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Purpose: To describe four cases of non-infectious hypertrophic pachymeningitis (HP)-associated with orbital inflammatory disease (OID). This study summarises the clinico-radiological features, outcomes, and management of HP-associated OID., Methods: Retrospective case-series of patients with radiological evidence of HP and OID. Comprehensive literature review of all published English-language non-infectious causes of HP-associated OID. Reference lists were screened for inclusion of relevant articles., Results: Thirty-seven cases of HP-associated OID (Mean age: 49.2 ± 17.4 years old; Male: 15) were identified, including four cases from our institution. Aetiologies included ANCA-associated vasculitis (12/37), non-specific/idiopathic (11/37), IgG4/multifocal fibrosclerosis (11/37), neurosarcoidosis (1/37), inflammatory myofibroblastic tumour (1/37), and giant cell arteritis (1/37). Orbital pain, headache, visual deterioration, and cranial nerve palsies were common clinical presentations. Both "focal" and "diffuse" HP were observed, with the most common sign of orbital involvement being an inflammatory orbital mass, typically with orbital apex involvement. Orbital myositis and dacryoadenitis were less common. The cavernous sinus was the most common site of extra-orbital inflammation. There was no single differentiating specific radiological feature between non-specific and specific forms of HP-associated OID., Conclusion: The clinico-radiological manifestations of HP-associated OID differ from those described in isolated HP or OID. There is no single specific radiological marker differentiating non-specific/idiopathic disease from secondary causes; however, the co-existence of HP in OID should prompt suspicion of an underlying cause. The disease may be refractory or resistant to initial treatment, although guidelines surrounding its management and the long-term prognosis remain to be determined.

Abstract: Point-of-care ultrasound was used in a child presenting to the emergency department with progressively worsening left eyelid swelling. The early use of ultrasound was helpful in detecting a soft tissue mass that is exhibiting pressure effect on the eyeball. Point-of-care ultrasound helped expedite advanced imaging and consultation with the specialist. The patient eventually had a biopsy and was diagnosed with idiopathic orbital inflammation., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

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.)

Orbital inflammatory pseudotumor (OIP) is a kind of orbital idiopathic benign space-occupying lesion with no clear etiology and mainly characterized by inflammatory response. The clinical diagnosis of OIP should be based on exclusion of other diseases or surgical biopsy. Medication and surgical excision are both diagnostic and therapeutic methods. The choice of which is a problem that baffles clinicians. This article discusses a new strategy basing on the imaging features and classifications of OIP. It is expected to be further discussed and promoted in clinical practice to improve the diagnosis and treatment of OIP.

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.

A 57-year old woman presented with a 6-month history of a left red eye associated with diplopia. Examination was remarkable for 2 mm left-sided axial proptosis and restriction on left upgaze and abduction associated with diplopia. She had no previous history of trauma or surgery to the face. Magnetic resonance imaging revealed an enhancing infiltrative mass in the left intraconal space. Biopsy of the anterior orbital fat revealed extensive lipogranulomatous inflammation with no abnormalities noted on flow cytometry, culture, or special stains. The patient was managed with a tapering course of oral prednisolone and two 40 mg intraorbital triamcinolone injections resulting in complete resolution of her presenting symptoms. This represents a rare case of idiopathic lipogranulomatous orbital inflammation.

