[Role of imaging classification in the diagnosis and management of orbital inflammatory pseudotumor].

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.

Differential Diagnoses of Diseases Involving the Extrinsic Ocular Musculature ­ A Pictorial Essay

Introduction There are some inflammatory, infectious, and neoplastic diseases affecting the extrinsic orbital musculature (EOM) that present with pain, decreased visual acuity, and proptosis. Imaging is fundamental to the differential diagnoses of these diseases with similar clinical presentations. The present case series report has as main objective to illustrate and discuss the main pathologies that affect the orbit. Material and Methods The present series of cases discusses the main pathologies that can affect the extraocular musculature that can be characterized by computed tomography (CT) or magnetic resonance imaging (MRI) using cases from our institution. Results and Discussion The present study compiled several cases of ophthalmopathy from our institution to illustrate and address some of these pathologies, such as orbital lymphoma, Grave disease, metastases, periorbital cellulitis, and idiopathic orbital inflammatory syndrome. The diseases are discussed according to the presentation of clinical cases with emphasis on the main imaging findings of each pathology. Conclusion Computed tomography and MRI can help in the diagnosis and follow-up of the diseases that affect the EOM. We must be conversant with the main characteristics of the pathologies presented in the present case series report, since such findings together with clinical data can confirm the diagnosis of these diseases or at least help to narrow the differential diagnoses.

Disease modifying drugs in idiopathic sclerosing orbital inflammatory syndrome.

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

Idiopathic Lipogranulomatous Orbital Inflammation: A Case Report and Literature Review.

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.

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

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

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

Improving diagnostic performance of differentiating ocular adnexal lymphoma and idiopathic orbital inflammation using intravoxel incoherent motion diffusion-weighted MRI.

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 mm2/s, P < 0.001; D = 0.34 ± 0.15 vs. 0.76 ± 0.25 × 10-3 mm2/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 mm2/s, 0.56 × 10-3 mm2/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.

Idiopathic Orbital Inflammation With Superior Rectus and Levator Complex may be Misdiagnosed as Involutional Ptosis.

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.

Ull vermell I exoftàlmia: a propòsit de dos casos d'inflamació orbitària idiopàtica / Ojo rojo y exoftalmos: a propósito de dos casos de inflamación orbitaria idiopática / Red eye and exophthalmos: report of two cases of idiopathic orbital inflammation

INTRODUCCIÓ: La inflamació orbitària idiopàtica (IOI), o pseudotumor orbitari idiopàtic, forma part del diagnòstic diferencial d'ull vermell I exoftàlmia. És una malaltia inflamatòria, benigna I sense causa identificable, que respon majoritàriament a corticoteràpia. L'interès de tots dos casos recau en la variabilitat de la resposta al tractament de primera línia I el maneig complex en casos d'evolució atípica. OBSERVACIÓ CLÍNICA: Cas 1. Nen de 12 anys amb ull vermell d'evolució tòrpida, associat posteriorment a exoftàlmia, quemosi I dolor ocular. Es va diagnosticar d'IOI, amb una resposta excel·lent a la corticoteràpia, però amb corticodependència a la retirada progressiva del fàrmac. Va necessitar biòpsia per l'evolució atípica I tractament immunosupressor. Cas 2. Nena de 6 anys amb cefalea frontal I rinorrea, orientada inicialment com a sinusitis. Posteriorment va presentar ull vermell, dolor ocular, exoftàlmia, diplopia I altres signes inflamatoris locals. Es va diagnosticar d'IOI I es va iniciar la corticoteràpia, amb resposta excel·lent I remissió. COMENTARIS: La IOI requereix un exhaustiu diagnòstic diferencial, amb un diagnòstic final d'exclusió que descarti l'associació de malalties sistèmiques. N'és característica la ràpida resposta a glucocorticoides. La corticodependència observada en el primer cas és poc freqüent, I va caldre un esglaó terapèutic superior per aconseguir el control dels símptomes

