with AUC of (0.933 and 0.920), and accuracy of 89% and 90% for both observers respectively. The combined TBF and ADC used for differentiating IOIP from orbital lymphoma had AUC of (0.973 and 0.970) and accuracy of (91% and 89%) for both observers respectively. TBF and ADC alone and in combination are useful for differentiating IOIP from orbital lymphoma. • Significant difference (P = 0.001) in TBF between patients with IOIP and orbital lymphoma • Significant difference (P = 0.001) in ADC between patients with IOIP and orbital lymphoma • Combined TBF and ADC can differentiate IOIP from orbital lymphoma. [ABSTRACT FROM AUTHOR]

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., (Copyright © 2024 Sociedad Española de Neurocirugía. Published by Elsevier España, S.L.U. All rights reserved.)

Purpose: This report will describe a case of orbital pseudotumor that is associated with underlying Crohn's disease in a pediatric patient. Observations: An 8-year-old male with a past medical history of chronic constipation who presented to the ophthalmologist in July 2017 with a 7-month history double vision, left upper lid ptosis, left abducens nerve palsy, and an abnormal thyroid test. The patient's family history was negative for any autoimmune disease including, juvenile idiopathic arthritis, rheumatoid arthritis, thyroid disease, type 1 diabetes mellitus or inflammatory bowel disease. Diagnosis of orbital pseudotumor of the left eye was made based on CT scan findings and he was then treated with a one-week course of oral prednisone. After resolution of his initial symptoms, he presented a month later with swelling in his left eye and was treated with a 6-month steroid taper with resolution of symptoms. In June 2018, the patient presented with swelling in his right eye and was treated with prednisone plus steroid sparing agents. Extraocular muscle biopsy was negative for IgG4 related disease, fungal infection, or malignant lymphoma and workup for sarcoidosis and granulomatosis with polyangiitis was unremarkable. In September 2018, the patient presented with bloody stools, diagnosed and treated for a perirectal abscess. Subsequent colonoscopy performed in January 2019 confirmed Crohn's disease. He is currently undergoing treatment with adalimumab and is in remission in terms of orbital pseudotumor. Conclusion and Importance: In conclusion, although the association between orbital pseudotumor and Crohn's disease is very rare, medical professionals should remember this connection when a patient presents with idiopathic orbital pseudotumor. To rule out this possibility, we recommend a thorough history of GI findings should be taken on the initial patient encounter. Crohn's disease may be an underlying cause of certain cases of orbital pseudotumor, and treatment and control of the underlying Crohn's disease may help to reduce recurrence rates of orbital pseudotumor. Additional studies need to be performed to better understand the association between the two diseases.

We report a case of exacerbation of posterior scleritis and orbital pseudotumor in a patient with granulomatosis polyangiitis, with initial rituximab infusion. Modifications in rituximab protocols including reduction in maintenance dose for subsequent infusions with increase in premedication dose of intravenous methyl prednisolone may be useful to prevent periinfusional flares. In this case report, we highlight a rare occurrence and modification in rituximab protocol to prevent post-infusional flare of inflammation.

Orbital inflammatory syndrome (OIS) or orbital pseudotumor is the most frequent cause of an orbital mass. Most cases are steroid responsive, but treatment of patients with refractory OIS may also include radiotherapy. Presently described are 3 cases of orbital pseudotumor that had a partial steroid response or recurrence and were treated successfully with CyberKnife (Accuray Inc., Sunnyvale, CA, USA).

Abstract Background To report a case of orbital inflammation after bisphosphonate infusion in a patient who was already receiving immunosuppressive therapy. Case presentation A 56-year-old woman presented to the ophthalmology clinic with acute onset of right eye pain 24 h after receiving her first Zolendronic acid infusion. She has a past medical history of chronic inflammatory demyelinating polyneuropathy, Sjogren’s syndrome, and systemic lupus erythematosus that have been controlled with immunosuppressive therapy for three years. Clinical ophthalmic exam and MRI studies were significant for right orbital inflammation. The patient was started on oral prednisone with rapid resolution of symptoms. Conclusions This is the first case report of a patient receiving chronic immunosuppressive therapy to develop orbital inflammation after Zoledronic acid infusion. In addition, it demonstrates that corticosteroids can be an effective first line therapy in treating orbital inflammation in similar patients. Physicians should be aware of this rare but serious potential side effect of bisphosphonates, and have bisphosphonate-related orbital inflammation on their differential for proper initiation of treatment.

Lacrimal gland lesions account for approximately 9 to 10% of all biopsied orbital masses. Potential causes include nongranulomatous and granulomatous inflammation, autoimmune disease, lymphoproliferative disorders, benign epithelial proliferation, malignant neoplasia, and metastatic disease. Inflammatory lesions and lymphoproliferative disorders are the most common and may be unilateral or bilateral; they may also be localized to the orbit or associated with systemic disease. Both benign and malignant epithelial lacrimal gland masses tend to be unilateral and involve the orbital lobe, but a more rapid onset of symptoms and periorbital pain strongly suggest malignant disease. On orbital imaging, both inflammatory and lymphoproliferative lesions conform to the globe and surrounding structures, without changes in adjacent bone, whereas epithelial lacrimal gland masses often show scalloping of the lacrimal gland fossa. Malignant epithelial lacrimal gland tumors can also have radiographic evidence of bony invasion and destruction. Masses of the lacrimal gland may be due to a broad range of pathologies, and a good working knowledge of common clinical characteristics and radiographic imaging findings is essential for diagnosis and treatment. All patients with inflammatory, lymphoproliferative, and epithelial neoplastic lesions involving the lacrimal gland require long-term surveillance for disease recurrence and progression. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the effectiveness of bag-of-features (BOF)-based radiomics for differentiating ocular adnexal lymphoma (OAL) and idiopathic orbital inflammation (IOI) from contrast-enhanced MRI (CE-MRI).Methods: Fifty-six patients with pathologically confirmed IOI (28 patients) and OAL (28 patients) were randomly divided into training (n = 42) and testing (n = 14) groups. One hundred sixty texture features extracted from the CE-MR image were encoded into the BOF representation with fewer features. The support vector machine (SVM) with a linear kernel was used as the classifier. Data augmented was performed by cropping orbital lesions in different directions to alleviate the over-fitting problem. Student's t test and the Holm-Bonferroni method were employed to compare the performance of different analysis methods. The chi-square test was used to compare the analysis with MRI and human radiological diagnosis.Results: In the independent testing group, the differentiation by the BOF features with augmentation achieved an area under the curve (AUC) of 0.803 (95% CI: 0.725-0.880), which was significantly higher than that of the BOF features without augmentation and that of the texture features (p < 0.05). In addition, the same radiomic analysis with pre-contrast MRI obtained an AUC of 0.618 (95% CI: 0.560-0.677), which was significantly lower than that with CE-MRI. The diagnostic performance of the analysis with CE-MRI was significantly better than the radiology resident (p < 0.05) but had no significant difference with the experienced radiologist, even though there was less consistency between the radiomic analysis and the human visual diagnosis.Conclusions: The BOF-based radiomics may be helpful for the differentiation between OAL and IOI.Key Points: • It is challenging to differentiate OAL from IOI due to the similar clinical and image features. • Radiomics has great potential for the noninvasive diagnosis of orbital diseases. • The BOF representation from patch to image may help the differentiation of OAL and IOI. [ABSTRACT FROM AUTHOR]