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RESUMO 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. ABSTRACT 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 café-au-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.

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.Multicentric retrospective study.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.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.

Orbital inflammatory disease (OID), commonly known as orbital pseudotumour, is an inflammatory disease of unknown cause. It has different forms of presentation and different degrees of severity. Its variable nature is the main cause for this disease to be misdiagnosed and misclassified. The prognosis of OID depends on the tissues affected and the histology. OID usually responds favourably to systemic steroid treatment. However, empiric steroids may mask other underlying diseases that respond well to this treatment as well, namely, IgG4-related disease or lymphoproliferative disorders. This fact has led to controversy among various authors as some recommend performing a biopsy in most of the cases, whereas others defend that this procedure should only be performed if the patient has not responded to empiric steroid treatment. Although steroids have been the mainstream treatment of OID, the side effects, relapse rates and lack of response in some cases have resulted in them being replaced by immunosuppressive and immunomodulator therapies that currently stand as a key steroid-sparing treatment option, in addition to radiotherapy and surgery. The aim of this review is to update the evidence on the diagnosis and treatment of OID.

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.

Orbitale Entzundungen umfassen ein Spektrum von Erkrankungen. Die idiopathische orbitale Entzundung (IOI) ist meist eine Ausschlussdiagnose, die von Infektionen, systemischen entzundlichen Erkrankungen und Tumorerkrankungen abgegrenzt werden muss. Die IOI umfasst die anteriore Entzundung mit Dakryoadenitis, Myositis, Perineuritis des Sehnervs, Periskleritis, die diffuse sklerosierende Entzundung und die Entzundung der Orbitaspitze. Es erfolgte die differenzialdiagnostische Darstellung des Krankheitsbilds mit seinen Unterkategorien sowie mit Diagnostik und Therapieubersicht. Die Diagnose der IOI ist vielfach eine Ausschlussdiagnose mit typischen Zeichen in klinischem Befund, Computertomographie (CT) und Magnetresonanztomographie (MRT) und Histologie. Die Therapie umfasst Kortikosteroide sowie Immunmodulatoren, Immunsuppressiva und Strahlentherapie.

Aims Orbital inflammatory pseudotumor is considered a non-neoplastic inflammatory process. The finding of clonality of B or T-cell receptors in cases pathologically diagnosed as orbital inflammatory pseudotumor has unknown clinicopathologic significance. We sought to investigate potential B and T-cell clonality and concomitant diseases in cases pathologically diagnosed as orbital inflammatory pseudotumor. Methods Cases diagnosed as orbital inflammatory pseudotumor at our institution were retrospectively analyzed. Hematoxylin and eosinstained slides, immunohistochemically stained slides and polymerase chain reactions on cell block material for the investigation of clonality of B and T-cell receptors were evaluated, to confirm the diagnosis and investigate the prevalence of concomitant diseases. Results A total of 13 cases showing characteristic histopathologic features of orbital inflammatory pseudotumor were identified. CD138, IgG, and IgG4 showed varying numbers of plasma cells in each case, with 5 cases (5/13, 38%) exhibiting relative increase in the presence of IgG4 plasma cells. However, no cases showed diagnostic findings of IgG4-related disease (IgG4-RD). polymerase chain reactions analysis showed clonal B-cell populations in 2 cases (2/13, 15%). No cases showed anaplastic lymphoma kinase expression by immunohistochemistry. There were no clinical reports of progression to lymphoma or development of systemic IgG4-RD in any of the patients (average follow-up of 300 days), with 38% of patients showing systemic autoimmune conditions. Conclusion A small but significant percentage of typical orbital inflammatory pseudotumor on histology showed B-cell clonality on polymerase chain reactions analysis of B-cell receptors, or features suggestive, but not diagnostic of IgG4-RD. Close follow-up of these patients to identify development of lymphoma, systemic IgG4-RD, or other rheumatologic conditions may be clinically warranted.

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.

