Idiopathic Orbital Pseudotumor Research Articles

Correlation Between IVIM-DWI Parameters and Pathological Classification of Idiopathic Orbital Inflammatory Pseudotumors: A Preliminary Study.

ObjectiveTo investigate the correlation between intravoxel incoherent motion diffusion-weighted imaging (IVIM-DWI) and the pathological classification of idiopathic orbital inflammatory pseudotumors (IOIPs).MethodsNineteen patients who were diagnosed with IOIPs (a total of 24 affected eyes) between November 2018 and December 2020 were included in the study. All the patients underwent magnetic resonance imaging orbital plain scans and IVIM-DWI multiparameter scans before an operation. The true diffusion coefficient (D), pseudodiffusion coefficient (D*), and perfusion fraction (f) values were obtained. Based on histopathology, the lesions were divided into three types: lymphocytic infiltration, fibrosclerotic, and mixed. The correlation between IVIM-DWI parameters and pathological classification was tested with the histopathological results as the gold standard. The data were analyzed using SPSS version 17.0, with P < 0.05 defined as significant.ResultsAmong the 19 patients (24 eyes) affected by IOIP, there were no significant differences between IOIP pathological classification and gender or age (P > 0.05). There were statistically significant differences between the D and f values for different pathological types of IOIP and IVIM parameters (P < 0.05), and there was no significant difference in D* value between the different pathological types (P > 0.05).ConclusionThe D and f values showed correlation with different types of IOIP, and the sensitivity of the D value was higher than that of the f value. The D* value showed no significant distinction between pathological types of IOIP.

Tolosa-Hunt Syndrome: A Review of Diagnostic Criteria and Unresolved Issues.

Purpose:To review the diagnostic criteria for Tolosa–Hunt syndrome (THS) and utility of recent modifications.Methods:We searched PubMed for keywords Tolosa Hunt and magnetic resonance imaging. We compared the three editions of International Classification of Headache Disorders and isolated case reports and case series with the assessment of cavernous internal carotid artery (ICA) caliber to find the prevalence of vascular anomalies. We also evaluated cases of THS with the involvement of extracavernous structures and the possible role of idiopathic hypertrophic pachymeningitis (HP). Cases diagnosed falsely as THS were also reviewed for the presence of atypical features and relevance of criterion D. We assessed nonconforming cases (those with normal neuroimaging benign THS) and idiopathic inflammatory orbital pseudotumor (IIPO).Results:Vascular abnormalities were found in 36.36% of THS cases. Benign THS may also show changes in ICA caliber. Evidence suggestive of idiopathic HP could be found in 57% of cases with the involvement of extracavernous structures, such as facial nerve and pituitary gland. Both THS and IIPO are steroid-responsive pathologies with similar clinical and radiological features. False-positive diagnosis of THS results from early labeling, based solely on clinical features and symptom resolution after steroid therapy.Conclusions:Benign THS may be a result of limitation of resolution of available neuroimaging technique or early testing. Early and late vascular changes can be seen in both THS and its benign variant; some of them are not innocuous. THS may be considered a type of focal idiopathic HP. IIPO may represent an anterior variant of THS. In the absence of histopathological diagnosis, steroid-induced resolution of symptoms should be confirmed radiologically and followed-up.

Bilateral idiopathic orbital pseudotumour in a child: a case report

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

Idiopathic Orbital Pseudotumor Preceding Systemic Inflammatory Disease in Children.

To describe four pediatric cases in which isolated orbital pseudotumor preceded the development of a systemic inflammatory disease by months to years. The medical records of all patients with the clinical diagnosis of orbital pseudotumor seen at the Ocular Oncology Service of Wills Eye Hospital and Northern Virginia Ophthalmology Associates from 2010 to 2015 were reviewed retrospectively, and those associated with systemic inflammatory disease were selected for further study. Data were retrospectively collected from medical record review regarding patient demographics and clinical features, time to development of systemic inflammatory disease, and medical management. In four pediatric patients, isolated orbital pseudotumor preceded the development of a systemic inflammatory disease, including pauciarticular juvenile idiopathic arthritis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), granulomatosis with polyangiitis (Wegener's granulomatosis), and Crohn's disease. Orbital pseudotumor may be an antecedent to systemic inflammatory disease in children. Because this was a small case series, the authors are not ready to suggest that a full systemic work-up for systemic inflammatory disease is warranted for every patient with orbital pseudotumor. However, close observation and suspicion for systemic inflammatory conditions may be reasonable in children who present with idiopathic orbital pseudotumor. [J Pediatr Ophthalmol Strabismus. 2019;56(6):373-377.].

