Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune disorders that cause inflammation and necrosis of small- to medium-sized blood vessels. It most commonly affects the kidneys and lungs, leading to complications such as rapidly progressive glomerulonephritis and pulmonary hemorrhage. (1) Ocular involvement occurs in up to 50% of patients with AAV, most commonly presenting as scleritis, episcleritis, or orbital inflammatory disease (also known as orbital pseudotumor). Other ocular findings may include keratitis, uveitis, or retinal vasculitis. (2)(3) Eyelid necrosis with destructive orbital inflammation, however, is extremely rare and poses a significant diagnostic challenge because its presentation can mimic infections, malignancies, or other inflammatory orbital conditions. Prompt recognition is critical to prevent irreversible tissue damage and vision loss. (4) We report the case of a 74-year-old Hispanic woman with multiple vascular risk factors who presented with recurrent pustules of the right upper eyelid that progressed to full-thickness eyelid necrosis, medial orbital mass effect, and corneal compromise. Initial management was directed toward presumed orbital cellulitis; however, orbital imaging revealed a destructive extraconal mass with mass effect and associated venous stasis. Sequential biopsies demonstrated extensive necrosis with histiocytoid granulomatous inflammation and focal features of chronic vasculitis. Negative stains and cultures excluded infectious and neoplastic causes. After thorough evaluation and exclusion of alternative etiologies, the findings were most consistent with ANCA-negative ANCA-associated vasculitis (AAV). The patient was treated with high-dose intravenous corticosteroids and rituximab induction, resulting in stabilization of orbital disease and preservation of vision. This case highlights the potential for destructive orbital vasculitis to masquerade as infection or malignancy and underscores the importance of considering AAV in atypical orbital presentations. Prompt recognition and initiation of immunosuppressive therapy are critical to prevent irreversible morbidity.

Non-specific orbital inflammation (NSOI), formerly known as idiopathic orbital inflammatory disease (IOID), is a rare, exclusion-based orbital disorder with diverse clinical manifestations. This study evaluates the demographic patterns, clinical features, histopathological profiles, and treatment outcomes of NSOI cases managed at a tertiary referral centre in Malaysia. A six-year retrospective review was conducted at a tertiary referral centre in northern Malaysia, involving 36 patients diagnosed with NSOI between January 2018 and December 2023. Diagnosis was based on clinical features, exclusion of systemic and infectious causes through serology, and supportive imaging or biopsy findings. Only cases with histopathological confirmation and immunohistochemical staining negative for lymphoma, carcinoma, and other malignancies were included. Data included demographics, clinical presentation, imaging and histopathological findings, serologic evaluations, and treatment modalities. Outcomes were assessed based on symptom resolution, radiologic improvement, recurrence, and treatment response. Most patients were male (61.1%), with a mean age of 43.6 years. Unilateral involvement predominated (77.8%). Common presentations included periorbital swelling (69.4%), ophthalmoplegia (22.2%), conjunctival mass (22.2%), and proptosis (19.4%). Imaging revealed frequent involvement of the lacrimal gland (45.8%), extraocular muscles (37.5%), and conjunctiva (37.5%). The main histopathological findings included reactive lymphoid hyperplasia (40%), granulomatous inflammation (20%), and chronic inflammation (23.3%). Of the 36 patients, 19 received medical treatment, with 84.2% given systemic corticosteroids, while the remaining 17 patients were managed conservatively without any medical treatment, and they remained clinically stable throughout follow-up with no evidence of disease progression. Among treated cases, recurrence occurred in 25%, predominantly in males. NSOI shows varied clinical and anatomical patterns. Corticosteroids remain the mainstay of treatment, but conservative management is appropriate in stable, nonprogressive cases when close monitoring and diagnostic exclusion are assured. These findings support individualised therapeutic strategies and long-term followup.

Immunoglobulin-G4 Related Disease (IgG4-RD) is a group of multi-system, fibro-inflammatory conditions characterised by elevated IgG4 levels and unique histopathological features. Clinical presentations of the disease are highly variable, albeit there exist distinct phenotypes in the presentations of the disease. IgG4-RD is a differential diagnosis that should be missed in any case of chronic inflammatory and tumefactive pathology. We present the case of a 47-year-old diabetic female who presented with features suggestive of acute pyelonephritis. On further evaluation, our findings unmasked a rare constellation of pancreatitis, orbital pseudotumor, and venous thrombosis. After an astute clinical observation by our team, we were able to decipher these as features of distinct phenotypes under the IgG4-RD spectrum with minimal overlap (pancreato-hepatobiliary disease, head and neck disease, and retroperitoneal disease groups, respectively). We have presented an extensive review of the recent literature pertaining to the etiopathogenesis, clinical features, diagnostic protocol and management consensus for IgG4-related disease. A detailed account of the possible clinical presentations and work-up strategy for suspected patients has been emphasised in particular. IgG4-RD is diverse clinico-pathological entity that warrants a high degree of clinical suspicion. Once identified, efforts must be made to screen all possible foci of disease activity characteristic to the disease, as a multi-focal disease presentation of IgG4-RD is not unlikely.