Purpose: To investigate the utility of intravoxel incoherent motion diffusion-weighted MRI (IVIM-DWI) derived diffusion and perfusion parameters in differentiating ocular adnexal lymphoma (OAL) from idiopathic orbital inflammation (IOI), and to assess whether IVIM-DWI provides improved diagnostic performance for the distinction., Method: Twenty-one patients with OAL and 24 patients with IOI underwent IVIM-DWI. Apparent diffusion coefficient (ADC) and IVIM-DWI parameters including true diffusion coefficient (D), pseudo-diffusion coefficient (D*), and perfusion fraction (f) were measured in lesions by two independent radiologists. The MRI parameter differences between OAL and IOI were tested using two-sample t-test. The receiver operating characteristic (ROC) analysis curves were used to determine the diagnostic performance of significant parameters for differentiation between OAL and IOI., Results: The ADC, D, and f were lower in OAL than those in IOI (ADC = 0.78 ± 0.12 vs. 0.99 ± 0.16 × 10 -3 mm 2 /s, P < 0.001; D = 0.34 ± 0.15 vs. 0.76 ± 0.25 × 10 -3 mm 2 /s, P < 0.001; f = 0.31 ± 0.06 vs. 0.41 ± 0.08 × 100 %, P < 0.001). There was no significant difference in D* between OAL and IOI (P = 0.235). The optimal cut-off values of ADC, D, and f in differentiating OAL from IOI were 0.83 × 10 -3 mm 2 /s, 0.56 × 10 -3 mm 2 /s, and 0.36 × 100 %, respectively. No significant differences were found in areas under the curve (AUCs) among ADC, D and f (all P > 0.05). The combination of D and f provided significantly higher AUC than ADC (AUC = 0.984 vs. 0.838, Z = 2.128, P = 0.033), and had higher sensitivity of 95.24 %, specificity of 95.83 %, and accuracy of 95.56 %., Conclusions: IVIM-DWI is valuable in differentiating OAL from IOI, and D combined f can improve the performance of differential diagnosis., (Copyright © 2020 Elsevier B.V. All rights reserved.)

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.

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.

Orbital pseudotumor is a rare, idiopathic, inflammatory process within the orbit. Early diagnosis is essential to prevent corneal, retinal and extraocular muscle damage. This article describes the presentation and clinical course of a 3-year-old male with bilateral orbital pseudotumor.

Background: The overlap of morphological feature and mean ADC value restricted clinical application of MRI in the differential diagnosis of orbital lymphoma and idiopathic orbital inflammatory pseudotumor (IOIP). In this paper, we aimed to retrospectively evaluate the combined diagnostic value of conventional magnetic resonance imaging (MRI) and whole-tumor histogram analysis of apparent diffusion coefficient (ADC) maps in the differentiation of the two lesions., Methods: In total, 18 patients with orbital lymphoma and 22 patients with IOIP were included, who underwent both conventional MRI and diffusion weighted imaging before treatment. Conventional MRI features and histogram parameters derived from ADC maps, including mean ADC (ADC mean ), median ADC (ADC median ), skewness, kurtosis, 10th, 25th, 75th and 90th percentiles of ADC (ADC 10 , ADC 25 , ADC 75 , ADC 90 ) were evaluated and compared between orbital lymphoma and IOIP. Multivariate logistic regression analysis was used to identify the most valuable variables for discriminating. Differential model was built upon the selected variables and receiver operating characteristic (ROC) analysis was also performed to determine the differential ability of the model., Results: Multivariate logistic regression showed ADC 10 (P = 0.023) and involvement of orbit preseptal space (P = 0.029) were the most promising indexes in the discrimination of orbital lymphoma and IOIP. The logistic model defined by ADC 10 and involvement of orbit preseptal space was built, which achieved an AUC of 0.939, with sensitivity of 77.30% and specificity of 94.40%., Conclusions: Conventional MRI feature of involvement of orbit preseptal space and ADC histogram parameter of ADC 10 are valuable in differential diagnosis of orbital lymphoma and IOIP.

Background: Idiopathic orbital inflammatory syndrome (IOIS) is an inflammatory condition of unknown etiology. The inflammation may affect all the structures within the orbit (anterior, diffuse, apical, myositic, dacryoadenitis) and corresponds to uniquely orbital inflammation without an identifiable local cause or systemic disease. The goal of this study is to describe the clinical and radiographic characteristics of IOIS and discuss the role of orbital biopsy in this condition., Patients and Methods: This is a retrospective review of the charts of 24 patients diagnosed with IOIS at Fattouma Bourguiba hospital, Monastir, Tunisia, from January 2007 to December 2015. This study included all patients with IOIS and a minimum follow-up of six months. All patients had a complete ophthalmological examination and orbital and head CT scan and/or MRI. A work-up was performed in all cases to rule out local causes and systemic disease. Only 11 patients underwent biopsy. The diagnosis of the clinical entity IOIS was made according to the Rootman criteria. Oral steroids were the first line therapy. A bolus of intravenous methylprednisolone was administered first in vision-threatening cases. Response to treatment was defined as disappearance of signs and symptoms of IOIS., Results: Orbital pain was the most common symptom (62.5%), followed by proptosis and decreased vision (37.5% each). Best-corrected visual acuity (BCVA) was greater than 5/10 in 70.7% of patients. Lacrimal gland enlargement was observed in 3 patients. Oculomotor disorders were present in 70% of cases and 20.8% of patients had compressive optic neuropathy. Orbital imaging showed, in most cases, oculomotor muscle inflammation (87.5%) involving particularly the superior rectus muscle (54.2%) and inflammation of orbital fat (66.7%). Fifty percent had myositic inflammation. Biopsy was performed in 11 patients, showing nonspecific inflammation (n=10) and the sclerosing form (n=1). A total of 83.3% of patients received oral corticosteroids for a mean duration of 5.5 months., Conclusion: IOIS is a diagnosis of exclusion, based on history, clinical course, response to steroid therapy, laboratory tests, or even biopsy in selected cases. Orbital imaging provides valuable clues for diagnosis of IOIS and for identification of affected structures. Prolonged steroid therapy is necessary as IOIS classically responds to steroids; nevertheless, partial recovery or relapses often occur., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemia.