Orbital Pseudotumor Initially Presented as Chronic Sinusitis: A Case Report

Idiopathic orbital pseudotumor, is defined as a benign, non-infective lesion characterized by orbital inflammatory process. A 73-year-old man, with a 1 year history of severe headache and unilateral left-sided chronic pan-sinusitis without orbital involvement underwent functional endoscopic sinus surgery (FESS) surgery due to sinusitis recurrence. Also Dacryocystorhinostomy (DCR) surgery and re-surgery of sphenoid sinus and middle turbinecomy were conducted. About 5 months later, patient presented with ocular pain, diplopia and reduced left eye movements. He underwent surgery and biopsy was conducted for histopathology and immunohistochemistry (IHC) and their reports confirmed the diagnosis of orbital pseudotumor. The initial symptoms did not start from orbit, interestingly. Notably, one of the differential diagnoses of unilateral chronic sinusitis can be orbital pseudotumor.

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.

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-

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.

Idiopathic Orbital Myositis

There is a paucity of reliable data and limited experience on the clinical features and therapeutic outcomes of orbital myositis. The purpose of this study was to collate data on the clinical features, imaging, diagnosis, and therapeutic effects of case reports from patients with myositis of idiopathic orbital inflammation pseudotumor in a tertiary eye hospital in China. A retrospective study was performed on the records of 44 Chinese patients with orbital myositis. Data were obtained from the period of January 1, 2000, to August 31, 2010, from patients treated at the Eye Hospital of the Zhongshan Ophthalmic Center at Sun Yat-sen University, Guangzhou, China. Twenty-five patients were women and 19 men. The mean age was 39.1 years (range, 11-77 years). Right eyes were involved in 18 cases, left in 17 cases, and both eyes in 9 cases. Dysfunctions of the affected muscles included pure paretic (20.5%), pure restrictive (45.5%), or mixed paretic-restrictive (34.1%). The ratio of acute to subacute stage was 1:3. The rank order of affected muscles was as follows: superior rectus (29.1%), lateral rectus (25.6%), medial rectus (24.4%), inferior rectus (19.8%), and superior oblique (1.16%). The proportion of single muscle involvement was 37.5%, and tendon involvement was seen in 40.9% of the muscles. All patients were treated with systemic corticosteroids (prednisone or dexamethasone). Full recovery was achieved in 38.6% of patients, whereas 59.1% achieved partial recovery with an average of 6.4 recurrences (range, 2.0-8.0 recurrences). Recurrences occurred in 81.8% of the patients. Orbital myositis occurs in multiple clinical manifestations and may be recurrent. Imaging is an important technique for use in diagnosis. Systemic corticosteroid represents an effective approach for treatment.

Restrictive orbital myofibroblastic sarcoma in a cat – Cross‐sectional imaging (MRI&amp;CT) appearance, treatment, and outcome