INTRODUCCIÓN: La inflamación orbitaria idiopática (IOI), o pseudotumor orbitario inflamatorio, forma parte del diagnóstico diferencial de ojo rojo y exoftalmos. Es una enfermedad inflamatoria, benigna y sin causa identificable, que responde mayoritariamente a corticoterapia. El interés de ambos casos recae en la variabilidad de la respuesta al tratamiento de primera línea y el manejo complejo en casos de evolución atípica. OBSERVACIÓN CLÍNICA: Caso 1. Niño de 12 años con ojo rojo de evolución tórpida, asociado posteriormente a exoftalmos, quemosis y dolor ocular. Se diagnosticó de IOI con excelente respuesta a corticoterapia, pero presentando corticodependencia a la retirada progresiva del fármaco. Precisó biopsia por la evolución atípica, y tratamiento inmunosupresor. Caso 2. Niña de 6 años con cefalea frontal y rinorrea, orientada inicialmente como sinusitis. Posteriormente presentó ojo rojo, dolor ocular, exoftalmos, diplopía y otros signos inflamatorios locales. Se diagnosticó de IOI y se inició corticoterapia, con excelente respuesta y remisión. COMENTARIOS: La IOI requiere un exhaustivo diagnóstico diferencial, con un diagnóstico final de exclusión descartando la asociación a enfermedades sistémicas. Es característica la rápida respuesta a glucocorticoides. La corticodependencia apreciada en el primer caso es poco frecuente, y se requirió un escalón terapéutico superior para conseguir el control de los síntomas

INTRODUCTION: Idiopathic orbital inflammation (IOI) or orbital inflammatory pseudotumor is within the differential diagnosis of red eye and exophthalmos, and it is defined as a benign inflammatory disease without identifiable cause that responds to corticosteroid therapy. The two cases reported here highlight the variability of the response to the first line treatment, and the complex management in cases of atypical evolution. Case 1: A 12-year-old boy presented with a red eye with indolent evolution associated with exophthalmos, chemosis and eye pain. He was diagnosed with IOI and had an excellent initial response to corticosteroid therapy. However, the disease was corticosteroiddependent requiring a biopsy for confirmation, and initiation of immunosuppressive therapy. Case 2: A 6-year-old girl presented with frontal headache and rhinorrhea and was initially diagnosed with sinusitis. She later presented with painful red eye, exophthalmos, diplopia and other local inflammatory signs. She was diagnosed with IOI and had an excellent response and long-term remission to corticosteroid therapy. COMMENTS: IOI requires a comprehensive differential diagnosis, with a final diagnosis of exclusion ruling out the association with systemic diseases. The rapid response to glucocorticoids is characteristic of IOI. Corticosteroid dependence is rare and requires an increment in immunosuppressive therapy for disease control

Bisphosphonate-induced orbital inflammation in a patient on chronic immunosuppressive therapy.

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.

Differentiation of orbital lymphoma and idiopathic orbital inflammatory pseudotumor: combined diagnostic value of conventional MRI and histogram analysis of ADC maps.

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 (ADCmean), median ADC (ADCmedian), skewness, kurtosis, 10th, 25th, 75th and 90th percentiles of ADC (ADC10, ADC25, ADC75, ADC90) 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 ADC10 (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 ADC10 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 ADC10 are valuable in differential diagnosis of orbital lymphoma and IOIP.

[Idiopathic orbital inflammatory syndrome: Report of 24 cases]. / Syndrome d'inflammation orbitaire idiopathique : à propos de 24 cas.

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.

Bilateral Orbital Pseudotumor in a 3-Year-Old Child: A Case Report.

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.

Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review.

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.

Spontaneous regression of inflammatory myofibroblastic tumor of the orbit: A case report and review of literature.

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.

Head and neck inflammatory pseudotumor: Case series and review of the literature.

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.

Orbital Inflammation in Pregnant Women.

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.