Significance Idiopathic sclerosing orbital inflammation (ISOI) is characterized by insidious, chronic, progressive inflammation and fibrosis that damage ocular structures and produce a mass effect. This case highlights the challenges in diagnosis and management of ISOI, as well as the associated ocular morbidities, including potential vision loss. Purpose The purpose of this study was to provide education regarding a rare condition that exhibits variable presentation and has an unpredictable success rate with regard to treatment paradigm. Improved therapeutic options are promising. Ultimately, early detection and management are key and may allow for better visual outcome. Case report A 46-year-old woman presented with complaints of chronic right-sided facial headaches and eye pain and gradual right globe prominence over the previous 6 months. Worsening vision and decreased right peripheral visual field were also noted. Upon examination, an afferent pupillary defect and florid disc edema were evident. Imaging studies revealed an orbital and extraorbital infiltrative mass involving the right orbital apex, inferior orbital fissure, pterygopalatine fossa, and cavernous sinus. Right anterior orbitotomy with biopsy revealed fragments of fibroconnective and adipose tissue with sclerosis and chronic focal inflammation, consistent with ISOI. Treatment included intravenous methylprednisone, followed by oral prednisone, beginning at 60 mg/d with a slow taper thereafter. Signs and symptoms improved dramatically and eventually resolved. Vision significantly improved, and the afferent pupillary defect resolved. The patient remained asymptomatic at 3-month follow-up. Conclusions Idiopathic sclerosing orbital inflammation is difficult to diagnose and manage. No large studies exist because of the rare nature of the disease. Slowly progressive, nonspecific signs and symptoms may delay recognition and treatment. Orbital imaging and histopathologic analysis are critical for definitive diagnosis. Conventional treatment with corticosteroids is not uniformly successful, but newer combined therapy options can improve outcomes. Early identification and treatment are key to management and ultimate preservation of function and vision.

Purpose 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. Material and methods 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. Results 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. Conclusion TBF and ADC alone and in combination are useful for differentiating IOIP from orbital lymphoma.

The periorbital area is probably the most important aesthetic unit to show early signs of ageing due to volume depletion of soft tissues. Dermal filler injections have been increasingly used as a p...

Eosinophilic angiocentric fibrosis (EAF) is an infrequent and slowly progressive disease, represented by fibroinflammatory lesions of unknown origin, which mainly involves the sinonasal structures and upper respiratory tract. Occasionally, it can affect the orbit and ocular adnexa causing symptoms such as proptosis, globe displacement and periorbital edema. In very rare cases, ocular manifestation as an orbital inflammatory pseudotumor can be the primary localization of the disease. Current literature proposes a relation between EAF and immunoglobulin G4-related disease spectrum. We describe the case of a 69-year-old man presented with antecedents of left periorbital edema, epiphora and retroocular pain. Examination showed a nonaxial proptosis, severe limitation in left eye adduction and lateral globe displacement. Orbital imaging revealed a left medial orbital mass with involvement of the inferior rectus and the medial rectus muscles. An orbital biopsy of the mass illustrated an inflammatory infiltrate with a notable eosinophilic component, “onion-skin appearance” of vessels and surrounding concentric fibrosis, highly suggestive of EAF. Further investigations showed a high expression of IgG4 and excluded other possible diseases. There was a favorable evolution of the orbital inflammatory pseudotumor following a 4-month treatment course with oral glucocorticoids. Abbreviations: EAF = Eosinophilic angiocentric fibrosis, CT = Computed tomography, MRI = Magnetic resonance imaging, GPA = Granulomatosis with polyangiitis, EGP = eosinophilic granulomatosis with polyangiitis, MPA = microscopic polyangiitis, ANCA = Anti-neutrophil cytoplasmic antibodies, Ig = Immunoglobulin

Background Idiopathic orbital pseudotumour is rare in children. We report a case of bilateral paediatric idiopathic orbital pseudotumour and review the characteristics of this case. Case presentation A 14-year-old female patient presented at our Department of Pulmonary and Critical Care Medicine (PCCM) with complaints of recurrent severe cold and cough for 3 weeks, which had been treated with an intravenous antibiotic. Meanwhile, the patient developed swelling of both eyelids during the period of cold and cough, but her symptoms did not improve after the ocular administration of tobramycin dexamethasone eye drops. The patient was referred from the respiratory medicine ward to our department because of gradually worsening ocular pain, visual deterioration, increased intraocular pressure and serious nausea/vomiting within 24 h of hospitalization. The diagnosis of bilateral idiopathic orbital pseudotumour was made ultimately because of the course of the disease, exclusion of diagnoses such as bacterial ocular infection or malignant tumours and subsequent evidence from orbital magnetic resonance imaging (MRI). Favourable progress in the ocular tension and eyelid swelling was achieved through treatment with intravenous dexamethasone. The binocular intraocular pressure gradually declined to approximately 15 mmHg, and there was favourable progression in the patient’s vision to 20/40 in both eyes on the ninth day of hospitalization. Conclusions Our patient developed rapidly progressive acute orbital signs and symptoms and anterior inflammation, such as pain, proptosis, limited ductions, periorbital oedema, chemosis, vision loss and high intraocular pressure. This case highlights that idiopathic orbital pseudotumour is an uncommon but important cause of acute orbital syndrome in children.