A 16-year-old spayed female cat was evaluated for lagophthalmos and chronic exposure keratitis in both eyes. Ophthalmic examination revealed upper and lower eyelid entropion of the left eye (OS) and markedly decreased retropulsion, restricted eye movement, marked episcleral congestion, and severe keratitis of both eyes (OU). Magnetic resonance imaging of both orbits revealed extensive, irregular, contrast-enhancing tissue without evidence of osteolysis considered compatible with diffuse inflammatory tissue. Feline herpesvirus DNA was not detected in conjunctival samples. Partial temporary tarsorrhaphies were placed OU, and the cat was treated with topically administered erythromycin ointment OU, orally administered famciclovir and prednisolone, and sublingually administered buprenorphine. Little improvement was noted after 2weeks. Six weeks after initial presentation, a left exenteration was performed and histopathology was consistent with idiopathic sclerosing orbital pseudotumor (ISOP). Ten weeks after initial presentation, the patient represented for weight loss and jaw pain. Computed tomography demonstrated disease progression in the right orbit and the patient was euthanized. Histopathology of the decalcified skull revealed an aggressive and highly infiltrative mass involving the right orbit with extension to the maxilla, hard palate, nasal cavity and gingiva most consistent with feline restrictive orbital myofibroblastic sarcoma (FROMS). Clinical data from this patient support the reclassification of ISOP as FROMS. MRI and CT may provide supportive evidence for FROMS, but histopathology is necessary for definitive diagnosis. Aggressive and early surgical treatment, including bilateral exenteration, with adjunctive radiotherapy and/or chemotherapy should be considered for patients with FROMS.

Quantification of Epstein-Barr virus DNA in patients with idiopathic orbital inflammatory pseudotumor.

Inflammatory pseudotumors (IPT) are soft tissue tumors that include a diverse group of lesions characterized by inflammatory cell infiltration and variable fibrotic responses. Idiopathic orbital inflammatory pseudotumors (IOIP) are IPTs of unknown etiology that develop in the orbit. Due to the lack of well-defined pathogenic mechanisms, diagnosis and treatment of this disease remain a significant challenge. Epstein-Barr virus (EBV) infection, which causes significant lymphocyte infiltration, has been proposed to be involved in IOIP. This study tries to validate the relationship between EBV infection and the development of IOIP. Sixteen IOIP tissue samples were obtained from patients during surgical resection of the lesion. One Graves' ophthalmopathy tissue sample and 20 normal donors' plasma serves as controls. The plasma level of five EBV antibodies, including VCA-IgG, VCA-IgA, VCA-IgM, EA-IgG and EBNA1-IgG were examined. All plasma samples were EB-VCA-IgG positive and EB-VCA-IgM negative, suggesting that all people tested had been infected with EBV but not in the acute infection stage. EBV-DNA was detected in 15/16 (94%) of IOIP tissue samples despite different levels of lymphocyte infiltration and 5/16 plasma samples (31%) were detected EBV DNA positive which is higher than the normal controls (10%). Percent of positive plus suspected positive samples with one or more of the three important risk markers (VCA-IgA, EA-IgG, EBV-DNA) is 50% of the patients (8/16) which is much higher compare with the normal controls (20%). The results further reveal the relationship between IOIP and EBV infection.

