Gaze-evoked amaurosis from idiopathic orbital inflammation.

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

Graves' orbitopathy, idiopathic orbital inflammatory pseudotumor and Epstein-Barr virus infection: a serological and molecular study.

OBJECTIVE: One of the hypotheses on the pathogenesis of autoimmune diseases, including Graves' disease (GD) and Graves' orbitopathy (GO), involves bacterial or viral infections. Recently, Epstein-Barr virus (EBV) has been proposed to play a role in the pathogenesis of idiopathic orbital inflammatory pseudotumor (IOIP) in Asians. The aim of the present study was to investigate the possible association of GO with EBV infection/exposure, as compared with IOIP, using serum and tissue samples, as well as primary cultures of orbital fibroblasts. METHODS: Thirty-one patients were studied, including four with IOIP, ten with GO, nine with GD without GO and eight control patients without IOIP, GD and GO. All patients with IOIP and GO underwent orbital decompression. Control patients underwent palpebral surgery. Fibroadipose orbital tissue samples were collected. Serum anti-EBV antibodies were measured in all patients. EBV-DNA was measured in blood samples, orbital tissue samples and primary cultures of orbital fibroblasts. RESULTS: Serum assays showed that the vast majority of patients have had a previous exposure to EBV, but no one had an acute infection. EBV-DNA was detected in ~40% of blood samples from GO, GD and control patients, but in none of the IOIP samples. EBV-DNA was not detected in any of the orbital tissue samples tested or in primary cultures of orbital fibroblasts. CONCLUSIONS: EBV infection does not seem to be associated with GD, GO and IOIP in Caucasians. Whether EBV is involved in IOIP in Asians or other populations remains to be confirmed.

A case of concurrent silent sinus syndrome, thyroid eye disease, idiopathic orbital inflammatory syndrome, and dacryoadenitis.

This is a retrospective case description of a single female patient found to have concomitant bilateral silent sinus syndrome, bilateral thyroid eye disease, unilateral dacryoadenitis, and idiopathic orbital inflammation that presented as progressive unilateral right-sided proptosis. The spectrum of inflammatory orbital diseases can make discerning between different entities challenging, but more unique in this case was the simultaneous presentation with bilateral silent sinus syndrome, a rare entity in its own right. Identifying each of these concurrent disease processes is important to establishing a multidisciplinary treatment approach to address all the patient's orbital and peri-orbital pathology. We hope to highlight the clinical and radiographic findings unique to each of these entities and share our approach to treatment in this complex case.

Bilateral orbital pseudotumor in a patient with Takayasu arteritis: a case report and review of the literature.

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.

A case of non-lacrimal immunoglobulin G4 (IgG4)-related orbital disease with mastitis.

IgG4-related orbital disease is a recognised cause for orbital inflammation. As its awareness increases and diagnostic accuracy improves there will be an increased number of cases being identified. This unique case demonstrates for the first time, with histological evidence, a case of a non-lacrimal IgG4-related orbital disease with concurrent IgG4-related mastitis. We describe a 47 year old who presented with a supraorbital swelling and mass. This was initially successfully treated with oral steroids and was later excised on recurrence. Immunohistochemical and blood serum analysis confirmed IgG4-related orbital disease. On systemic enquiry she was found to have a mass of the breast, which was shown to be IgG4-related mastitis. She is currently asymptomatic with no sign of recurrence and is under long-term surveillance. This case highlights the importance of systemic work up in patients presenting with orbital foci of IgG4 disease.

Concomitant Idiopathic Orbital Inflammatory Pseudotumor and Thyroid-Associated Ophthalmopathy.

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.

Successful "medical" orbital decompression with adjunctive rituximab for severe visual loss in IgG4-related orbital inflammatory disease with orbital myositis.

IgG4-related disease (IgG4-RD) is an inflammatory condition of unknown etiology that can cause tumefactive lesions in a number of tissues and organs, including the orbit and ocular adnexa. Diagnostic criteria for IgG4-RD, including pathology and clinical features and pathology, have been recently proposed. This study presents the first case of unilateral acute visual loss secondary to IgG4-related orbital inflammatory disease with orbital myositis that was complicated by severe compressive optic neuropathy. After initial treatment with pulsed intravenous methylprednisolone, followed by rituximab and radiotherapy, there was a marked improvement in orbital inflammation and clinical and radiological improvement in the compressive optic neuropathy. After 9 months of follow up, the orbital inflammatory disease remained in remission.

Orbital Inflammatory Pseudotumor following mRNA COVID-19 Vaccination.