Inflammatory myofibroblastic tumor is a rare entity characterized by the presence of myofibroblasts and inflammatory cells within a fibrous stroma. It typically occurs in the pediatric population. The most common site of occurrence is the lung though it has been reported throughout the body. Although rare, it has been reported in the orbit. The clinical course is ill defined in the literature; here we report a case of pediatric IMT with delayed spontaneous regression.

Inflammatory pseudotumor (IP) is an uncommon idiopathic lesion that often imitates malignancy clinically and radiologically. Inflammatory pseudotumors have been found to occur in various sites but rarely in the head and neck. The histopathology, imaging, and treatment of three unique cases of head and neck inflammatory pseudotumors are described in this case series. Patients in Cases 1 and 2 presented with right level II neck mass and left parotid tail mass, respectively. The patient in Case 3 presented with otalgia, jaw pain and trismus, and a left parapharyngeal space mass. The tumors in Cases 1 and 3 significantly decreased in size with tapered courses of oral corticosteroids. The tumor in Case 2 was surgically excised without disease recurrence. Malignancy must be ruled out with incisional or excisional biopsy. Treatment includes surgical excision, oral corticosteroids, or both. The literature shows that radiotherapy and small-molecule inhibitors may be promising alternatives., (© The Author(s) 2016.)

Objective: To analyze overlaps between pregnancy and orbital inflammation (OI)., Design: Retrospective observational case series., Methods: Eight new cases from 1997 to 2015 and 2 previously published cases were identified for inclusion in this investigation to provide the fullest clinical picture. Medical records, imaging studies, and the results of biopsies were reviewed., Results: Three categories of association were discovered: (1) OI arising for the first time during pregnancy (5 cases); (2) OI arising within 3 months of delivery (2 cases); and (3) previously diagnosed OI reactivated or exacerbated by pregnancy (3 cases). One patient had a preexistent systemic autoimmune disease and another's was later diagnosed. One patient had attacks during sequential pregnancies. Findings included eyelid swelling and erythema, conjunctival chemosis, pain on eye movement, minimal diplopia, the usual absence of proptosis, and general preservation of visual acuity. Imaging studies disclosed extraocular muscle swelling (8 cases), most frequently of a single lateral rectus muscle. There were 2 cases of dacryoadenitis; 1 of these and an additional case displayed inflammation of the retrobulbar fat. Corticosteroids effected resolution of most symptoms. Singleton births were normal with the exceptions of an intrauterine fetal demise owing to acrania and a molar pregnancy., Conclusion: OI usually affects a single rectus muscle (typically the lateral) and, less often, the lacrimal gland and is often mild when it arises during or after pregnancy. Independent systemic autoimmune disease is an uncommon feature. Corticosteroids were efficacious except in 1 case with severe orbital scarring. No definitive causal relationships between pregnancy and OI could be established based on the clinical data., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Inflammatory orbital pseudotumor is often associated with rheumatologic disorders. It has been reported commonly with ANCA-associated vasculitides, especially granulomatosis with polyangiitis (Wegener's granulomatosis). There are also a few cases of large vessel vasculitis such as giant cell arteritis and Behcet's disease. Here, we report a patient with undiagnosed Takayasu arteritis presenting with proptosis and diplopia, with later diagnosis of an inflammatory pseudotumor of the orbit. In this case, we believe extensive involvement of blood vessels, including bilateral pulmonary artery stenosis, and elevated inflammatory markers that show disease activity may be related to pseudotumor formation in Takayasu arteritis. Since this is an unusual and unreported presentation of the disease, better estimation of a causal relationship may be possible in the future with further information. In conclusion, although uncommon, this case highlights that orbital pseudotumor may be an important finding in Takayasu arteritis. For early diagnosis, better treatment, and good prognosis, it should be considered in patients presenting with ocular symptoms similar to the other vasculitides.