Imaging Appearance of Orbital Inflammatory Processes

Correspondence to : Dr. Argha Chatterjee E-Mail : arghachat84@gmail.com The clinical diagnosis of orbital inflammation is often straightforward and in mild cases, imaging may not be necessary. However, imaging is warranted when there is clinical evidence of post-septal inflammation (e.g. proptosis, conjunctival chemosis, ophthalmoplegia) to determine the extent of the lesion and to look for complications such as abscess formation, optic nerve compression, vascular Figure 1: A. 17 year old male with Orbital cellulitis B. coronal and C. axial post-contrast CT shows enhancing increased soft issue and periorbital fat infiltration more prominent superonasally. Note anterior ethmoid sinus opacification (arrowheads) suggesting cellulitis is likely to be secondary to sinus pathology. Figure 2: A. 19 year old girl with Cavernous sinus thrombosis. B. axial post-contrast CT shows enlarged right sinus with filling defects (white arrow). C. coronal post-contrast CT shows dilated superior ophthalmic vein right side (thick arrow) in and absence of sinus opacification. Figure 3: Post-enucleation Intraorbital abscess, right side. A. T2 weighted and B. fat saturated T1 weighted MR images show a well defined ovoid intraorbital lesion showing T2 hyperintensity. C. post-gadolinium MR images show strong rim-like enhancement of the lesion suggesting an abscess. D. diffusion weighted image show strong hyperintensity suggestion diffusion restriction. Figure 4: Subperiosteal abscess-value of USG. A. coronal post-contrast CT shows an ill-defined extraconal variably enhancing lesion in the superonasal part of right orbit (arrow). B. USG clearly shows the cystic nature of the lesion with internal fluid level (arrowhead). Figure 5: A. 9 year old boy with Bilateral anterior ethmoid mucocele. B. coronal post-contrast CT reveals well-defined hypodense lesion expanding both anterior ethmoidal air-cells, left larger than right. Note absence of lamina papyracea on left side and elevated enhancing periosteum (arrowheads) displacing the left eye.Figure 6: A. 34 year old male with Orbital Cysticercosis. B. axial post-contrast CT reveals ring enhancing cystic lesion surrounded by phlegmon involving superior rectus-levator palpebre superioris complex (arrow). Insert show magnified view of the lesion. Note the hyperdense dot representing the scolex.Figure 7: Idiopathic Orbital Inflammatory Pseudotumour. A. Axial post-contrast CT reveals enhancing soft tissue surrounding the globe on left side with involvement of lacrimal region (white arrow) and uveoscleral thickening. B. Fluid attenuated inversion recovery (FLAIR) and C. T1 weighted MRI reveals hypo to isointense soft tissue replacing bright orbital fat. Proximal part of optic nerve is thickened (black arrow in B). Note uveoscleral thickening better demonstrated in MR (arrowhead in B).Figure 8: A. 53 year old withThyroid orbitopathy. B. Axial CT reveals enlargement of medial and lateral recti of both orbit, left more than right side. Note sparing of attachment sites to globe. Figure 9: A. 35 year old lady with Orbital hematoma mimicking cellulitis. B. axial USG, C. axial CT, C. Axial fat-saturated T1 weighted MR and D. axial T2 weighted MR images show a well-defined lesion in the left orbit with fluid level (arrow). Hyperdensity on CT, hypointensity on T2 and corresponding hyperintensity on T1 suggested the diagnosis of hematoma. 1(a)

Tolosa-Hunt syndrome

Tolosa-Hunt syndrome (THS) is a rare disorder indicated by recurrent painful ophthalmoplegia caused by non-specific inflammation of the cavernous sinus or superior orbital fissure (SOF). The disease shares histopathological features with idiopathic orbital pseudotumour; however, owing to its anatomical location, it produces characteristic clinical manifestations.1 Recurrent retro-orbital pain, with palsies of the third, fourth or sixth cranial nerves as well as the first and second divisions of the trigeminal nerve, are typical. Clinically, immediate response to steroid therapy is a hallmark of the condition. The clinical presentation of THS has a wide differential diagnosis, and timely and appropriate imaging – as an adjunct to pertinent laboratory investigations – can greatly assist clinicians with early accurate diagnosis and management.

Myositic Type of Idiopathic Orbital Pseudotumor in a 4-Year-Old Child: A Case Report

Idiopathic orbital pseudotumor is a benign, noninfectious, and nonneoplastic disease with unknown cause. It is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorder. Idiopathic orbital pseudotumor is extremely rare in pediatric age group and may cause real diagnostic problems. This paper describes a 4-year-old girl who presented with sudden ptosis in the right eye and swollen eyelid. She recovered completely with high-dose steroid therapy. We report clinical and magnetic resonance imaging findings of orbital myositis, which is a rare subtype of idiopathic orbital pseudotumor in children and needs to be differentiated from other orbital disease especially malignancy.

Acute acquired comitant esotropia after orbital cellulitis

Acute extraocular muscle restriction in children is a rare condition; orbital myositis is the most common cause. Most neuropathic or myopathic causes of strabismus present as an incomitant deviation. We report a case of orbital cellulitis preceding acute comitant esotropia in a patient showing no evidence of myositis or neurologic pathology.

