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Purpose: The current study queries the American Academy of Ophthalmology (AAO) Intelligent Research in Sight (IRIS) registry for data on the epidemiology, work-up, and management patterns of autoimmune orbital inflammation. Methods: Analysis and description of patient data from the IRIS registry between 2013 and 2019 reviewing patients with autoimmune or idiopathic orbital inflammation with filters based on International Classification of Disease (ICD) and Current Procedural Terminology (CPT) codes. Patients with thyroid eye disease, orbital cellulitis, and orbital abscess were excluded. Main Outcome Measures: Demographic descriptions included gender, age, geographic region, and treatment. Sub-analysis was performed by assessing rates of imaging, biopsy, lab work-up, and diagnostic categories. Results: In a final cohort of 20,584 patients, the mean age of onset of orbital inflammation was 51.7 years; 67% female; and 63% Caucasian, 21% unknown, 12% Black, 2.6% Asian, and 1.5% other. Only 49 had imaging, 78 had laboratory work-up, and 1,411 had biopsy codes. Treatment results showed 166 patients receiving antibiotics, 224 patients receiving steroids, and 35 patients receiving both. Conclusions: This study assessed the epidemiology, diagnostic patterns, and treatment patterns for orbital inflammation through the AAO IRIS registry. Practise patterns suggest a relatively low overall rate of imaging and laboratory studies compared to biopsies, although this certainly under-represents the actual number of imaging and laboratory studies and exemplifies the inherent imprecision of using a large database. However, the methodology of this study provides a framework of approaching the IRIS registry for oculoplastic research. [ABSTRACT FROM AUTHOR]

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

Purpose: Orbital inflammatory disease has been historically diagnosed with computed tomography (CT) and magnetic resonance imaging (MRI). Orbital ultrasound has served as a non-radiation alternative that has been successful at diagnosing many orbital pathologies but is not commonly used in clinical practice due to need for specialized ultrasound training and equipment needs. We demonstrate use of handheld ultrasound for detecting orbital inflammation. Observations: We present five patients with orbital inflammation where a handheld ultrasound probe was able to capture features consistent with concurrent CT scans. Conclusions and importance: Handheld ultrasound is an accessible and portable method that can assist in the diagnosis and monitoring of orbital pathology.

Purpose: Ophthalmic manifestations of varying severity are often associated with systemic autoimmune conditions. Superior orbital fissure syndrome (SOFS) is a rare cranial neuropathy affecting nerves passing through the superior orbital fissure that causes a distinctive pattern of extraocular and pupillary findings. We report the coexistence of SOFS, relapsing polychondritis (RP) and Sjögren's syndrome (SS) in a 52-year-old female who presented with a past medical history of hypothyroidism, Raynaud's syndrome, and intermittent dry mouth and a 1-week history of worsening chemosis, proptosis, diplopia, and painful ophthalmoplegia. Methods: Following a comprehensive eye examination, the patient underwent a CT head with contrast, MRI of the orbit, lumbar puncture, and laboratory investigations. Results: CT and MRI examination revealed inflammatory standing in periorbital subcutaneous soft tissues and bilateral exophthalmos with right intraconal fat stranding surrounding the intraorbital and intracanalicular segments of the nerve, respectively. Lumbar puncture and laboratory investigations revealed an elevation in inflammatory biomarkers, a negative infectious workup, and ruled in SS when considering her history alongside a positive Schirmer test. She was started on high-dose steroids, which led to significant improvement; however, treatment revealed type 2 diabetes, necessitating a faster steroid taper, during which there was a reoccurrence of scleritis and ophthalmoplegia, leading to the initiation of rituximab infusions. After completing rituximab course, she was transitioned back to steroid therapy and was successfully tapered without event. Conclusion: This case is notable for the rare coexistence of SOFS with RP/SS overlap syndrome and highlights the management of concurrent orbital inflammatory syndrome and autoimmune diseases. [ABSTRACT FROM AUTHOR]

Orbital inflammatory disease (OID) comprises approximately 6% of orbital conditions, affecting individuals across all ages. The range of the primary orbital inflammation's differential diagnosis is extensive, encompassing autoimmune disorders such as thyroid diseases, vasculitis, sarcoidosis, connective tissue diseases, immunoglobulin G4-related disease (IgG4-RD), and giant cell myositis, whereas secondary causes span from infections to drug-induced causes. Analyzing histopathological aspects and cell populations could enhance our comprehension of the etiology of orbital inflammatory involvement in systemic diseases such as IgG4-RD. We present a series of four patients from our Rheumatology clinic, each with distinct systemic diseases, illustrating diverse manifestations of OID. This series was conducted to facilitate discussions and diagnoses of challenging cases of OID in a rheumatologic setting. The difficulty in the differential diagnosis arises from the extensive range of structures involved, resulting in a significant variation of clinical manifestations. Furthermore, the lack of definitive diagnostic laboratory tests and, often, histological findings add to the complexity. OID poses diagnostic challenges with variable clinical manifestations and overlapping imaging findings. As a diagnosis of exclusion, a comprehensive evaluation is crucial, often necessitating an orbital biopsy for confirmation. Collaborative efforts among specialists are essential for managing these intricate cases. [ABSTRACT FROM AUTHOR]