Purpose: We aimed to evaluate the role of histogram analysis of apparent diffusion coefficient (ADC) maps for differentiating benign and malignant orbital tumors., Methods: Fifty-two patients with orbital tumors were enrolled from March 2013 to November 2014. Pretreatment diffusion-weighted imaging was performed on a 3T magnetic resonance scanner with b factors of 0 and 800 s/mm2, and the corresponding ADC maps were generated. Whole-tumor regions of interest were drawn on all slices of the ADC maps to obtain histogram parameters, including ADCmean, ADCmedian, standard deviation (SD), skewness, kurtosis, quartile, ADC10, ADC25, ADC75, and ADC90. Histogram parameter differences between benign and malignant orbital tumors were compared. The diagnostic value of each significant parameter in predicting malignant tumors was established., Results: Age, ADCmean, ADCmedian, quartile, kurtosis, ADC10, ADC25, ADC75, and ADC90 parameters were significantly different between benign and malignant orbital tumor groups, while gender, location, SD, and skewness were not significantly different. The best diagnostic performance in predicting malignant orbital tumors was achieved at the threshold of ADC10=0.990 (AUC, 0.997; sensitivity, 96.2%; specificity, 100%)., Conclusion: Histogram analysis of ADC maps holds promise for differentiating benign and malignant orbital tumors. ADC10 has the potential to be the most significant parameter for predicting malignant orbital tumors.

Rationale and Objectives: The aim of this study was to evaluate the added value of histogram analysis of apparent diffusion coefficient (ADC) maps in differentiating indeterminate orbital malignant tumors from benign tumors, compared to using magnetic resonance (MR) morphological features alone., Materials and Methods: We retrospectively evaluated 54 patients with orbital tumors from March 2013 to February 2015. All the patients were assessed by both routine MR and diffusion-weighted imaging, and divided into benign group and malignant group. Routine MR imaging features and histogram parameters derived from ADC maps, including mean ADC (ADCmean), median ADC (ADCmedian), standard deviation, skewness, kurtosis, and 10th and 90th percentiles of ADC (ADC10 and ADC90), were compared between two groups. Univariate and multivariate logistic regression analyses were used to identify the most valuable variables in predicting malignancy. Receiver operating characteristic (ROC) curve analysis was used to determine the diagnostic value of significant variables., Results: Multivariate logistic regression analysis indicated that two or more quadrants involved, iso-intense on T2-weighted imaging (T2WI), and ADC10 were significant predictors for orbital malignancy. By using model 2 (iso-intense on T2WI + two or more quadrants involved + ADC10 < 0.990) as the criterion, higher AUC and specificity could be achieved than by using model 1 (iso-intense on T2WI + two or more quadrants involved) alone, (model 2 vs model 1; area under curve (AUC), 0.827 vs 0.793; sensitivity, 65.4% vs 69.2%; specificity, 100% vs 89.3%)., Conclusions: Iso-intense on T2WI, two or more quadrants involved, and ADC10 are risk factors for orbital malignancy. Histogram analysis of ADC map might provide added value in predicting orbital malignancy., (Copyright © 2015 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Introduction: Pseudo-tumoral fongal rhinosinusitis is a rare and benign illness due to saprophyte germ, Aspergillus flavus., Observation: We reported two cases of invasive pseudo-tumoral fongal rhinosinusitis. CT scan helped with diagnosis and allowed for extension assessment. Complete surgical excision was done through external approach. Bacteriological examination evidenced the germ., Discussion: Pseudo-tumoral invasive fongal rhinosinusitis remains a potentially serious pathology because of its local aggressiveness and its multiple extensions., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Sarcoidosis is a systemic multiorgan disorder of unknown etiology characterized by a non-caseating granuloma reaction. Ocular involvement has been reported in 25-60% of the patients. Seven percent of the patients with sarcoidosis may first see an ophthalmologist due to ocular complaints. This report aims to present our diagnostic and treatment approach to a female patient with significant unilateral lacrimal gland swelling and musculoskeletal involvement, who was diagnosed with sarcoidosis on the basis of tests and histological studies.