PURPOSE: To investigate the clinical features and treatment outcomes of IgG4-related ophthalmic disease (IgG4-ROD) among idiopathic orbital inflammatory disease (IOID) patients. METHODS: The medical records of 165 biopsy-proven IOID patients were retrospectively reviewed. Biopsy specimens were immunostained to detect IgG4 and IgG, and data regarding the clinicopathologic features, treatment outcomes, and recurrence were analyzed. RESULTS: Among the 165 IOID patients enrolled, 100 (60.6%) were histopathologically IgG4-positive. The IgG4-positive patients had a lower rate of painful swelling or mass (17.0% versus 33.8%, p = 0.013), a longer symptom duration (p = 0.070), and a lower proportion of eyelid hyperemia (39.0% versus 58.5%, p = 0.014) than the IgG4-negative patients. Increased Ki-67 expression (15.02 ± 6.86%, p

A 27-year-old woman presented with acute onset of double vision, right-sided orbital pain exacerbated by ocular movements, and ipsilateral conjunctival hyperemia. Her personal medical history was negative and she denied any recent illness, journey, or sick contact. Family history was positive for Hashimoto thyroiditis in her mother. Neurologic evaluation revealed complete lateral and partial upward gaze palsy in her right eye, with binocular horizontal diplopia. The remaining clinical examination was unremarkable; in particular, pupillary reflex was preserved and there was no evidence of ptosis, conjunctival chemosis, proptosis, eye discharge, or visual defects.

Purpose To compare the clinical features, treatment outcomes, and prognoses of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and idiopathic sclerosing orbital inflammation (ISOI). Methods This retrospective case series included 40 and 22 biopsy-proven cases of IgG4-ROD and ISOI, respectively. The authors reviewed medical charts and images to determine the clinical presentation, involvement pattern, and treatment outcomes. The main outcome measures were differences in disease manifestation, treatment modalities and efficacy, medication-free remission rate, and predictive factors for remission. Results The median ages were 51 (range 31-72) and 39 (range 9-73) years in the IgG4-ROD and ISOI groups, respectively. The most frequently involved sites were the lacrimal gland (39 cases, 98%) in IgG4-ROD patients and the extraconal region (15 cases, 68%) in ISOI patients. No significant difference was observed in the initial treatment response between the groups. Medication-free remission was achieved in 22 (55%) IgG4-ROD patients and 10 (45%) ISOI patients; none of the IgG4-ROD patients showed permanent ocular dysfunction, while half of the ISOI patients had permanent visual loss or limited ocular motilities. Multivariate analysis indicated that extraocular muscle enlargement (odds ratio, 0.11; 95% confidence interval, 0.01-0.98) and IgG4:IgG ratio on histopathology (odds ratio, 0.08; 95% confidence interval, 0.01-0.86) were negatively associated with medication-free remission in IgG4-ROD patients. Conclusions Although IgG4-ROD and ISOI share common histological findings of mass-forming sclerosis, IgG4-ROD frequently has indolent clinical courses, whereas ISOI often demonstrates more an aggressive clinical course, resulting in ocular dysfunction even after remission. Different pathogeneses may account for the different disease characteristics.

ObjectiveIdiopathic orbital inflammation (IOI) is a group of orbital inflammatory diseases of unknown etiopathogenesis. We investigated whether patients with IOI have clinical heterogeneity based on the presence (typical group) or absence (atypical group) of a unique onset that periocular inflammatory symptoms emerge suddenly but progress slowly.Methods and analysisThis retrospective cohort study included 195 patients diagnosed with IOI. We analysed the clinical data of patients, including the outcomes of corticosteroid treatment, in two subgroups stratified on the basis of the presence (130 patients) or absence (65 patients) of the unique onset.ResultsPatients in the typical group were significantly younger at disease onset than those in the atypical group (median age; 52 vs 65 years, p=0.002); had more ocular adnexa-specific lesions, namely, dacryoadenitis, myositis, scleritis and optic perineuritis (78% vs 45%, p=0.00001); and had significantly fewer associations with immune-mediated inflammatory diseases (4% vs 15%, p=0.004). Among 30/119 patients (25%) who were steroid refractory in the typical group, a long period of time from symptom onset to initiation of treatment was a significant steroid-refractory risk factor (OR: 16.7), whereas, among the 18/40 patients (45%) who were steroid refractory in the atypical group, intraconal diffuse lesions were a significant steroid-refractory risk factor (OR: 8.8).ConclusionThis cohort study suggests clinical heterogeneity between the two subgroups of patients with IOI.