We performed a comprehensive systematic review to identify medication-associated orbital inflammation and to characterize its clinico-radiological features. We reviewed English-language articles describing medication-associated orbital inflammation (i.e., orbital myositis, dacryoadenitis and orbital fat) published to June, 2023. Isolated inflammation of the intraocular structures or globe alone (i.e. uveitis, scleritis, optic neuritis and perineuritis) were excluded. In medication-associated orbital inflammation, the extraocular muscles are preferentially affected, occurring in isolation or in combination with other orbital and/or intraocular structures. Clinico-radiological manifestations may be non-specific; however, certain medications may be distinguished according to the presence of systemic prodrome, laterality, associated intraocular inflammation, and predisposition to involve certain orbital structures. Rapid identification, discontinuation of the provoking medication, and systemic corticosteroid therapy (if appropriate) typically achieves a favorable visual prognosis. As new medications become adopted by clinicians, rare adverse effects will be further delineated.Medication-associated orbital inflammation is an important diagnostic consideration in orbital inflammatory disease. A careful medication history and clinical assessment may be revealing, permitting timely discontinuation of the offending agent and initiation of appropriate management. [ABSTRACT FROM AUTHOR]

Immune checkpoints (ICPs) are essential regulators of the immune system, ensuring a delicate balance between self-tolerance and autoimmune responses. ICP therapy is a rapidly growing cancer treatment strategy that inhibits the interaction between ICPs and their ligands. This biological interaction increases the ability of the immune system in combating cancer. However, in some cases, the use of these agents may lead to immune hyperactivity and, subsequently, autoimmune diseases. Graves' disease (GD), thyroid eye disease (TED), and orbital myopathy are complex autoimmune disorders characterized by the production of autoantibodies. The emergence of these treatment-related adverse events underscore the critical need for a deeper understanding of the immune-checkpoint axis in autoimmune diseases. In this review article, we provide a comprehensive survey of the biological mechanisms of ICPs that are most frequently targeted in cancer therapy, including CTLA-4, PD-1, PDL-1, and LAG3. Furthermore, we investigate the latest scientific findings on the adverse events associated with the inhibition of these ICPs. This paper will particularly focus on the potential risks these complications pose to ocular and orbital tissues, which are a concern in the context of cancer treatment. [ABSTRACT FROM AUTHOR]

VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a newly recognised adult-onset multisystem autoinflammatory disease caused by a somatic mutation in the UBA1 gene in myeloid or erythroid precursor cells. This report describes an atypical presentation of recurrent dacryoadenitis associated with VEXAS syndrome and provides a review of the literature. A 68-year-old male presented with three episodes of unilateral alternating dacryoadenitis followed by bilateral involvement over a 4-year period. Each episode of orbital inflammation was characterised by upper lid swelling, oedema and enlarged lacrimal glands. In addition, he experienced intermittent flares of angioedema-like lesions involving the face and extremities, recurrent jaw aches, rash, progressive pulmonary fibrosis, and myelodysplastic syndrome. His inflammatory symptoms lessened with prednisolone but were refractory to methotrexate. Mycophenolate was subsequently trialled with a reasonable clinical response. Genetic testing established the diagnosis of VEXAS syndrome and tofacitinib, a JAK inhibitor, was commenced with resolution of inflammatory symptoms. [ABSTRACT FROM AUTHOR]

Purpose of review: This review aims to provide a critical appraisal of current diagnostic and therapeutic strategies for patients with orbital inflammatory disease (OID). We present the reader with a review of clinical, imaging, laboratory, and biopsy based assessment of OID and review the current treatment modalities utilized including corticosteroids, corticosteroid-sparing (immunomodulatory) agents, radiation, antibiotics, and disease specific therapy. Recent findings: Two major developments and trends have emerged in the management of orbital inflammation. First, improved understanding and distinction of inflammation subtypes (myositis, dacryoadenitis, or infiltrative) allows for more nuanced workup and treatment. Second, immunomodulatory agents have shown promise in achieving disease control in cases of truly idiopathic or corticosteroid-resistant OID. Together, these advances have led to fewer adverse effects and better efficacy. Summary: The optimal treatment of OID depends on distinguishing between nonspecific and specific inflammation. Nonspecific inflammation tends to respond to corticosteroid therapy with a lower chance of relapse, while specific orbital inflammation often requires targeting the underlying disease with steroid-sparing therapy and immunomodulatory agents. [ABSTRACT FROM AUTHOR]

