Orbital Pseudotumor Research Articles

Relationship between dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and paranasal sinusitis.

To determine the clinical features of the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and its relationship with paranasal sinusitis. A retrospective analysis of 46 patients who received surgical treatment at the Department of Ophthalmology, Beijing Tongren Hospital, Capital Medical University for the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor from October 2010 to December 2012. Each patient underwent magnetic resonance imaging (MRI) of the orbits and the 4 paranasal sinuses. Disease status and the level of serum immunoglobulin G4 (IgG4) was measured before and 6mo after surgery. The initial clinical feature of the idiopathic dacryoadenitis type of orbital inflammatory pseudotumor was redness or swelling of the eyelids. Masses were palpated in the area of the lacrimal gland in some patients. Of the 46 patients, 16 also suffered from sinusitis (34.8%), with 14 cases of ethmoid sinusitis, 8 cases of maxillary sinusitis, 9 cases of sphenoid sinusitis, and 8 cases of frontal sinusitis. Of the 16 patients with sinusitis, 4 patients had a medical history of rhinitis (range: 10mo to 15y previously), 10 patients had occasional nasal congestion, and 2 patients had no nasal congestion. Thirteen of the 46 patients had elevated serum IgG4 levels. Nine of these 13 patients had MRI signs of sinusitis. All patients (n=46) received oral glucocorticoid treatment for approximately 3mo after surgery. No sign of recurrence was found in the orbital MRI 6mo after surgery. Of the 16 patients with sinusitis, 9 cases of elevated serum IgG4 levels improved after treatment with decreased serum IgG4 level and 7 cases of normal serum IgG4 levels remained unchanged. Some patients with the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor may also suffer from paranasal sinusitis. The incidence of paranasal sinusitis was much higher in patients with IgG4-elevated dacryoadenitis subtype orbital inflammatory pseudotumor than in those with normal IgG4 levels. Dacryoadenitis subtype orbital inflammatory pseudotumor and paranasal sinusitis may both the clinical manifestations of IgG4-related disease involved in different locations.

Role of IgG4 serology in identifying common orbital lymphoproliferative disorders.

To explore the role of IgG4 serology in identifying common orbital lymphoproliferative disorders. Eighty-one patients with orbital lymphoproliferative diseases were treated in the Department of Ocular Oncology, Beijing Tongren Hospital, Capital Medical University between September 2010 and December 2012. Serum IgG4 levels were measured in 46 cases of idiopathic orbital inflammatory pseudotumor (IOIP), 17 benign lymphoepithelial lesion (BLEL), 12 cases of orbital mucosa-associated lymphoid tissue (MALT), and 6 cases of diffuse large B-cell lymphoma (DLBL) using immuno-scatter turbidmetry (ISTM). The frequency of elevated IgG4 levels in patients with IOIP, BLEL, MALT, and DLBL was 30.43% (14/46), 76.47% (13/17), 8.33% (1/12), and 0.00 (0/6), respectively. Among the patients with elevated serum IgG4 levels, all IgG-IOIP patients were male, and 92.31% of the IgG4-BLEL patients were female (12/13). The mean serum IgG4 level of IgG4-IOIP patients was lower than that of individuals with IgG4-BLEL, but the variation in serum IgG4 levels was larger in IgG4-IOIP than IgG4-BLEL patients. Only one case of IgG4-MALT with elevated serum IgG4 levels had a medical history >10y, which was significantly longer than the MALT patients with normal serum IgG4 levels. There was no significant elevation of serum IgG4 levels in patients with DLBL. Detecting serum IgG4 levels plays an important role in the differential diagnosis of orbital lymphoproliferative diseases.

Excision of a Lower Eyelid Inflammatory Pseudotumor Combined with Lower Blepharoplasty.

Excision of a Lower Eyelid Inflammatory Pseudotumor Combined with Lower Blepharoplasty.