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.

Orbital pseudotumor (OST) is a chronic inflammatory disease affecting Orbital tissue around the eye in a behavior just like a tumor but it does not invade or metastasize. Treatment of OST may be conservative in mild cases while in patients with significant moderate to severe course need treatment that may be medical or surgical. Objective: Compare between corticosteroid and methotrexate effectiveness and less complications in treatment of OST. Method: A case series study (47 females and 28 males) with proptosis of one or both eyes, redness of the eye or periorbital pain either alone or in combination. Patients having clinical feature suggestive of OST with threat to the eye function or its surrounding muscles and neurovascular tissue that need medical treatment included in this study while others that need surgical treatment excluded from this study. Results: This study made on 75 patients having clinical feature rise the suspension of OST, all ages involved with high incidence at 6th decade followed by the 2nd decade. Group A patients get response to treatment progressively in the 1st three months but later after one year of follow up the percentage decrease to about 45% while in group B although there is slow response to the treatment but with follow up after one year about 87.5% have a good response to treatment. Conclusion: This study suggest that Methotrexate is a well-tolerated medication in patients with Orbital Pseudotumor disease to avoid Corticosteroid therapy failure.

Background Orbital compartments harbor a variety of tissues that can be independently targeted in a plethora of disorders resulting in sight-threatening risks. Orbital inflammatory disorders (OID) including Graves’ ophthalmopathy, sarcoidosis, IgG4 disease, granulomatosis with polyangiitis, and nonspecific orbital inflammation constitute an important cause of pain, diplopia and vision loss. Physical examination, laboratory tests, imaging, and even biopsy are not always adequate to classify orbital inflammation which is frequently deemed “nonspecific”. Tear sampling and testing provide a potential “window” to the orbital disease process through a non-invasive technique that allows longitudinal sampling as the disease evolves. Using PubMed/Medline, we identified potentially relevant articles on tear proteomics published in the English language between 1988 and 2021. Of 303 citations obtained, 225 contained empirical data on tear proteins, including 33 publications on inflammatory conditions, 15 in glaucoma, 15 in thyroid eye disease, 1 in sarcoidosis (75)(75) and 2 in uveitis (77,78)(77,78). Review articles were used to identify an additional 56 relevant articles through citation search. In this review, we provide a short introduction to the potential use of tears as a diagnostic fluid and tool to investigate the mechanism of ocular diseases. A general review of previous tear proteomics studies is also provided, with a focus on Graves' ophthalmopathy (GO), and a discussion of unmet needs in the diagnosis and treatment of orbital inflammatory disease (OID). The review concludes by pointing out current limitations of mass spectrometric analysis of tear proteins and summarizes future needs in the field.

Background To identify the prevalence of positive IgG4 immunostaining in orbital tissue among patients previously diagnosed with nongranulomatous idiopathic orbital inflammation (IOI) and to compare the clinical characteristics of patients with and without IgG4-positive cells. Methods A retrospective review of all patients with a histopathologic diagnosis of IOI was performed. Immunohistochemical staining was performed to identify IgG-positive cells and IgG4-positive cells. Multivariate analysis was performed using likelihood ratio-test logistic regression on the differences between IgG4-related disease (IgG4-RD) and non-IgG4-RD. Results Of the 45 patients included, 21 patients (46.7%) had IgG4-positive cells, with 52.4% being male and a mean age of 55.9 ± 13.4 years. Bilateral ocular adnexal involvement (adjusted odds ratio [aOR] = 9.45; P = 0.016) and infraorbital nerve enlargement (aOR = 12.11; P = 0.008) were frequently found in IgG4-RD patients. Complete remission occurred in 23.8% of IgG4-RD patients and 41.7% of non-IgG4-RD patients. IgG4-RD patients had more frequent recurrent disease than non-IgG4-RD patients. Conclusions Nearly 50% of IgG4-RD patients were previously diagnosed with biopsy-proven IOI. IgG4-RD was more frequent in patients with bilateral disease and infraorbital nerve enlargement, showing the importance of tissue biopsy in these patients. Immunohistochemistry studies of all histopathology slides showing nongranulomatous IOI are highly recommended to evaluate for IgG4-RD.