Purpose: Coexistence of idiopathic orbital inflammatory pseudotumor (IOIP) and thyroid-associated ophthalmopathy (TAO) is extremely rare. The purpose of this article is to analyze the clinical features, image findings, and therapeutic outcomes of concomitant IOIP and TAO in China., Materials and Methods: Detailed clinical records of 3 Chinese patients with concomitant IOIP and TAO were reviewed, including their clinical history, symptoms and signs, ultrasonography, computed tomography (CT), and steroid therapy., Results: Among the 3 patients, were 2 men and 1 woman, aged 42, 49, and 48 years, respectively. The right orbit was involved in 1 patient and both orbits in 2 patients. In addition to showing the typical features of TAO, such as hyperthyroidism, upper eyelid retraction, and enlarged extraocular muscles with tendon sparing, all 3 patients showed ambiguous soft tissue masses in one or both orbits. Pathologic examination after biopsy of the mass in 1 patient confirmed the diagnosis of lymphatic IOIP. All the patients responded extremely well to steroid treatment., Conclusions: Although rare, a simultaneous coexistence of IOIP and TAO can occur. Therefore, it is important for clinicians to be aware of the potential for concomitant IOIP and TAO.

Purpose: To review the clinical efficacy and safety of rituximab for treatment of IgG4-related orbital disease (IgG4-ROD)., Design: Retrospective multicentre interventional case series., Methods: Chart review for five cases of biopsy-confirmed IgG4-ROD (IgG4+>10/HPF, ratio of IgG4+/IgG+>40%) treated with rituximab. Information retrieved included the dosing schedule, adverse events and the magnitude, temporality, and duration of the clinical response., Results: All cases of IgG4-ROD were either steroid dependent or steroid resistant. Rituximab doses for induction therapy included two doses of 1000 mg at 2-weekly intervals, and four doses at 375 mg/m(2) at weekly intervals. Two months after starting rituximab, three cases achieved complete clinical resolution and two cases achieved partial clinical resolution. Complete radiological resolution occurred in one case, and partial radiological resolution in three cases. Three cases received rituximab maintenance therapy and one case was commenced on mycophenolate. No relapse occurred during a mean follow-up of 33 months (range: 7-65 months). One disease relapse occurred when the dosing interval of rituximab maintenance therapy was extended to 6-monthly intervals; remission was swiftly achieved with rituximab reinduction therapy. The only adverse effects reported were one episode of fatigue lasting 1 week and two episodes of orbital discomfort., Conclusion: Rituximab may be an effective treatment option for IgG4-ROD that is steroid dependent or steroid intolerant. Rituximab therapy resulted in swift clinical and radiological improvement, many months free of relapse, and few side effects.

Purpose: To report the case of an atypical sino-orbital inflammatory myofibroblastic tumor., Methods: Case report., Results: A 71-year-old man presented slowly progressive painless diplopia followed by unilateral proptosis of the left eye with slight edema of the upper eyelid. Visual acuity was 0.4 with a relative afferent pupillary defect (RAPD). Abduction and depression of the left eye were limited. Imaging showed a left sino-orbital tumor with mass effect on the medial rectus muscle resulting in proptosis. The lesion showed intracranial extension through the superior orbital fissure with bone erosion and sclerotic bone reaction. Endoscopic ethmoidal, maxillary, and orbital biopsies revealed an inflammatory myofibroblastic tumor. Full blood count showed an eosinophilia while antineutrophil cytoplasmic antibodies and C-reactive protein were negative. To date, 7 cases of orbital myofibroblastic tumor have been described, though none of the prior studies have described bone invasion., Conclusions: This erosive sino-orbital inflammatory myofibroblastic tumor is a novel finding mimicking a malignant tumor.

The purpose of this report is to describe the clinical, radiographica, and histopathologic findings in two pediatric patients with orbital IgG4-related disease.