Orbital Pseudotumor in a Patient Treated With Zoledronic Acid: A Case Report and Pertinent Literature Review

ABSTRACT Objective: To enhance recognition of ocular inflammation as a rare side effect of zoledronic acid. Methods: A case report of bisphosphonate-induced orbital inflammation with permanent visual deterioration and pertinent literature review. Results: A 66-year-old man with newly diagnosed osteoporosis and concomitant hypertension, hyperlipidemia, and gastroesophageal reflux disease was treated with intravenous zoledronic acid. He developed acute anterior uveitis on day 4 after treatment, manifested by orbital erythema, swelling, and decreased visual acuity. His symptoms resolved after a course of systemic glucocorticoid. However, he developed blurred vision 3 months after treatment with zoledronic acid and was diagnosed with cystoid macular edema and wrinkling retinopathy, thought to be related to prior history of uveitis. He underwent intravitreal steroid injection, with a partial response 6 months after use of zoledronic acid, later followed by retinal surgery. At 24 months after zoledronic acid in...

Sclerosing orbital pseudotumor

Sclerosing orbital pseudotumor is a rare subtype of idiopathic orbital inflammatory pseudotumor. It's more common in adults and presents diagnosis of exclusion. Steroids represent the first option of treatment. The sclerosing orbital pseudotumor subtype shows moderate response to steroids due to the predominance of fibrosis and collagen in its histology. We report on a case of a patient with histologic diagnosis of sclerosing orbital pseudotumor successfully treated with corticosteroid associated with azathioprine.

An Unexpected Cause of Amaurosis Fugax

IgG4-RD is an increasingly recognized group of immune-mediated conditions that share specific pathologic, serologic, and clinical features. It is a systemic inflammatory disorder that typically causes tumor-like swelling of involved organs, a lymphoplasmacytic infiltrate with predominance of IgG4-positive plasma cells with a variable degree of fibrosis, and elevated levels of serum IgG4. Lacrimal and orbital involvement is rare and when present is referred to as IgG4-related ophthalmic disease. Biopsy is the gold standard for diagnosis. The optimal treatment strategy has yet to be determined but glucocorticoids and rituximab have shown success. We report a case of amaurosis fugax and orbital inflammatory pseudotumor as an unusual presentation of IgG4-RD. IgG4-RD though rare, is likely underdiagnosed and often misdiagnosed. It is important that physicians are aware of the clinical spectrum of IgG4-RD as recognition and accurate diagnosis is crucial and early treatment may induce remission and prevent progression.

Implications of enlarged infraorbital nerve in idiopathic orbital inflammatory disease

PurposeTo investigate the clinical characteristics of idiopathic orbital inflammatory (IOI) disease with infraorbital nerve (ION) enlargement.DesignRetrospective, comparative case series.MethodsParticipants: Consecutive patients who were diagnosed with IOI between January 2009 and...

A Case Study on Idiopathic Orbital Pseudotumor: Surgery and Steroid Treatment

This is a case study of five isolated orbital nerve inflammatory pseudotumor cases presenting with protrusion and visual acuity of the right eye. Optic disk edema was observed by ophthalmoscopy. Plain and contrast-enhanced magnetic resonance imaging (MRI) were used to examine the orbital fat and enlargement of the right optic nerve sheath along with orbital magnetic resonance imaging diagnostic test. The visual acuity (VA) of all the admitted patients was 1.5/20. All the patients reported attacks of retrobulbar pain and severe headache at fairly regular intervals. The patients on clinical examination were found to have protrusion, reduced visual acuity of the right eye with an ipsilateral afferent pupillary defect. There was no motility of the right eye and the exophthalmos of the right part was 27 mm. On examination, patients were diagnosed to have idiopathic orbital inflammation and received steroid therapy for 6 months. Thereafter, patients received surgical treatment to remove the superior wall of the orbit followed by cortisone therapy for a further 6 months. The follow-up examination of the patients for one year revealed a gradual improvement in the vision of all the patients. The VA of the right eye for all the patients was 6/20, and the exophthalmos was now around 19 mm. Thus, a dramatic response to surgery and steroid treatment was observed in all the patients. Keywords: Orbital fat, Visual acuity, Optic disk edema, Optic nerve, Cortisone therapy

Inhibitory effects of dexamethasone on proliferation of fibroblasts derived by idiopathic orbital inflammatory pseudotumor