Purpose: To evaluate the clinical characteristics, histopathology, and treatment outcomes in adult and pediatric patients with nonspecific orbital inflammation (NSOI). Methods: This retrospective study evaluates 76 patients with NSOI. The patients were categorized in 9 groups according to the site of involvement and histopathology results. These groups included: anterior involvement, dacryoadenitis, myositis, perineural involvement, acute fat involvement, focal mass, orbital apex involvement, diffuse sclerosing form, and multiple tissue involvement. The course of the disease was categorized as acute, subacute, or chronic. The cases with symptom duration of less than 1 week were classified as acute, 1 week to 1 month as subacute, and more than 1 month as chronic. Results: 36 (47.4%) patients were males. The mean age was 41.68 ± 17.62 (6–75) years. The most common signs and symptoms were periorbital pain, periorbital edema, decreased ocular movements or diplopia, and conjunctival injection. The most common group was dacryoadenitis in 29 (38.1%) cases. The most common form of disease was the acute involvement (50% of patients). Most of the patients were treated by oral corticosteroids. Duration of follow-up was 7.17 ± 6.26 months. Recurrence occurred in 9 (11.8%) of patients during the follow-up period. Conclusions: This study presents a new categorization in which multiple tissue involvements were separated. Some of the NSOI features differ between adults and children. In most patients, treatment especially with corticosteroids, resolves the clinical findings. Keywords: Idiopathic orbital inflammation, Clinical, Histopathology, Orbital pseudotumor, Pediatric

Idiopathic orbital inflammation developed in the right orbit of a woman in her mid-thirties, causing tearing, photophobia, diplopia, altered depth perception, proptosis, and pain on eye movements. Computed tomography disclosed a mass involving the intraconal and extraconal nasal right orbit, extending to the orbital apex with anterior displacement of the globe, effacement of the medial rectus muscle, portions of the fat plane, and the superior oblique muscle, and bone destruction with extension of the mass through the orbital floor into the superior maxillary sinus and through the lamina papyracea into the ethmoid sinus. Orbital biopsy disclosed dense fibrous connective tissue with numerous lymphocytes and macrophages. Immunohistochemical stains supported a diagnosis of idiopathic inflammatory pseudotumor involving the orbit and sinus mucosa. Treatment with a prednisone taper and a retrobulbar injection of triamcinolone acetonide have relieved her symptoms and diminished her proptosis. This patient highlights the rare potential of idiopathic orbital inflammation to erode though bone into adjacent cranial structures.

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.A differential diagnostic overview of IOI is presented, including its subcategories, diagnosis, and treatment.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.HINTERGRUND: Orbitale Entzündungen umfassen ein Spektrum von Erkrankungen. Die idiopathische orbitale Entzündung (IOI) ist meist eine Ausschlussdiagnose, die von Infektionen, systemischen entzündlichen Erkrankungen und Tumorerkrankungen abgegrenzt werden muss. Die IOI umfasst die anteriore Entzündung mit Dakryoadenitis, Myositis, Perineuritis des Sehnervs, Periskleritis, die diffuse sklerosierende Entzündung und die Entzündung der Orbitaspitze.Es erfolgte die differenzialdiagnostische Darstellung des Krankheitsbilds mit seinen Unterkategorien sowie mit Diagnostik und Therapieübersicht.Die Diagnose der IOI ist vielfach eine Ausschlussdiagnose mit typischen Zeichen in klinischem Befund, Computertomographie (CT) und Magnetresonanztomographie (MRT) und Histologie. Die Therapie umfasst Kortikosteroide sowie Immunmodulatoren, Immunsuppressiva und Strahlentherapie.

This study defines consensus criteria for diagnosis of idiopathic orbital inflammation using a survey of members of the Expert Panel of the Orbital Society.