Systemic sclerosis (SSc) is a rare, chronic disease with diverse clinical presentations, and only a few cases with ocular manifestations have been reported in the literature. In this case report, we describe the case of a 51-year-old South Asian woman who initially complained of painless swelling in her left upper eyelid. An ultrasound examination of the left eye revealed an enlarged lacrimal gland with increased vascularity. The presence of dacryoadenitis, which was unresponsive to initial conservative management with oral antibiotics and warm compresses, along with positive antinuclear antibodies, prompted further investigation. Dacryoadenitis or orbital inflammation is a common presentation in systemic conditions such as Sjogren’s syndrome, systemic lupus erythematosus, sarcoidosis, or granulomatosis with polyangiitis. However, it can also be a rare initial symptom in a patient with CREST syndrome. To our knowledge, this is only the second case in the literature of dacryoadenitis in the context of calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome. This case highlights the significance of serological markers and peripheral clinical presentations in individuals with chronic orbital inflammation, emphasizing the importance of considering further systemic associations.

Ruxolitinib is an oral Janus associated kinase (JAK) inhibitor commonly used in the treatment of primary myelofibrosis. We describe a case of an 86-year-old woman on ruxolitinib for primary myelofibrosis who presented with a three-day history of worsening left eyelid swelling, pain, and decreased vision. Her exam was notable for left upper lid ptosis, periorbital edema, and nearly complete external ophthalmoplegia along with diffuse conjunctival injection and microcystic corneal edema. An orbital computed tomography demonstrated left proptosis accompanied by extensive inflammatory changes of the preseptal and orbital soft tissues. She was diagnosed with acute left orbital inflammatory syndrome (OIS) and treated with intravenous methylprednisolone, one gram over 48 hours followed by an oral steroid taper as well as discontinuation of her ruxolitinib. Complete recovery was noted at her one-week follow-up visit and ruxolitinib was restarted. However, 3 weeks later, she presented with new right periorbital swelling and pain and was found to have a sequential right OIS for which she was again treated with methylprednisolone and discontinuation of ruxolitinib with the goal of transitioning to an alternative biologic agent. Although there are no previous documented cases of ruxolitinib associated orbital inflammatory syndrome, a similar JAK inhibitor medication, Fedratinib, has been reported to cause a similar side effect. We propose that her ruxolitinib paradoxically lead to a pro-inflammatory state leading to bilateral, sequential orbital inflammatory syndrome. [ABSTRACT FROM AUTHOR]

Ipilimumab, an immune checkpoint inhibitor used in the treatment of metastatic melanoma, can cause immune-related adverse events including rare ocular-related inflammation. This is a case of a 54-year-old man with metastatic melanoma and bilateral orbital inflammation associated with ipilimumab that occurred after drug rechallenge 6 years after initial orbital inflammation with ipilimumab use. Imaging revealed tendon-involving myositis. He was managed with intravenous corticosteroid and intravenous immunoglobulin therapy with improvement in his orbital inflammation. This case demonstrates the potential ocular effects of ipilimumab use, differentiation of ipilimumab-associated orbital inflammation from thyroid orbitopathy and management considerations to effectively reduce orbital inflammation. [ABSTRACT FROM AUTHOR]

Introduction: Osteopetrosis is a rare heritable disorder characterized by increased bone density resulting from osteoclast dysfunction. Major complications include bone fracture, osteomyelitis, anemia, and cranial nerve compression. Optic atrophy can occur due to compression of the optic nerve. Although osteomyelitis of the jaw is a common complication, it rarely occurs in the maxilla. Here, we report a case of a 74-year-old female with osteopetrosis who developed maxillary osteomyelitis, leading to orbital inflammation. Case Presentation: She was referred to our clinic for 2 months of ptosis and swelling of the left eyelid and temporal region. Previous imaging revealed a left intraorbital occupying lesion, but a biopsy of the temporal subcutaneous tissue did not provide a definitive diagnosis. After 7 months, she presented with severe temporal swelling and purulent discharge. Upon examination, maxillary osteomyelitis resulting from caries of the upper jaw was observed. Treatment with oral antibiotics, drainage of the temporal skin fistula, and regular cleaning of the maxillary drainage improved her symptoms. Conclusion: This is a rare case of maxillary osteomyelitis associated with osteopetrosis, causing orbital inflammation.