Background Idiopathic orbital inflammatory pseudotumor (IOIP) is a common orbital disease, but its etiology is still unclear, so the effect of glucocorticoid treatment is unsatisfied. Objective This study was to investigate the effects of dexamethasone on orbital fibroblasts from IOIP patients and explore the action machanism. Methods Six pieces of IOIP tissues from 6 IOIP patients and 3 pieces of normal orbital connective tissues from lacrimal gland prolapse patients were obtained during the surgery in Beijing Tongren Hospital from November 2011 to January 2012.The orbital fibroblasts were cultured using explant culture method.The morphology of the cells were observed under the optical microscope, and biomarks of the cells were detected by immunochemistry.The growth and proliferation of the cells were assayed using WST-8.The expression of ICAM-1 in the cells in both the control group and the IOIP group was detected by immunochemistry.The fibroblasts were incubated in 96-well plates, and different concentrations of dexamethasone (0, 1×10-3, 1×10-4, 1×10-5 and 1×10-6 mol/L) were respectively added into the medium for 24, 48 and 72 hours, and then the proliferation of the cells was detected by WST-8 assay.The contents of ICAM-1 in different concentrations of dexamethasone groups were assayed by ELISA. Results The characteristics of the cells were similar between the control group and the IOIP group with the spindle shape and long protructions.The cells showed the positive response for vimentin and absent response for desmin, S-100, cytokeratin (CK). Compared with the control group, the growth speed of fibroblasts was fast in the IOIP group.The proliferative values of the cells (absorbancy) were gradually reduced with the increase of dexamethasone concentrations (Fconcentration=36.27, P=0.00) and the lapse of acting time (Ftime=3.69, P=0.00). In cultured cells without dexamethasone for 24, 48 and 72 hours, the mean expression levels of ICAM-1 were 0.298±0.008, 0.312±0.003 and 0.319±0.011, showing a gradually increasing trend.However, the expression of ICAM-1 was gradually reduced with the increases of concentrations and the lapse of acting time of dexamethasone (Fconcentration=75.17, P=0.00; Ftime=3.11, P=0.00). Conclusions Occurrence and development of IOIP is probably associated with the over-expression of ICAM-1 in orbital fibroblasts.Dexamethasone plays anti-inflammation and treating effects on IOIP by down-regulating the expression of ICAM-1 and inhibiting the proliferation of orbital fibroblasts. Key words: Orbital pseudotumor/drug therapy; Fibroblasts/drug effects; Glucocorticoids/pharmacology; Dexamethasone; Intercellular adhesion molecule-1; Down-regulation; Cell culture

Orbital tumours and tumour-like lesions: exploring the armamentarium of multiparametric imaging.

Although the orbit is a small anatomical space, the wide range of structures present within it are often the site of origin of various tumours and tumour-like conditions, both in adults and children. Cross-sectional imaging is mandatory for the detection, characterization, and mapping of these lesions. This review focuses on multiparametric imaging of orbital tumours. Each tumour is reviewed in relation to its clinical presentation, compartmental location, imaging characteristics, and its histological features. We herein describe orbital tumours as lesions of the globe (retinoblastoma, uveal melanoma), optic nerve sheath complex (meningioma, optic nerve glioma), conal-intraconal compartment (hemangioma), extraconal compartment (dermoid/epidermoid, lacrimal gland tumours, lymphoma, rhabdomysarcoma), and bone and sinus compartment (fibrous dysplasia). Lesions without any typical compartmental localization and those with multi-compartment involvement (veno-lymphatic malformation, plexiform neurofibroma, idiopathic orbital pseudotumour, IgG4 related disease, metastases) are also reviewed. We discuss the role of advanced imaging techniques, such as MR diffusion-weighted imaging (DWI), diffusion tensor imaging, fluoro-2-deoxy-D-glucose positron emission tomography CT (FDG-PET CT), and positron emission tomography MRI (MRI PET) as problem-solving tools in the evaluation of those orbital masses that present with non-specific morphologic imaging findings.Main messages/Teaching points• A compartment-based approach is essential for the diagnosis of orbital tumours.• CT and MRI play a key role in the work-up of orbital tumours.• DWI, PET CT, and MRI PET are complementary tools to solve diagnostic dilemmas.• Awareness of salient imaging pearls and diagnostic pitfalls avoids interpretation errors.