To clarify the incidence, demography and clinical features of orbital tumors diagnosed in a single institute in Japan. Retrospective, observational case series. Patients with primary orbital tumors including tumor-like lesions diagnosed clinically or histopathologically at Tokyo Medical University Hospital between 1995 and 2019 were analyzed. Incidence of all orbital tumors, demographic profile and clinical features of major benign and malignant tumors were reviewed retrospectively. Totally 1000 cases of primary orbital tumor were diagnosed clinically or histopathologically during the study period. Benign tumors accounted for 72% and malignant tumors 28%. 55% of benign tumors and 99% of malignant tumors were proven histopathologically. The most common benign orbital tumor was idiopathic orbital inflammation (27%), followed by IgG4-related ophthalmic disease (17%), cavernous venous malformation (13%) and pleomorphic adenoma (9%). The most common malignant tumor was lymphoma (70%), followed by adenoid cystic carcinoma (7%) and solitary fibrous tumor (5%). Epidemiology of orbital tumors has changed by the improvement of imaging techniques, establishment of novel clinical and histopathological criteria, and changes in population age structure associated with the aging society. Currently, lymphoproliferative diseases including lymphoma and IgG4-related ophthalmic diseases form the major orbital tumors in Japan.

Gaze-evoked amaurosis is a transient monocular vision loss elicited by eccentric gaze and has been reported in many orbital conditions but is most classically associated with intraconal tumors, such as cavernous hemangioma and optic nerve sheath meningioma. Here, the authors report a case of gaze-evoked amaurosis due to idiopathic orbital inflammation. The patient was a 35-year-old man who presented with vision loss only when abducting his left eye. He had a history of sclerosing idiopathic orbital inflammation with a left orbital intraconal mass diagnosed 15 months prior to the current presentation. The patient had difficulty with immunosuppressive therapy, which was stopped 5 months prior to presentation. Repeat imaging during the current presentation revealed enlargement of the mass. This case demonstrates that idiopathic orbital inflammation should be considered in the differential diagnosis for gaze-evoked amaurosis, which may be the first symptom of disease progression.

本文报道了1例中南大学湘雅医院耳鼻咽喉头颈外科收治的眼眶颅底炎性假瘤患者。患者中年男性，以“左侧眼眶疼痛2个月，左眼视力下降1个月”入院，入院诊断为眼眶颅底肿瘤（左）。行鼻内镜下颅底病变活检术，临床表现结合影像学及病理学结果，考虑诊断为眼眶颅底炎性假瘤（左）。术后予以泼尼松口服，密切随访1年无复发。.

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.

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.

A Man with Chronic Kidney Disease A 74-year-old man presented for evaluation of his chronic kidney disease before a planned transcatheter aortic-valve replacement. Pancreatitis had been diagnosed 2...

Three patients (3 female patients; aged 7, 35, and 61 years) who had recalcitrant idiopathic sclerosing orbital inflammation were treated with rituximab. The disease was bilateral in 1 patient (4 orbits in total): diffuse in 2 and localized in 2 orbits. It caused optic neuropathy in 1 orbit of each patient. Conventional immunotherapy and tumor debulking surgery were unsuccessful in controlling the disease. After rituximab infusions (375 mg/m2/week for 4 weeks), all patients improved symptomatically. Radiologically, the local lesions resolved completely and diffuse lesions partially. Two patients with recurrent inflammation during follow up (78, 58, and 51 months) responded well to immediate, short-term steroid treatments. Short-term rituximab therapy can induce effective remissions in patients with refractory idiopathic sclerosing orbital inflammation. Early and local lesions may respond better to treatment than diffuse lesions. Nevertheless, inflammatory exacerbations can occur during late follow up.

The patient was a 70-year-old man with idiopathic orbital inflammation (IOI) that appeared on the severely affected side of preceding myasthenia gravis (MG). The patient was diagnosed with MG 5 years prior to the onset of IOI. When IOI was diagnosed, an edrophonium test was negative. IOI was considered because he complained of left orbital pain, eyelid swelling, and cerebral MRI exhibited the enhanced lesions along the left orbital periosteum. A biopsy specimen revealed pathological findings compatible with IOI. The administration of corticosteroids was effective for improving the ocular symptoms. IOI should be considered when ocular symptoms deteriorated with soft tissue swelling/pain in MG patients.