Purpose: To determine whether serologic factors correlate with early relapse in cases of IgG4-related orbital inflammation, a lymphoproliferative disorder., Design: Retrospective cohort study., Methods: We examined the orbital images and serum data of 30 patients with (n=24) or without (n=6) corticosteroid treatment before and after corticosteroid treatment and 6 months after discontinuation of corticosteroid treatment., Results: Seven patients (23%) (all with corticosteroid treatment) had elevated serum levels of rheumatoid factor. Of the 24 patients with corticosteroid treatment, 24 (100%) showed regression of the lesion, but 8 of the 24 (33%) showed relapse. The serum IgG4 levels before and after corticosteroid treatment were not significantly different between the relapsed and nonrelapsed groups (P=.5 and P=.2, respectively). However, the incidence of patients who were rheumatoid factor-positive was significantly higher in the relapsed group (P=.02). The 6 patients without corticosteroid treatment showed minor proliferation or regression of the lesion at the 6-month follow-up., Conclusions: The serum rheumatoid factor level may correlate with proliferative activity in IgG4-related orbital inflammation., (Copyright © 2013 Elsevier Inc. All rights reserved.)

An observational study was conducted to demonstrate the role of Computed Tomographic (CT) scan to detect clinically suspected adult orbital mass in 47 patients which could not be differentiated clinically. The CT findings were compared and correlated with the findings of fine needle aspiration cytology (FNAC) or histopathology. CT diagnosis of optic nerve sheath meningioma were 12 cases, among them only nine cases confirmed cytopathologically as meningioma and rest three as lymphoma. Among ten cases of hemangioma, eight cases were confirmed cytopathologically as cavernous hemangioma and rest two were pseudotumor and chronic inflammatory lesion. Seven cases diagnosed as pseudotumor in CT were confirmed cytopathologically. Seven cases diagnosed as paranasal sinus masses with orbital extension (nasopharyngeal angiofibroma) in CT were confirmed cytopathologically. Among three cases of thyroid ophthalmopathy diagnosed in CT, only two cases confirmed cytopathologically and rest one cytopathologic diagnosis was not possible due to inadequate tissue supply during FNAC. Two cases of chronic inflammatory lesion diagnosed in CT, also confirmed cytopathologically. Two cases of metastatic lesion diagnosed in CT, also confirmed cytopathologically. Two cases of lacrimal gland tumor diagnosed in CT, also confirmed cytopathologically i.e., pleomorphic adenoma. Two cases of melanoma diagnosed in CT, only one confirmed cytopathologically and rest one cytopathologic diagnosis was not possible due to frank blood came out during FNAC. Pseudotumors were subsequently diagnosed the non-diagnostic cases on the basis of clinical and radiological findings. It is evident from these findings that CT is a useful modality in the diagnosis of adult orbital masses.

Objective: To report an atypical case of orbital inflammatory pseudotumor associated with optic neuritis revealing Behçet's disease., Case Report: A 37-year-old man was worked up for headache and progressive decreased bilateral visual acuity most profound in the left eye. Initial ophthalmologic exam was without abnormal findings. Orbital CT scan and MRI appeared consistent with orbital inflammatory pseudotumor involving the left lateral rectus muscle (orbital myositis). The patient was treated with oral corticosteroids, but clinical response was incomplete. Follow-up ophthalmologic exam revealed acute inflammatory right optic neuritis (visual acuity: 5/10, papillitis on fundus exam and papillary edema on fluorescein angiography) and persistence of left orbital myositis. The diagnosis of Behçet's disease with ocular complications was made on the basis of recurrent oral ulcers, scars from genital ulcerations and pseudofolliculitis. It was decided to treat the patient with a bolus of IV methylprednisolone for 3 consecutive days, followed by oral prednisolone and cochicine, leading to stabilization of the ocular manifestations., Conclusion: To our knowledge, this is the first report of such an association leading to a diagnosis of Behçet's disease. The coexistence of these two manifestations as well as the contralateral nature of the involvement speaks to the pathogenic mechanism of these complications (systemic vasculitis)., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Objectives: To describe the clinical, laboratory, histopathological presentations and final diagnoses for children presenting to a tertiary paediatric rheumatology service with an inflammatory lesion of the orbit., Methods: This was a retrospective descriptive case series of children with an inflammatory lesion of the orbit presenting to a single paediatric rheumatology service between January 1999 and July 2010., Results: Ten patients, median age 11.5 (range 3.1-16.2) years at referral to the paediatric rheumatology department were identified; median duration of symptoms at referral was 9 (0.75-17) months. Imaging was performed in 9/10 cases: orbital MRI (n = 4), orbital CT scan (n = 1), both MRI and CT scan (n = 4). All 10 patients had an orbital biopsy; 2 patients had repeat biopsies. The final diagnoses were granulomatosis with polyangiitis (Wegener's) (n = 5; ANCA positive n = 4, ANCA negative n = 1), idiopathic orbital inflammation (n = 3), atypical mycobacterial infection (n = 1) and sarcoidosis (n = 1)., Conclusion: Inflammatory mass lesion of the orbit is an unusual presentation in children. The differential diagnosis is wide and may evolve over time. Orbital biopsy and screening for systemic features is essential before treatment with CSs or other immunosuppressants to exclude malignancy, infection, vascular lesions, autoimmune conditions or other causes of orbital inflammation that can be associated with serious systemic manifestations.