Sarcoidosis. Debut as orbital pseudotumour

Case report78-Year-old female patient being investigated for a left orbital pseudotumour of 17 days onset. She had proptosis, pain, with no limitation of eye movements, periorbital edema, and upper eyelid ptosis, compatible with an orbital pseudotumour. The MRI showed an upper outer left orbital mass adjacent to the side of eyeball and an increase in soft tissue at the level of lacrimal gland. The histopathology study of an eyelid lesion demonstrated the presence of non-caseating granulomas. DiscussionA definitive diagnosis of orbital sarcoidosis was made. Oral treatment with corticosteroids and methotrexate achieved gradual control of the disease.

Orbital Pseudotumor with Extraorbital Extension in Childhood - A Case Report and Review of Literature

Orbital Pseudotumor with Extraorbital Extension in Childhood - A Case Report and Review of Literature

A report of metachronous orbital involvement by IgG4 disease treated successfully with low-dose radiation therapy

A 27-year-old woman with a history of hypothyroidism presented with a complaint of left eye pain and swelling that had progressed over the period of a year, in addition to a band-like frontal headache. A magnetic resonance imaging scan of the face and orbits was performed, demonstrating a 2.5 × 1.8 × 1.7-cm contrast-enhancing lesion within the intraconal space of the left orbit, inferolateral to and superomedially displacing the optic nerves with extension toward the orbital apex. The distal optic nerve appeared to be encased by the lesion. Representative axial and coronal images are shown in Fig 1A, B. At the time, the right eye did not appear to be involved radiographically or clinically. A biopsy of the left orbital mass demonstrated a chronic inflammatory infiltrate consisting of polyclonal mature B and T lymphocytes. She was treated empirically with a trial of steroids for several months without significant durable relief, including a period of hospitalization for high-dose intravenous steroids. Because of the progression of her orbital pain, opioid analgesics were ultimately required. She was subsequently treated with 3-dimensional conformal radiation therapy to the left orbit for the diagnosis of idiopathic orbital pseudotumor. A total dose of 2550 cGy was prescribed in 17 fractions and delivered with a mix of 6-

Ophthalmologic manifestations of the granulomatosis with polyangiitis (Wegener)

PurposeWegener's granulomatosis (WG) is a severe focal granulomatous inflammation with variable ophthalmic manifestationsMethodsPatients with a confirmed WG, seen in a single tertiary center between 2008 and 2014 were included retrospectively. WG was proven based on biological or histological analysisResultsFive patients (3W/2M) aged 26–55 years, were included. Clinical features included one scleritis with dacryoadenitis, one bilateral episcleritis complicated with uveo‐scleritis, one bilateral Mooren's ulcer and two orbital inflammatory pseudotumor. Time between the onset of symptoms and the diagnosis was 100 months (range: 1–276 months). All patients had anti‐cytoplasm antibodies to polynuclear neutrophils and underwent biopsies. Histological features included vasculitis inflammatory necrotizing granulomas. Extraocular features were pulmonary lesions (100%), rhinosinusal involvment (80%), renal disease (40%), neurological injury (40%). All patients were initially treated with high‐dose corticosteroids, wich were progressively decreased and received maintenance immunosupressive drugs. Immunosuppressive treatment was initiated on average 64 months after the onset of signs (range: 1–276 months)ConclusionsWG‘s ophthalmologic symptoms are variable. Scleritis remains the most common manifestation, followed by orbital inflammation. Atypical manifestations such as Mooren's ulcer or uveitis can delay the diagnosis. A multidisciplinary approach is mandatory to control this life threatening condition. WG should be considered in all cases of chronic orbital inflammation, scleritis or keratitis to ensure prompt diagnosis and improve the systemic prognosis.

IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients

Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as ‘definite’ or ‘possible’ according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI).Results: Forty-one patients (15 females, 26 males) were included in this study: 26 with ‘definite’ IgG4-RD and 15 with ‘possible’ IgG4-RD. The median age at diagnosis was 62 years. The median follow-up was 36 months (IQR 24–51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking.Conclusions: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.

Sarcoidosis. Comienzo como pseudotumor orbitario

Case report78 year-old female patient being investigated for a left orbital pseudotumour of 17 days onset. She had proptosis, pain, with no limitation of eye movements, periorbital oedema, and upper eyelid ptosis, compatible with an orbital pseudotumour. The MRI showed an upper outer left orbital mass adjacent to the side of eyeball and an increase in soft tissue at the level of lacrimal gland. The histopathology study of an eyelid lesion demonstrated the presence of non-caseating granulomas. DiscussionA definitive diagnosis of orbital sarcoidosis was made. Oral treatment with corticosteroids and methotrexate achieved gradual control of the disease.