Adenocarcinoma of the esophagus is the oncologic entity with the most progressive incidence in western countries over the last 30 years. This is caused by, among other factors, a growing rate of obesity and the associated gastroesophageal reflux disease. Typical sites of metastasis include the liver, lymph nodes and peritoneum. Adrenal glands and thoraco-abdominal skeleton can also be affected. Cerebral metastasis is infrequent and there are only a handful cases described in the literature. The case presented here relates to a 61-year-old woman with osteolytic metastasis that was infiltrating the orbital cavity and was initially diagnosed as a dacryoadenitis.

Background/aims This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor. Methods We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG4 was performed. Finally, six of IgG4-related ophthalmic disease patient and 10 of orbital inf lammatory pseudotumor patient were analyzed. Results The IgG4-related ophthalmic disease group had more IgG4-positive plasma cells and a higher IgG4/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG4-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG4-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG4-related ophthalmic disease group (p = 0.035). Conclusion The location of the lesion (lacrimal gland), count and ratio of IgG4-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG4-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG4-related ophthalmic disease to prevent recurrence.

To describe a case series of patients investigated in internal medicine for orbital inflammation (OI) since the individualization of the clinical entity of the IgG4-related orbital disease (IgG4 ROD).Thirty four patients were consecutively referred by a specialized center where orbital biopsy was performed when the lesion was surgically accessible. Fourteen patients were excluded in case of missing data or lymphoma, periocular xanthogranuloma or Graves' orbitopathy. Patients with systemic or auto-immune disease according to the international criteria, or presenting with idiopathic orbital inflammation syndrome (IOIS), were included. Knowing the histological similarities between IOIS and IgG4 ROD, immunohistochemical assessment of plasma cells for IgG4 positivity was performed for every patient with available biopsy. Clinical and biological characteristics, treatment and response to treatment of included patients are reported.Among 22 included patients, 10 presented with orbital manifestation of a systemic or autoimmune disease including 2 sarcoidosis (9%) and 8 (36%) cases of non specific OI which were reclassified in IgG4 ROD. Finally, IOIS of various clinicopathological presentation was diagnosed for 12 patients including 10 with histological documentation. Whereas relapse and resistance were not found to be related to IgG4 positivity (50% in both IOIS and IgG4 ROD groups), another treatment in addition to corticosteroids was more often necessary in IgG4 ROD patients (50%) than in IOIS patients (25%).After ruling out auto-immune orbital diseases, especially IgG4 ROD, IOIS should be discussed. Factors conditioning the corticosteroid response are yet to be determined.

Idiopathic orbital inflammation (IOI) is a benign inflammatory disease usually confined to the orbit. Two middle-aged patients with IOI have been reported who could have been misdiagnosed as involutional ptosis. Two patients presented to the clinic with eyelid ptosis. One patient's erythrocyte sedimentation rate was slightly increased; the other patient's immunoglobulin G4 (IgG4), IgG, and thyroid-stimulating hormone results were higher than the upper limits of normal. The computed tomographic images and orbit magnetic resonance imaging tests showed an inflammatory condition around the droopy eyelids. They were both admitted to the hospital with diagnosis of IOI, and intravenous antibiotic and steroid treatment were administered, which improved their symptoms.

The field of idiopathic orbital inflammation has come into motion: Ever since the first description of IgG4-associated disease about 10 years ago, the group of idiopathic lesions has shrunk by about half. The differential diagnosis has become much more demanding because the clinical and imaging overlap is considerable. This article presents a review of idiopathic orbital inflammation, IgG4-associated disease, and orbital lymphoid hyperplasia. All three conditions can have significant - and quite amazing - clinical and imaging similarities. At the same time, however, the necessity of diagnostic assessment differ just as much as the optimal therapy or therapy duration.In das Feld der idiopathischen orbitalen Entzündung ist „Bewegung“ gekommen: Mit der Erstbeschreibung der IgG4-assoziierten Erkrankung vor etwa 10 Jahren ist die Gruppe der idiopathischen Läsionen um etwa die Hälfte geschrumpft. Die Differenzialdiagnose ist deutlich anspruchsvoller geworden, weil die klinische und bildgebende Überlappung dieser Krankheitsbilder erheblich ist. Der vorliegende Artikel gibt einen Überblick über dieses im Umbruch befindliche Feld.