Abstract Immune checkpoints (ICPs) are essential regulators of the immune system, ensuring a delicate balance between self-tolerance and autoimmune responses. ICP therapy is a rapidly growing cancer treatment strategy that inhibits the interaction between ICPs and their ligands. This biological interaction increases the ability of the immune system in combating cancer. However, in some cases, the use of these agents may lead to immune hyperactivity and, subsequently, autoimmune diseases. Graves' disease (GD), thyroid eye disease (TED), and orbital myopathy are complex autoimmune disorders characterized by the production of autoantibodies. The emergence of these treatment-related adverse events underscore the critical need for a deeper understanding of the immune-checkpoint axis in autoimmune diseases. In this review article, we provide a comprehensive survey of the biological mechanisms of ICPs that are most frequently targeted in cancer therapy, including CTLA-4, PD-1, PDL-1, and LAG3. Furthermore, we investigate the latest scientific findings on the adverse events associated with the inhibition of these ICPs. This paper will particularly focus on the potential risks these complications pose to ocular and orbital tissues, which are a concern in the context of cancer treatment.

IntroductionZoledronate is a commonly prescribed medication to maintain bone health; however, a rare side effect includes ocular inflammation. We report a case of simultaneous anterior uveitis and orbital inflammation associated with zoledronate infusion in a patient with metastatic breast cancer. We also performed a literature search to provide an up-to-date summary of cases with zoledronate-associated ocular inflammation.MethodsThis is a case report with literature review. Literature search (timeline 2010 to 2023) was performed using PubMed with the search team: (zoledronate) AND (uveitis OR scleritis OR orbital inflammation OR ocular inflammation).ResultsA 48-year-old female presented with left eye pain, swelling, and decreased vision 2 days after receiving zoledronic acid infusion. An ophthalmic exam showed non-granulomatous anterior uveitis. CT orbits and ocular ultrasound showed signs of posterior scleritis and orbital inflammation. Ocular inflammation caused by an infection or metastatic cancer was ruled out. The patient was treated with both topical and systemic corticosteroids. Complete resolution of the inflammation occurred after 2.5 weeks.ConclusionOrbital inflammation and uveitis are an uncommon side effect of zoledronate but needs to be promptly recognized and treated to prevent sight-threatening complications.

Introduction: Osteopetrosis is a rare heritable disorder characterized by increased bone density resulting from osteoclast dysfunction. Major complications include bone fracture, osteomyelitis, anemia, and cranial nerve compression. Optic atrophy can occur due to compression of the optic nerve. Although osteomyelitis of the jaw is a common complication, it rarely occurs in the maxilla. Here, we report a case of a 74-year-old female with osteopetrosis who developed maxillary osteomyelitis, leading to orbital inflammation. Case Presentation: She was referred to our clinic for 2 months of ptosis and swelling of the left eyelid and temporal region. Previous imaging revealed a left intraorbital occupying lesion, but a biopsy of the temporal subcutaneous tissue did not provide a definitive diagnosis. After 7 months, she presented with severe temporal swelling and purulent discharge. Upon examination, maxillary osteomyelitis resulting from caries of the upper jaw was observed. Treatment with oral antibiotics, drainage of the temporal skin fistula, and regular cleaning of the maxillary drainage improved her symptoms. Conclusion: This is a rare case of maxillary osteomyelitis associated with osteopetrosis, causing orbital inflammation. [ABSTRACT FROM AUTHOR]

We report a rare case of orbital inflammation complicating hemophagocytic lymphohistiocytosis (HLH) patient. HLH is a rare, life-threatening disorder characterized by uncontrolled activation of cytotoxic T lymphocytes, natural killer cells, and macrophages. A 37-year-old man known to have HLH, presented with a left periorbital swelling that was unsuccessfully treated as an orbital cellulitis, with intravenous antibiotics. A computed tomography (CT) scan of the orbits revealed inflammatory changes with no orbital collection or paranasal sinus disease. An orbital biopsy demonstrated lymphoplasmacytic infiltrations admixed with histiocytes. The patient deteriorated and was admitted to the intensive care unit. Ensuing blood results supported a diagnosis of HLH, and the patient responded well to subsequent immunosuppression. This case report highlights the importance of re-considering the diagnosis of orbital cellulitis in treatment resistant cases, particularly in the absence of sinus disease. To our knowledge, this is the third case of orbital inflammation associated with HLH patients. [ABSTRACT FROM AUTHOR]