Idiopathic orbital pseudotumour (IOP) is a benign inflammatory condition usually confined to the orbit. This may involve single or multiple intraorbital structures. Extraorbital extension can also occur. The imaging appearances often mimic other orbital diseases. Both computed tomography (CT) and magnetic resonance imaging (MRI) are frequently used to investigate orbital diseases, and it is important for radiologists to be aware of the variety of imaging appearances that occur in IOP. We present the imaging appearances in histopathologically confirmed cases of IOP and discuss the clinical features, natural history, and differential diagnosis of this condition., (Copyright © 2011 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Objective: To describe clinical, radiographic, and morphologic findings in patients with IgG4-positive cells present on orbital biopsy specimens., Design: Retrospective review (from January 1, 1993, through December 31, 2006) of patients with orbital biopsy specimens that excluded lymphoma; comparison of patients with and without IgG4-positive cells on immunostaining., Results: Of 21 patients, 11 had increased IgG4-positive cells (defined as >10 cells on biopsy). Symptoms included eyelid or periocular swelling (8 patients) or proptosis (3 patients), with bilateral involvement in 6 patients. Computed tomographic imaging displayed lacrimal gland mass in 10 patients; 6 patients had lesions in other organs. Two patients had increased serum IgG4 levels. In 10 patients without IgG4-positive cells (≤10 cells on biopsy), 6 had proptosis, 1 had eyelid swelling, 2 had eyelid mass, and 1 had diplopia, all unilateral. None had systemic symptoms. Patients with IgG4-positive cells had longer symptom duration, and their biopsy specimens showed more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils., Conclusions: The clinical appearance, high incidence of bilateral disease, association with lesions in other organs, and increased IgG4 serum levels in some patients-with an increased number of IgG4-positive cells in the biopsy specimen, which shows more background fibrosis, lymphoid hyperplasia, plasma cells, and eosinophils-indicate that these patients have an orbital manifestation of IgG4-associated systemic disease.

An 8-year-old boy developed vision loss to no light perception on the left side over a 2-day period. He initially presented with unilateral eyelid swelling, which progressed to bilateral edema and an eventual left-sided orbital apex syndrome. Orbital imaging revealed enlarged extraocular muscles, and biopsy confirmed idiopathic orbital inflammation. Despite subsequent orbital decompression, high-dose steroids, and additional steroid-sparing therapy, he did not regain vision after 9 months of follow up.

We present a case of idiopathic orbital inflammatory disease that was responded favorably to low-dose cyclosporine-A.