Analysis of the pathological composition ratio of orbital space-occupying lesions in 7 515 cases: single-center retrospective study.

IntroductionThyroid-associated eye disease (TAED) is commonly linked to the presence of thyrotropin receptor antibodies (TRAb). However, this case presents an unusual occurrence of TAED in a patient with long-standing Hashimoto’s thyroiditis, demonstrating unilateral orbital involvement despite negative TRAb antibodies. This raises questions about other contributing factors beyond TRAb presence, such as chronic hypothyroidism and poor medication adherence.Rare cases of TAED in hypothyroid patients challenge the conventional understanding of its pathogenesis. This case highlights the possibility of alternative mechanisms influencing orbital involvement.Clinical CaseA 48-year-old male, Mr. H.M., with a 25-year history of Hashimoto’s thyroiditis-induced hypothyroidism, was referred by a neurologist in June 2023 for reassessment of thyroid function. He reported experiencing diplopia since early 2022.Orbital imaging—including CT scan and MRI—revealed homogenous enhancement and enlargement of the right inferior rectus muscle, as well as retrobulbar heterogeneous fat enhancement and enlargement. Initially suspected to be an orbital pseudotumor or, less likely, thyroid-associated eye disease (TAED), the patient underwent a comprehensive workup for potential pseudotumor etiologies such as IgG4-related disease, sarcoidosis, and lupus. All investigations returned negative results, leading to the exclusion of pseudotumor and confirming TAED.The patient was subsequently referred to an ophthalmologist, who diagnosed unilateral right proptosis with mild inactive orbitopathy. Conservative management was advised, including artificial lubricants, the use of sunglasses, and optimization of thyroid function through close follow-up.Two months later, the patient returned with improved thyroid function, reflected by a reduction in TSH levels to 13.6 uIU/ml. He reported better vision, though mild diplopia persisted. At the six-month follow-up, the patient demonstrated good adherence to thyroxine therapy, achieving well-controlled thyroid function with normalized TSH levels. Despite overall improvement in his general condition, mild diplopia remained. The ophthalmologist subsequently recommended surgical intervention for further management.The absence of TRAb in a patient with TAED presents an interesting anomaly. This case suggests that poor thyroid control and non-adherence to treatment may contribute to the development of orbital involvement, possibly through inflammatory pathways independent of TRAb. Further research is warranted to understand the potential mechanisms and alternative risk factors influencing TAED.ConclusionThis report highlights the importance of considering TAED in hypothyroid patients, especially those with chronic disease and poor compliance. Clinicians should remain vigilant for atypical presentations and explore factors beyond TRAb when diagnosing and managing orbital complications in thyroid disorders.Figure 1:Thyroid eye disease

Transpalpebral exenteration is indicated in cases of neoplasia, infection or severe trauma involving the globe and orbit. Limited peer-reviewed literature exists on the indications, complications and outcomes associated with this procedure in dogs. This retrospective study analysed medical records of 35 dogs undergoing transpalpebral exenteration at three UK veterinary referral centres between 2003 and 2023. The data included signalment, diagnostic findings, surgical details and postoperative complications. Kruskal‒Wallis and Fisher's exact tests were used to analyse relationships between clinical variables and complications. Orbital neoplasia was the most common indication (54.3%, 19/35), followed by orbital inflammatory disease (25.7%, 9/35), orbital cyst (11.4%, 4/35), orbital foreign body (5.7%, 2/35) and orbital pseudotumour (2.9%, 1/35). Immediate postoperative complications (<2 days) occurred in 60% (21/35) of cases, with surgical site swelling being the most frequent (90.5%). Short-term (2‒21 days) and long-term (>21 days) complications occurred in 20% (7/35) and 5.7% (2/35) of cases, respectively. Six patients (17.1%) required additional treatment. No significant correlations were found between the independent variables and complication rates. This study is limited by its retrospective nature and the small sample size. Transpalpebral exenteration is a safe procedure with primarily minor postoperative complications that typically resolve without intervention.