Idiopathic orbital inflammatory disease is a benign, non-infectious, and non-neoplastic space-occupying orbital and periorbital inflammation with no identifiable local or systemic causes. Patients usually present with eyelid and periorbital erythema and edema, proptosis, and decreased eye movements. In this report, a case of large serous retinal detachment accompanying a unilateral idiopathic orbital inflammatory disease attack is discussed. It demonstrates that very extensive and highly serous retinal detachment may accompany idiopathic orbital inflammatory disease attacks. Dramatic improvement in clinical findings and imaging can be observed with high-dose steroid treatment in a short time. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the study was to report three cases of orbital inflammation following administration of the COVID-19 vaccination, manifesting as Tolosa–Hunt syndrome (THS) and orbital myositis. Method: A retrospective case series and literature review of patients who developed orbital inflammation following a COVID-19 vaccination. Results: One patient presented with Tolosa–Hunt syndrome (THS) 14 days following her third (booster) COVID-19 vaccination, one patient developed orbital myositis 10 days following his first COVID-19 vaccination and one patient developed recurrent orbital myositis 1 and 7 days following her second and fourth COVID-19 vaccination. All patients received the Comirnaty vaccine (Pfizer-BioNTech). A thorough systemic autoimmune disease workup in both patients was unremarkable. Two patients had a prior history of orbital inflammation, with previous involvement of other different orbital structures. Characteristic MRI features for each pathology were observed, supporting the clinical presentation of THS and orbital myositis. There was complete resolution of THS following corticosteroids, with no recurrence at 2 months. Meanwhile, one case of orbital myositis self-resolved at 2 months without use of systemic corticosteroids, while the other patient with orbital myositis required treatment with intra-orbital steroid injections and oral corticosteroids. Conclusion: Orbital inflammation has been recognised as a rare adverse effect following COVID-19 vaccination. We present a case series of THS and orbital myositis as varied presentations of this entity. [ABSTRACT FROM AUTHOR]

Idiopathic orbital myositis is a subgroup of idiopathic orbital inflammation of unknown etiology that primarily involves extraocular muscles. Orbital myositis most commonly affects young adults in the third to fourth decade of life with a female predilection. Imaging is an important diagnostic technique. Inflammatory changes in the tendon and its insertion point to idiopathic orbital myositis. Other inflammatory, endocrine, or neoplastic diseases must be ruled out. We report a case of a 12-year-old girl who presented to the ophthalmologic emergency department with spontaneously developing pain during horizontal eye movement in the left eye and nonspecific symptoms, such as light sensitivity and slightly blurred vision. Her symptoms were nonspecific and physical examination was not diagnostic. However, computed tomography and magnetic resonance imaging of the head revealed isolated, monolateral, and monomuscular lateral rectus myositis as a manifestation of idiopathic orbital inflammation. The patient rapidly responded to systemic corticosteroids. Diagnostic radiology plays a significant role in the unsuspected diagnosis of myositis. Radiologists must be aware of this rare presentation of idiopathic orbital inflammation in preteens.

Purpose: We report histopathologic orbital tissue analysis from three patients with thyroid eye disease (TED) – active, chronic, and post-teprotumumab to better characterize orbital cellular populations in these varying states of TED. Observations: Orbital tissues in TED demonstrate minimal lymphocytic infiltration in fat and Mueller's muscle. Following teprotumumab treatment, the tissues were devoid of lymphocytes with only perivascular cuffs of T-lymphocytes remaining in orbital fat. Conclusions and importance: In active TED, post-teprotumumab treatment, and in quiescent TED, orbital fat may not show significant inflammatory infiltration. More work is warranted to characterize specific cellular effects of teprotumumab and other biologics.

The aim of this paper was to report the case of a patient with orbital inflammation and dacryoadenitis after COVID-19 vaccination. During the COVID-19 pandemic, we noticed an increase of post-viral syndromes, both linked to infection and to vaccination. A 53-year-old male presented with proptosis, chemosis, hypotropia, and ophthalmoplegia of the right eye, 1 day after his COVID-19 booster shot. Anecdotally, he suffered similar symptoms after his initial two vaccinations. Idiopathic orbital inflammation and dacryoadenitis were diagnosed, and the patient was successfully treated with oral steroids. Orbital inflammation and dacryoadenitis after infection or vaccination are no new entities, but due to the scale of the current pandemic and the associated vaccination programs, these rare ocular diseases could be encountered more often.

Necrotizing dacryometaplasia (ND) is a rare entity which can mimic an aggressive malignant tumor. It seems to be analogous to necrotizing sialometaplasia which generally affects the salivary glands and is an inflammatory condition. There is scarce information about this entity as only two case reports have been reported in the last two decades. Due to the presence of adjacent bony erosion, it may mimic an aggressive malignant tumor. Here, we report a 27-year-old male who presented with right upper eyelid swelling for 2 months, which clinically and radiologically pointed to be a malignancy turned out to be ND.