Idiopathic sclerosing orbital pseudotumor in seven cats

Objective To review the clinical presentation and histopathologic findings on a series of cats with orbital fibrotic disease and compare the data to that of humans with sclerosing orbital pseudotumor. Animals A retrospective study was undertaken, which identified tissue samples from seven cats between 1997 and 2002 with a history of orbital mass effect and pathology characterized by fibrous tissue proliferation. Procedure Information was obtained from medical records for affected cats, including age, sex, clinical signs, management, and outcome, with histopathology re‐examined. Results Six of seven cats presented with unilateral orbital involvement that progressed to bilateral orbital disease despite treatment. Onset was insidious, evolving over weeks to months and was associated with fixation of orbital structures. Owners of six of the cats opted for euthanasia because of disease progression and pain. Histopathology of affected orbital tissue included extensive fibrosis with encapsulation of normal tissues without characteristics of neoplasia. Conclusions Clinical findings and histopathology of globes and orbital tissues in cats bore many similarities to idiopathic sclerosing orbital pseudotumor in humans. In cats, the prognosis for the globe appears to be poor but an elucidation of the pathogenesis and earlier diagnosis coupled with more aggressive treatment modalities as indicated in humans may be beneficial.

Paquimeningite hipertrófica idiopática craniana associada a pseudotumor orbitário: relato de caso

Hypertrophic pachymeningitis is a rare disorder characterized by meningeal thickness, that can be caused by infection, tumoral infiltration, inflammatory disorders or idiopathic. We report the case of a 40 year-old man that presented with longstanding headache and progressive bilateral visual loss and proptosis. Cranial and orbital magnetic resonance imaging revealed diffuse dural thickness and bilateral extraconal orbital lesion. Extensive investigation did not reveal any systemic condition. Histopathological study after meningeal and orbital biopsy disclosed a chronic inflammatory process compatible respectively with idiopathic hypertrophic pachymeningitis (IHP) and orbital pseudotumor. This case emphasizes that orbital involvement can occur in IHP and that its early identification is of great importance in order to improve the visual prognosis of this condition.

Role of neuroendocrine factors on idiopathic orbital inflammatory pseudotumors

目的 探讨眼眶特发性炎性假瘤(IOIP)的发病是否与神经内分泌调控因子有关.方法选取近3年甲醛固定石蜡保存的IOIP标本30例,行免疫组织化学检测神经生长因子受体(NGFR)和生长激素(GH)表达情况.结果IOIP病变中所有淋巴滤泡组织内淋巴细胞都有NGFR的表达,淋巴滤泡结构以外的浸润淋巴细胞和其他炎症细胞等免疫组织化学结果为阴性.所有IOIP病变组织内GH的免疫组织化学检测均为阴性.结论NGF可能对IOIP患者眼眶局部淋巴细胞的积聚、活化、增殖等方面有促进作用,作为一个正调节因子在IOIP发病过程中发挥效应.GH在IOIP的发病过程中作用意义不大。

Angle-closure glaucoma in association with orbital pseudotumor

Purpose To describe the pathophysiology of angle-closure glaucoma secondary to idiopathic inflammatory orbital pseudotumor. Design Retrospective, small noncomparative case series. Participants Three patients with angle-closure glaucoma and orbital pseudotumor. Methods The pathophysiology of this entity was investigated using magnetic resonant imaging (MRI) and ultrasound biomicroscopy (UBM). Main outcomes measures Clinical features, anterior chamber angle configuration, and intraocular pressure. Results Angle closure from anterior rotation of the ciliary body caused by choroidal effusions secondary to pseudotumor was demonstrated using MRI and UBM. Two of the three cases resolved after treatment for orbital pseudotumor. Conclusions Idiopathic orbital pseudotumor is a cause of secondary angle-closure glaucoma. The mechanism of angle closure is anterior rotation of the ciliary body secondary to choroidal effusions resulting from the orbital inflammation.

IMAGING OF ORBITAL LYMPHOPROLIFERATIVE DISORDERS

Lymphomas and leukemias account for a large portion of orbital tumors. Orbital lymphoma accounts for 55% of malignant orbital tumors in adults. Idiopathic orbital inflammatory pseudotumors are pathologic entities that often challenge ophthalmologists and radiologists. This article describes the MR and CT features of orbital lymphoma, leukemia, and some other lymphoproliferative disorders.