&lt;b&gt;Introduction:&lt;/b&gt; Non-specific orbital inflammation (NSOI), or orbital pseudotumor, is the third most common orbital disease, yet its pathogenesis remains poorly understood. A dysregulated immune response to infections or trauma, including surgery, has been proposed as a contributing factor. However, reports of NSOI coexisting with sinusitis are scarce and mostly limited to isolated case reports.&lt;b&gt;Aim:&lt;/b&gt; This study examines a subset of NSOI patients with coexisting chronic rhinosinusitis with nasal polyps (CRSwNP), aiming to explore potential pathophysiological links and evaluate treatment outcomes.&lt;b&gt;Materials and methods:&lt;/b&gt; Between 2014 and 2024, 34 patients with NSOI were assessed at the Medical University of Bialystok. In 7 cases (20%), NSOI was associated with CRSwNP. Diagnosis was confirmed through orbital tissue biopsy and exclusion of systemic diseases.&lt;b&gt;Results:&lt;/b&gt; Orbital symptoms - diplopia, proptosis, and pain - developed after sinonasal symptoms and surgical intervention in all cases. Early bacteriological cultures showed various pathogens, but intraoperative cultures during remission were sterile. Tissue or blood eosinophilia, along with recurrent exacerbations and signs of bacterial infection, suggested a mixed type 2/type 3 inflammatory endotype. Imaging revealed orbital wall defects with new bone formation (osteogenesis), and histopathology showed marked fibrosis. Treatment with corticosteroids led to partial stabilization in four patients; two required immunosuppressants and two underwent radiotherapy. None achieved complete remission.&lt;b&gt;Conclusions:&lt;/b&gt; These findings suggest that chronic rhinosinusitis may trigger or worsen NSOI in susceptible individuals, particularly those with underlying immune dysregulation. Standard treatments are often ineffective in advanced fibrotic stages, highlighting the need for tailored, multidisciplinary approaches and further research into the underlying mechanisms.

Background: Somatic gain-of-function (GOF) mutations in STAT5B, particularly N642H, are well-established in hematologic malignancies, such as leukemia and lymphoma, often presenting with significant eosinophilia.A distinct phenotype involving nonmalignant manifestations has been rarely reported.This phenotype is characterized by early-onset hypereosinophilia, urticaria, dermatitis, and diarrhea.Case Presentation: We report the case of a 9-year-old boy with a history of early infancy hypereosinophilia, severe asthma, atopic dermatitis, and food allergies.He later developed lymphadenopathy, hepatosplenomegaly, subcutaneous nodules, orbital pseudotumor and failure to thrive, with growth parameters consistently below the 3rd percentile.His short stature was investigated and was attributed to growth hormone deficiency.Growth hormone replacement therapy failed to improve his height.The patient is the second child of non-consanguineous parents, with two healthy siblings and a family history of stomach cancer.Laboratory findings revealed, leukocytosis (19,000-68,000/ L), lymphocytosis (12,600-22,700/L) with eosinophilia (1500-38,000/L), serum IgE levels exceeding 5000 IU/ml.Lymphocyte immunophenotyping revealed lymphocytosis with dominant CD3CD8 cells.Esophagogastroduodenoscopy showed significant eosinophilic infiltration throughout the GI system.The whole exome sequencing identified a heterozygous STAT5B N642H missense mutation.He was managed initially with prednisone 4 mg/kg/day tapered based on clinical response then shifted to IL-5 antagonist monoclonal antibody Mepolizumab 100 mcg monthly.The patient showed clinical improvement, with resolution of lymphadenopathy, hepatosplenomegaly,orbital pseudotumor, subcutaneous nodules, and a marked reduction in hospitalizations and eosinophil count (200-600/L).Discussion: This case expands the clinical phenotype of STAT5B N642H heterozygous mutation to include hepatosplenomegaly, lymphadenopathy, subcutaneous nodules, orbital pseudotumor, and growth hormone deficiency.We consider the mutation a somatic gain of function based on the previous reported pathologies related to STAT5B N642H mutation.However, the growth hormone deficiency and the failure to respond to growth hormone replacement urge for further study to identify the impact of STAT5B N642H mutation on the protein function in our patient.Conclusion: This report highlights the diverse phenotypic presentations associated with the STAT5B N642H mutation.It also showed for the first time that Mepolizumab controls disease activity.Expanding knowledge about STAT5B mutations will contribute to improved recognition, management, and potentially targeted therapies for affected patients.