The authors present a case of orbital pseudotumor after mRNA COVID-19 vaccination. A 40-year-old otherwise healthy woman was referred to our oculoplastics unit because of left blepharoptosis of 2 months duration starting 1 week after she received her first Pfizer-BioNTech mRNA vaccination. On presentation, her best-corrected visual acuity was 20/20 in each eye. The external examination revealed left blepharoptosis and mild upper eyelid swelling. Orbital magnetic resonance imaging revealed left lacrimal gland enlargement with homogeneous contrast enhancement and diffuse mild enlargement of the left lateral and superior rectus muscles. The results of the extended workup for autoimmune and infectious etiologies and the systemic examination findings were normal. Systemic corticosteroids were started for the orbital pseudotumor. The presented case of orbital pseudotumor development after the mRNA vaccine may be considered to be an immunological process targeting the orbital tissue following immunization, although the cause-effect relationship remains uncertain.

Idiopathic orbital inflammation leading to unilateral blindness over a 2-day presentation in a child.

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.

Pterygomaxillary extension of orbital pseudotumor. Case report.

Idiopatic pseudotumor (IP) is a non-specific, non-neoplastic, inflammatory process without identifiable local or systemic causes. IP most commonly affects the orbit, comprising 5% to 8% of all orbital masses with occasional extension to other areas of the skull base. The ophthalmology literature has detailed reviews of IP as a space-occupying intraorbital lesion, but lesion involving only the skull base and sparing the orbit is exceptional. Understanding the presentation, work-up, and characteristics of the lesion is important for proper diagnosis and treatment. We report a rare case of orbital IP with extension into infratemporal fossa. Clinical and radiological findings and surgical approach are discussed.

Inflammatory pseudotumour affecting the lung and orbit.

This report describes a rare case of inflammatory pseudotumour arising metachronously from the orbit and lung. A 78-year-old man with a 7-year history of orbital inflammatory pseudotumour developed a solitary mass in the right lung. Serological studies showed elevated levels of IgG and antinuclear antigen. Wedge resection of the lesion was performed under VATS. Histology showed that lymphoplasmacytic infiltration with dense fibrosis and flow cytometry proved these cells to be polyclonal. Immunostaining revealed numerous IgG4-positive plasma cells diffusely infiltrating the lesion.

A case of relapsing flitting bilateral idiopathic orbital inflammation.

Idiopathic orbital inflammation (IOI) is defined as a benign non-infective clinical syndrome characterized by features of non-specific inflammation of the orbit without identifiable local or systemic causes. This can be called orbital myositis if the inflammation is predominantly in the orbital muscles. It is a diagnosis of exclusion based on clinical, radiological, and if necessary, histological findings. The most commons symptoms are swelling, ptosis, proptosis and painful eye movements. To our knowledge, this patient is the first with IOI to demonstrate relapsing flitting bilateral involvement of several individual extra-ocular muscles.

[Progressive axial exophthalmy--case report]. / Exoftalmie axiala progresiva--caz clinic.

INTRODUCTION: It is being presented the clinical case of a 67 year old male patient who has been under clinical observation and treatment for 10 years. The diagnosis rests upon clinical methods, imaging techniques (ocular-orbital examination, computerized tomography and magnetic resonance imaging) as well as post-operative histopathology reports. RESULTS: The clinical diagnosis was supported by the progressive decrease in visual acuity, painful, irreducible, non-pulsating axial progressive exophthalmia, associated with disorders of the ocular motility initially at the level of the RE, followed by bilateral involvement. The RE ocular-orbital ultrasound and the MRI examination point out a homogenous retro bulbar formation that caudally compresses and exceeds the optic-nerve. The mass lesion from the right orbit was afterwards partially removed by neurosurgical excision. The general treatment was initiated with steroidal and non-steroidal anti-inflammatory drugs in repetitive cures, under protection of antibiotics and anti-secretory drugs, the response to treatment being unfavorable. In time, the exophthalmia increased progressively, fact that imposed right de-compressive orbitotomy, with the surgical ablation of the lateral orbital wall. About 1 year after the neurosurgical intervention the initial clinical symptoms insidiously reinstalled throughout 3 - 4 years. The ultrasound, completed by the cranium CT and MRI examinations have identified the bilateral presence of myositis. DEBATES: Regardless of all the therapeutical means applied in time, not only the medical treatment (the systemic corticotherapy), but also the surgical one (with palliative effect), the patient's evolution was unfavorable; the axial exophthalmia persists, it is painful and irreducible. The evolution is aggravated also by the fact that both orbits have been affected; the specialty literature mentions cases with frequent unilateral involvement. CONCLUSIONS: In the case presented the axial exophthalmia is irreversible; it is determined by an inflammatory pseudotumor of both orbits, that represents a chronic inflammatory, idiopathic disease, with unpredictable clinical evolution. The diagnosis is usually one of exclusion, the complementary imaging examinations being necessary to rule out other pathologies of the orbit.

Idiopathic Orbital Pseudotumor Preceding Systemic Inflammatory Disease in Children.

PURPOSE: To describe four pediatric cases in which isolated orbital pseudotumor preceded the development of a systemic inflammatory disease by months to years. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.].

IgG4-related disease in patients with idiopathic orbital inflammation syndrome: data from the French SIOI prospective cohort.

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