Paraneoplastic neurologic syndromes (PNS) constitute a rare group of disorders whose optimal treatment is yet to be established. We report a patient with bilateral orbital inflammation associated with PNS, who responded well to surgical resection of the primary tumor. An 83-year-old woman was referred to our department for treatment of a progressive reduction in visual acuity and palpebral swelling in both eyes for the past 2 months. She was scheduled to undergo thoracic surgery for lung cancer. The best-corrected visual acuity (BCVA) in the right and left eye had worsened from 0.3 to 0.5 one month before she was referred to our department to 0.03 and 0.07, respectively. A slit-lamp examination revealed edema in both eyelids. Goldmann perimetry revealed several paracentral scotomas with constriction of the peripheral visual fields of both eyes, along with central absolute scotomas in V-4e isopter in the right eye. Magnetic resonance imaging revealed swelling of the bilateral extraocular muscles, which compressed the bilateral optic nerves at the orbital apex. Seven days after the resection of the lung cancer, the BCVA improved to 0.07 and 0.15 in the right and left eyes, respectively, without concomitant immunotherapy. Intravenous methylprednisolone (500 mg/day) was administered for 3 days to treat the residual orbital inflammation. Fourteen days after surgery, the BCVA further improved to 0.4 and 0.5 in the right and left eyes, respectively. Swelling of the bilateral extraocular muscles and the visual field abnormalities improved dramatically. Early diagnosis is crucial for the management of PNS.

Purpose: IgG4-related disease is an immune-mediated fibroinflammatory condition that can affect almost every major organ system. Orbital and adnexal involvement in IgG4-related disease though not uncommon can be varied depending on the site of the lymphoplasmacytic infiltration. This case of profound bilateral orbital inflammation is presented to demonstrate the significant clinical manifestations of IgG4-related ophthalmic disease. Observations: A 52-year-old man with a remote and seemingly unrelated history of lymphadenopathy presented to the Oculoplastics clinic with massive bilateral upper and lower lid swelling and induration. Hematologic testing demonstrated an elevation in serum IgG4 - a non-specific finding seen in various infectious, inflammatory, and malignant processes. Imaging demonstrated diffuse enlargement of orbital structures, including the lacrimal glands and extraocular muscles, as well as inflammatory changes of the adnexal and retrobulbar soft tissue. Orbital biopsy was required to confirm the diagnosis of IgG4-related ophthalmic disease. Conclusions and Importance: IgG4-related ophthalmic disease presents with various non-specific clinical signs and symptoms. The most common presentations include dacryoadenitis, enlarged orbital nerves, and orbital fat involvement; however, the extent of involvement both locally and systemically varies greatly. Clinical findings and imaging are helpful in narrowing the differential diagnosis; however, biopsy for histopathologic examination is essential to confirm IgG4-related disease. Physicians must maintain a high level of suspicion for the disease for proper management.

In rare cases, bisphosphonates are well established to cause ocular inflammation, presenting as uveitis, episcleritis, scleritis, orbital inflammation, and/or conjunctivitis. Some reports of bisphosphonate-associated neuro-ophthalmic complications also exist. We identified 101 reports in the literature relating to bisphosphonate-associated ocular complications. In a great majority of cases, symptoms resolve after discontinuation of the drug and anti-inflammatory treatment. Many cases recur if rechallenged with the same bisphosphonate. First-generation nonamino bisphosphonates, including clodronate and etidronate, are not associated with ocular inflammation. Only 2nd- and 3rd-generation amino bisphosphonates, including pamidronate, alendronate, risedronate, ibandronate, and zoledronate are associated with these complications. The mechanism of bisphosphonate-induced ocular inflammation may be related to activation of γ/δ T cells or M1 macrophages. Intravenous forms, such as pamidronate and zoledronate, tend to have higher rates and faster onset of ocular inflammation, generally presenting within days of infusion. In oral bisphosphonates, such as alendronate and risedronate, these complications present with more sporadic timing. Rates of complications are also higher when bisphosphonates are used for malignancy, as doses tend to be higher compared with doses for osteoporosis. [ABSTRACT FROM AUTHOR]

Thyroid eye disease (TED), an autoimmune inflammatory disorder of the orbit, presents with a potential array of clinical sequelae. The pathophysiology behind TED has been partially characterized in the literature. There remain certain elusive mechanisms welcoming of research advances. Disease presentation can vary, but those that follow a characteristic course start mild and increase in severity before plateauing into an inactive phase. Diagnosis and evaluation include careful physical examination, targeted laboratory work up, appropriate imaging studies, and tailored treatment regimens. Special consideration may apply to certain populations, such as pediatric and pregnant patients. [ABSTRACT FROM AUTHOR]