International audience; PURPOSE:To better characterize IgG4-related disease (RD) in the setting of idiopathic orbital inflammation syndrome (IOIS).METHODS:National, multicentre, prospective, observational cohort study. Among the patients consecutively included in the French multicentre SIOI cohort, we selected those who underwent orbital and/or adnexal biopsy. Clinical, morphological and pathological findings at diagnosis were blindly analysed. Serum IgG4 levels at inclusion were measured and all available biopsy specimens were immunostained for IgG4 and IgG. Biopsy samples with more than 10 IgG4-positive plasma cells per high-power field and a IgG4+/IgG+ plasma cell ratio above 40% were scored as positive. IgG4-positive patients were then screened for comprehensive diagnostic criteria for IgG4-RD.RESULTS:Of the 87 patients included, 35 had histologically documented IOIS. Thirteen patients (37%) with a mean age at onset of 27 years (range 21-78) had IgG4-positive biopsies, among which 10 patients (77%) and 3 (23%, with IgG4 serum levels >1.35 g/L) were considered as having probable and definite IgG4-RD, respectively. The latter 13 patients more frequently fulfilled histological criteria for IgG4-RD (including plasmacytic infiltrate (p = 0.006), fibrosis (p = 0.0025) and periphlebitis (p = 0.075)) than IgG4-negative patients. Storiform fibrosis was exclusively found in orbital tissues from IgG4-positive patients (n = 3, 23%). Eosinophilia associated with recurrent sinusitis or asthma was a prominent feature in patients with definite IgG4-RD.CONCLUSIONS:More than one-third of patients with biopsy-proven IOIS satisfied criteria for IgG4-RD, but only a few had a definite type.

To evaluate outcomes after definitive radiotherapy for orbital pseudotumor.We reviewed adult patients treated between 1971 and 2012.20 patients with 8.0-year median follow-up were analyzed. Symptoms improved in 75% of patients: 40% showed a complete response, 35% partial response, and 20% had stable disease. The disease progressed in 1 and recurred in 8 patients. Local control rates were 63% and 53% at 5 and 10 years, respectively. The most common toxicities were cataracts (10%) and dry eye (10%).Radiotherapy for orbital pseudotumor is well-tolerated and results in long-term control in most patients, with local control in over half.

This systematic review of biopsy proven cases of non specific orbital inflammation and sclerosing variant from 2010-2016 attempts to elucidate new findings regarding the demographic profile of the diseases. Discussion of histopathology is emphasized, as to describe the most common presenting features of non-specific orbital inflammation as well as to discuss its molecular make-up . This review also discusses the therapeutic approach and discusses the “steroids vs. biopsy first” controversy regarding non-specific orbital inflammation. The incidence of IgG4 and its relationship to non-specific orbital inflammation is also discussed.

Key Clinical Message Orbital inflammatory pseudotumor is an infrequent lesion that may mimic malignancy and may be associated with autoimmune disease. Orbital pseudotumor is in the differential in any patient with an orbital mass, especially in the setting of chronic infection or immunosuppression. This HIV‐positive patient presented with chronic sinusitis and orbital pseudotumor.

A retrospective single-center chart review identified 19 patients treated with intralesional corticosteroid injection between 2007 and 2015 for orbital inflammatory disease. Patients were treated in the office, or intraoperatively at the time of orbital exploration and biopsy, with 5-40 mg of triamcinolone acetonide. Average follow up time was 2.44 years. Information recorded included visual acuity and eyelid position measurements, pre-injection treatment, post-injection treatment, complications, and exam findings. Nine of the identified patients received intraoperative injections, 9 in the office setting, and one received an injection in each. Eight patients (42.1%) were on oral corticosteroids prior to the injection. All patients showed initial clinical improvement after 1-2 injections and no more than a 1-2 line difference in visual acuity. Overall, six patients (31.6%) required repeat injections due to recurrence of inflammation, and seven (36.8%) required additional oral corticosteroids beyond the initial taper. Patients pre-treated with oral corticosteroids were more likely to need repeat injections (83.3% vs 16.7% respectively, p = 0.04). Compared to injections performed in the office setting, intraoperative injections were associated with fewer repeat injections (0% vs. 60.0% respectively, p = 0.01) and less need for post-injection oral steroids (0% vs. 58.3% respectively, p < 0.01). There were no systemic or permanent side effects observed from the injections. Two patients (10.5%) had transient post-injection intraocular pressure elevation, which resolved. Intralesional orbital corticosteroid injection is an effective first-line and adjunctive treatment for localized and diffuse non-infectious orbital inflammation.