Retrobulbar granuloma is one of the serious complications in Wegener's granulomatosis and often shows resistance to conventional therapy during long-term treatment. The outcome of this complication includes visual loss, orbital and facial deformity, fistula formation, as well as infection. There has been increasing evidence that shows the efficacy of rituximab, a chimeric anti-B cell mAb, for the treatment of autoimmune diseases including Wegener's granulomatosis. We present a 22-year-old Japanese woman who was diagnosed with Wegener's granulomatosis complicated by refractory retrobulbar granuloma. She was admitted to our hospital with pain of the right eye and right proptosis during treatment with monthly IVCY for Wegener's granulomatosis. We diagnosed refractory retrobulbar granuloma by computed tomography (CT) scan and biopsy. She showed a refractory growth of retrobulbar granuloma in spite of negative ANCA. She was also complicated with pulmonary granulomatous lesions in bilateral apices. After approval by an institutional ethical committee and informed consent of this patient, rituximab 375 mg/m2 was intravenously administered weekly four times. Concomitant prednisolone 0.5 mg/kg was also administered for 2 weeks and gradually tapered. Treatment of rituximab resulted in prompt relief of symptoms in this case and the reduction of the granuloma. BVAS score also improved from 6 to 0 at 3 months and was kept in remission for 12 months. Circulating CD19-positive cells were kept less than 0.1% during the follow-up. There were no serious adverse events. This case suggests that rituximab is effective for refractory retrobulbar granuloma complicated in Wegener's granulomatosis even when ANCA titers are negative.

The authors report 2 patients with idiopathic sclerosing inflammation of the orbit who presented with periorbital paresthesia in the trigeminal nerve distribution. The diagnosis in both cases was confirmed with biopsy and both patients responded to corticosteroid treatment. Although periorbital paresthesia is usually a sign of malignancy, these cases illustrate that it may also occur in patients with sclerosing orbital inflammation.

Purpose: To report a patient with atypical idiopathic orbital inflammation who was successfully treated with intra-orbital injections of triamcinolone acetonide., Methods: Interventional case report and literature review., Main Outcome Measures: Resolution of orbital inflammation., Results: A 71-year-old female with right orbital mass and conjunctival inflammation underwent orbital biopsy that was consistent with the diagnosis of atypical idiopathic orbital inflammation. Inflammation has deteriorated despite oral steroids. The patient underwent two additional orbital biopsies that confirmed the initial diagnosis. Intra-orbital injections of triamcinolone acetonide resulted in significant improvement and cessation of active inflammation., Conclusions: Intra-orbital injections of triamcinolone acetonide may be an effective alternative in patients with atypical idiopathic orbital inflammation unresponsive to systemic steroids.

A 56-year-old black woman presented with bilateral orbital swelling in the lacrimal gland region 4 months after initiation of interferon-alpha and ribavirin treatment for occupationally-acquired hepatitis C infection. Histopathologic evaluation of bilateral lacrimal gland biopsy specimens revealed granulomatous inflammation. All other tests were negative for sarcoidosis including angiotensin converting enzyme level and chest x-ray. The use of interferon-alpha as an antiviral and/or chemotherapeutic agent is increasing. Therefore recognition of its possible orbital side effects is important for the oculoplastic surgeon. To the authors' knowledge, this is the first case of orbital granulomatous involvement associated with interferon-alpha therapy.

Fluorine-18-fluorodeoxyglucose positron emission tomography ([18F]FDG PET) was assessed as a method for providing information about the malignancy of orbital tumors. Twelve patients with 13 orbital tumors underwent [18F]FDG PET followed by biopsy or tumor removal via a transcranial approach. The accumulation ratio between the tumor and the contralateral normal tissue (T/N ratio) was calculated for 10 of the 13 lesions. The T/N ratio in benign lesions was compared with that in malignant tumors. Histological examination identified 7 lesions as malignant: anaplastic astrocytoma of the optic nerve in 1 patient, which recurred as glioblastoma of the optic nerve, malignant lymphoma of mucosa-associated lymphoid tissue type in 1 patient, malignant melanoma in 1 patient, adenoid cystic carcinoma in 2 patients, and adenocarcinoma (unknown origin) in 1 patient. The T/N ratio was 1.06 +/- 0.03 (mean +/- standard deviation) in benign tumors, and significantly higher at 1.81 +/- 0.27 in malignant tumors (p = 0.0027). Both patterns of high and iso uptake of [18F]FDG were found in orbital pseudotumor. [18F]FDG PET can determine the malignancy of orbital tumors, but cannot distinguish malignant tumor from inflammatory disease such as pseudotumor.

An 81-year-old woman with right orbital inflammation and acute retinal necrosis following Herpes zoster ophthalmicus was evaluated and treated. CT showed right massive superior and inferior oblique enlargement and moderate enlargement of the remaining extraocular muscles with tendon sparing. The myositis and acute retinal necrosis dramatically improved following prednisone and intravenous acyclovir therapy.