Anti-TNF-α agents (e.g. infliximab, adalimumab, etanercept) are effective management options in various inflammatory and autoimmune diseases (e.g. inflammatory bowel disease). The occurrence during anti-TNF-α agent therapy of a new onset or exacerbation of an inflammatory condition that usually responds to this class of drug has been termed a paradoxical adverse event (PAE). A wide range of ophthalmic PAEs have been reported including uveitis, optic neuritis/neuropathy, scleritis, orbital myositis, retinal vasculitis, and others. The patient reported herein developed a dramatic orbital inflammatory PAE during his infliximab infusions, which manifested as an acute orbital apex syndrome with vision loss. Physicians using this medication should be aware of this serious vision-threatening PAE, and urgent therapy with high dose intravenous corticosteroids may be required. [ABSTRACT FROM AUTHOR]

A 61-year-old man underwent left medial wall and floor fracture repair with a Suprafoil® implant. He had postoperative orbital congestion and lower eyelid swelling that persisted for over seven weeks. Examination demonstrated hyperglobus with supraduction, infraduction, and adduction deficits. Imaging revealed a 3.7 × 3.6 × 2.6 cm isodensity along the implant, thought to be hematoma. The patient elected to pursue exploration and possible drainage. Intraoperatively, there was no hematoma; rather, we found a fibroinflammatory rind along the periorbita surrounding the implant. This was biopsied, and the implant was removed, as the fractures had sufficiently healed. Pathology showed dense fibroconnective tissue with associated inflammation. The patient completed a steroid taper with improvement in all symptoms and resolution of diplopia. To our knowledge, this is the first reported case of such a prominent orbital inflammatory reaction to nylon foil, a departure from the delayed hematic cysts typically associated with these implants. [ABSTRACT FROM AUTHOR]

Orbital AA amyloidosis can induce a local inflammatory response, manifesting as orbital swelling, papillitis, posterior scleritis, choroiditis, and exudative retinal detachment. Choriocapillaris vasoocclusion with reactive inflammation to the presence of amyloid fibrils likely underlies this presentation. Purpose: To describe a case of amyloid A protein amyloidosis that produced an orbital inflammatory response with a novel presentation. Methods: Case report. Results: A 24-year-old Caucasian women with a history of intravenous heroin use was hospitalized for tricuspid valve endocarditis and methicillin-resistant Staphylococcus aureus bacteremia, as well as acute renal failure. She received hemodialysis and intravenous daptomycin and had negative blood cultures for 3 weeks, when she developed sudden bilateral orbital swelling and blurred vision. Visual acuity was 20/200 in the right eye and 20/400 in the left eye. Examination revealed proptosis, conjunctival chemosis and desiccation, optic disk swelling, creamy choroidal infiltrates, and inferiorly located exudative retinal detachments in both eyes. Multimodal imaging demonstrated thickening of the sclera, choroid, and choriocapillaris as well as outer retinal disruption, subretinal fluid, and deposits of hyperfluorescent debris within the choriocapillaris, outer retina, and vitreous. Oral prednisone at 60 mg per day resolved the choroidal infiltrates and exudative detachments. Persistent nephrotic syndrome called for a renal biopsy, which demonstrated amyloid A protein amyloidosis. Conclusion: Orbital and choroidal Amyloid A protein amyloidosis can induce a local inflammatory response manifesting as orbital swelling, papillitis, posterior scleritis, choroiditis, and exudative retinal detachment, which responds to steroid therapy. The underlying pathology is likely a reactive inflammatory, vasoocclusive process involving the choriocapillaris and orbital vasculature to the presence of amyloid fibrils. [ABSTRACT FROM AUTHOR]

Thyroid eye disease (TED) is a rare disease that can lead to decreased quality of life, permanent disfigurement, and vision loss. Clinically, TED presents with exophthalmos, periorbital edema, extraocular muscle dysfunction, and eyelid retraction, and can lead to vision-threatening complications such as exposure to keratopathy and dysthyroid optic neuropathy (DON). Over the last several years, significant advancements have been made in the understanding of its pathophysiology as well as optimal management. Ethnic variations in the prevalence, clinical presentation, and risk of vision-threatening complications of TED are summarized, and risk factors associated with TED are discussed. Additionally, significant advances have been made in the management of TED. The management of TED traditionally included anti-inflammatory medications, orbital radiation therapy, orbital surgical decompression, and biologic therapies. Most recently, targeted therapies such as teprotumumab, an insulin-like growth factor-1 receptor antagonist, have been studied in the context of TED, with promising initial data. In this review, updates in the understanding and management of TED are presented with a focus on the international variations in presentation and management.

Purpose: Porous orbital implants are commonly used materials following enucleation or evisceration. Implant-associated inflammation is a rare but serious complication which may necessitate explantation. Observations: We report a case of a patient who developed extensive orbital inflammation six months after implantation of a vicryl (polyglactin 910) mesh-wrapped Bioceramic (aluminum oxide) spherical implant. An orbital biopsy demonstrated an extensive fibroinflammatory reaction with multinucleated giant cells. Removal of the implant resulted in complete resolution of symptoms. Conclusions and importance: We surmise that the Bioceramic implant played a significant contributory role in this patient's orbital inflammation, a complication which has not been described previously.