Idiopathic orbital pseudotumor preceding systemic inflammatory disease in children

Idiopathic orbital pseudotumor is a noninfectious and non-neoplastic clinical syndrome characterized by the presence of an inflammatory mass within the orbit. Orbital pseudotumor has been observed in association with systemic inflammatory diseases. We present three cases of orbital pseudotumor occurring months to years before the systemic disease.

IgG4-Related Disease: Results From a Multicenter Spanish Registry.

IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis.Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses.Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent.This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD.

Orbital pseudotumor can be a localized form of granulomatosis with polyangiitis as revealed by gene expression profiling

Biopsies and ANCA testing for limited forms of granulomatosis with polyangiitis (GPA) are frequently non-diagnostic. We characterized gene expression in GPA and other causes of orbital inflammation. We tested the hypothesis that a sub-set of patients with non-specific orbital inflammation (NSOI, also known as pseudotumor) mimics a limited form of GPA. Formalin-fixed, paraffin-embedded orbital biopsies were obtained from controls (n=20) and patients with GPA (n=6), NSOI (n=25), sarcoidosis (n=7), or thyroid eye disease (TED) (n=20) and were divided into discovery and validation sets. Transcripts in the tissues were quantified using Affymetrix U133 Plus 2.0 microarrays.Distinct gene expression profiles for controls and subjects with GPA, TED, or sarcoidosis were evident by principal coordinate analyses. Compared with healthy controls, 285 probe sets had elevated signals in subjects with GPA and 1472 were decreased (>1.5-fold difference, false discovery rate adjusted p<0.05). The immunoglobulin family of genes had the most dramatic increase in expression. Although gene expression in GPA could be readily distinguished from gene expression in TED, sarcoidosis, or controls, a comparison of gene expression in GPA versus NSOI found no statistically significant differences.Thus, forms of orbital inflammation can be distinguished based on gene expression. NSOI/pseudotumor is heterogeneous but often may be an unrecognized, localized form of GPA.

Somatostatin receptor scintigraphy of orbital inflammatory pseudotumor associated with systemic vasculitis

Objective To explore the value of somatostatin receptor scintigraphy(SRS) in evaluating the immune activity of orbital inflammatory pseudotumor associated with systemic vasculitis. Methods Twenty-five patients with orbital inflammatory pseudotumor associated with systemic vasculitis (10 males, 15 females, average age: (51.2±14.2) years) underwent SRS. The uptake ratio (UR) of orbital inflammatory pseudotumor was obtained. (1)Patients were divided into group A (with immune activity) and group B (without immune activity) according to Birmingham vasculitis activity score (BVAS). The difference of UR between the 2 groups was compared by two-sample t test. The difference of UR before and after treatment in 12 patients was also compared. (2) Based on the results by BVAS, ROC curve was used to obtain the cut-off value of UR, as well as the diagnostic efficiency and Youden index. The consistency between SRS and BVAS was calculated. (3)Patients were divided into two groups according to the cut-off value of UR and the prognosis difference between them was compared by Fisher exact test. (4)The expression of SSTR2 and SSTR5 was observed by immunohistochemistry. Results (1)UR in group A was significantly higher than that in group B (2.09±0.44 vs 1.32±0.46, t=5.94, P 0.05). (2)ROC curve analysis identified UR cut-off value as 1.66, with the sensitivity of 87.5%, specificity of 95.7%, positive predictive value of 95.2%, negative predictive value of 88.0%, accuracy of 91.3% and Youden index of 83.2%. The consistency between SRS and BVAS was strong (Kappa=0.840). (3) The prognosis was significantly different between patients with UR≥1.66 and UR<1.66 (P<0.05). (4) The immunohistochemical results revealed high expression of SSTR2 and SSTR5 in inflammatory cells in patients with immune activity. Conclusion SRS has potential value in evaluating the immune activity of orbital inflammatory pseudotumor associated with systemic vasculitis. Key words: Orbital pseudotumor; Systemic vasculitis; Radionuclide imaging; Receptors, somatostatin