A 31-year-old woman with no history of systemic autoimmune disease developed bilateral pain, redness, periorbital edema, and proptosis while being hospitalized for mild coronavirus disease 2019 (COVID-19) pneumonia. The onset of ocular symptoms occurred six days after systemic COVID-19 symptoms. Computed tomography examination of the orbit revealed diffuse inflammation involving multiple orbital structures. A complete systemic workup was performed to exclude other systemic inflammatory diseases. She was initially diagnosed with probable orbital cellulitis secondary to sinusitis. However, she did not improve with antibiotic therapy. Empirical administration of systemic corticosteroids led to a dramatic clinical response. The patient has been maintained on a gradual steroid taper without inflammation relapse. This article describes a potential causal association between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and orbital inflammation. Ophthalmologists should be aware of this presentation when assessing patients with COVID-19 who present with orbital inflammatory symptoms.

A 6-month-old female presented with bilateral periorbital edema for 7 days. Laboratory testing was significant for active SARS-CoV-2 infection. Neuroimaging demonstrated soft tissue changes within the bilateral orbits and enlargement of the bilateral lacrimal glands. Although the patient initially improved with corticosteroid treatment, she later returned with recurrent left periorbital and eyelid edema. Orbital biopsy was performed and demonstrated findings in the lacrimal gland and the adjacent fibroconnective tissues that are similar to those of prior lung specimens seen in SARS-CoV-2 patients. Final diagnosis was bilateral orbital inflammation with features presumed secondary to SARS-CoV-2 infection. To the best of our knowledge, this is one of the first reports to document bilateral orbital inflammation as a sign of SARS-CoV-2 infection in the pediatric population with the associated pathological findings. [ABSTRACT FROM AUTHOR]

The aim of this paper was to report the case of a patient with orbital inflammation and dacryoadenitis after COVID-19 vaccination. During the COVID-19 pandemic, we noticed an increase of post-viral syndromes, both linked to infection and to vaccination. A 53-year-old male presented with proptosis, chemosis, hypotropia, and ophthalmoplegia of the right eye, 1 day after his COVID-19 booster shot. Anecdotally, he suffered similar symptoms after his initial two vaccinations. Idiopathic orbital inflammation and dacryoadenitis were diagnosed, and the patient was successfully treated with oral steroids. Orbital inflammation and dacryoadenitis after infection or vaccination are no new entities, but due to the scale of the current pandemic and the associated vaccination programs, these rare ocular diseases could be encountered more often. [ABSTRACT FROM AUTHOR]

Background and purpose: Bisphosphonates are widely used, notably for osteoporosis treatment. Their common side effects are well known. However, they can trigger less common effects such as orbital inflammation. Here, the case is reported of an orbital myositis triggered by alendronate. Methods: This is a case report at an academic medical center. An orbital magnetic resonance imaging scan, a thoraco‐abdominal computed tomography scan and blood sample analyses were performed. Results: A 66‐year‐old woman treated by alendronate for her osteoporosis was investigated. She developed an orbital myositis after the first intake. Neurological examination revealed a painful diplopia with decreased downward and adduction movements of the right eye and edema of the upper eyelid. Orbital magnetic resonance imaging showed an orbital myositis of the right eye. No other cause of orbital myositis was found than the alendronate intake. After alendronate arrest and a short course of prednisone, the symptoms resolved. Conclusion: This case highlights that alendronate can cause an orbital myositis whose early diagnosis is of major importance because it is a treatable side effect. [ABSTRACT FROM AUTHOR]

Purpose: We report the case of a 32-year-old woman with orbital myositis prior to onset of Behcet's disease (BD). Observations: A 32-year-old woman was referred to our hospital for a complaint of right eyelid swelling, eye pain, and diplopia. Her best-corrected visual acuity was 20/32 on the right, and 20/16 on the left. She was diagnosed as idiopathic orbital inflammation, and received two pulses of intravenous administration of methylprednisolone, followed by oral prednisolone. Three months later, she developed bilateral orbital myositis, and received one more pulse therapy, followed by oral prednisolone and cyclosporin. About one year after the first visit, oral aphthae, genital ulcers, and folliculitis-like skin rash appeared, and the physician diagnosed incomplete type BD. The patient had no uveitis during the disease course. Orbital inflammation and systemic manifestations of BD were ultimately well controlled with small doses of prednisolone. Conclusion and importance: BD may develop during the course of orbital inflammation.