Idiopathic orbital inflammatory disease (IOID) is a non-specific orbital inflammatory condition that is uncommon in children. Due to limited data and the unique characteristics of the developing immune system in children, this study examined the clinical features, imaging subtypes, treatment outcomes, and recurrence patterns. Pediatric patients (≤ 18 years) diagnosed with IOID at Beijing Tongren Hospital, Capital University between January 2020 and January 2025 were included in this retrospective review. Clinical data, imaging characteristics, treatment regimens, and recurrence patterns were collected. Cases were further classified into six anatomical subtypes based on orbital imaging. A total of 10 patients (12 eyes) were included, with a mean age of 9.9 ± 3.2 years. Imaging subtypes included diffuse (5, 50%), posterior scleritis (3, 30%), dacryoadenitis (1, 10%), and orbital apex involvement (1, 10%). Clinical symptoms were conjunctival injection (9, 90%), optic disc edema (8, 80%), decreased visual acuity (8, 80%), eyelid swelling (7, 70%), periorbital pain (6, 60%), ocular pain (4, 40%), and ptosis (4, 40%). Orbital MRI revealed posterior scleral and/or Tenon's capsule thickening with enhancement (8, 80%), optic nerve or sheath enhancement (5, 50%), lacrimal gland enlargement (4, 40%), and cavernous sinus thickening or dural enhancement (2, 20%). All patients received systemic corticosteroids, with a mean follow-up duration of 17.9 ± 12.5 months. Nine patients experienced recurrence, with seven of them related to steroid dependence in diffuse-type cases. Final visual acuity was ≥ 0.3logMAR in all relapsed cases. Pediatric IOID frequently presents with diffuse or deep orbital involvement on imaging. While corticosteroid therapy is generally effective, these subtypes may be more prone to steroid-dependent relapse during tapering. The association between steroid tapering and relapse remains to be fully substantiated, and further studies are needed to explore this potential relationship. Imaging-based classification may help predict recurrence risk and guide individualized long-term management strategies for pediatric IOID.

Rationale:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare autoimmune condition that can involve various organ systems. Ocular involvement, though uncommon, may present as the initial or even sole manifestation. Orbital inflammatory pseudotumor, particularly its sclerosing subtype, can pose significant diagnostic and therapeutic challenges due to its steroid dependence and potential for recurrence.Patient concerns:A 72-year-old Chinese man presented with a 6-month history of recurrent headaches. He later developed bilateral exophthalmos and progressive vision loss despite initial treatment. His past medical history included type 2 diabetes mellitus.Diagnoses:The patient initially tested positive for both p-ANCA and c-ANCA, with markedly elevated anti-myeloperoxidase and proteinase 3 antibody levels. Imaging revealed bilateral orbital masses consistent with orbital inflammatory pseudotumor, and biopsy confirmed sclerosing orbital inflammatory pseudotumor. Despite serological remission, clinical symptoms worsened during steroid tapering, suggesting steroid dependence.Interventions:The patient was initially treated with high-dose corticosteroids and oral cyclophosphamide. Although symptoms improved transiently, relapse occurred upon tapering steroids. Rituximab (RTX; 500 mg weekly for 4 weeks) was then administered as second-line therapy.Outcome:The patient experienced significant improvement following RTX therapy, which allowed successful steroid tapering. No further relapse occurred during short-term follow-up.Lessons:This case illustrates the limitations of cyclophosphamide-based regimens in managing steroid-dependent ANCA-associated orbital disease. RTX may be a more effective alternative in relapsing or refractory cases, particularly when ocular symptoms persist despite ANCA seronegativity. Early recognition and individualized immunosuppressive therapy are essential for preserving vision and improving outcomes in ocular ANCA-associated vasculitis.

Hyalohyphomycosis is an opportunistic infection of the subcutaneous tissue caused by hyaline fungi Hyphomycetes that form septate hyphae within tissue. Immunocompromised states and tropical climates are the main predisposing factors. Rhino-orbital hyalohyphomycosis involving the paranasal sinuses and orbit in an immunocompetent host is a very rare presentation. We report the case of a 27-year-old male crop surveyor who presented with left-sided proptosis and double vision. Based on clinical evaluation and imaging studies-contrast enhanced tomography CECT and magnetic resonance imaging MRI of the paranasal sinuses and orbits - the findings were suggestive of an orbital pseudotumour or fungal mass such as mucormycosis. The patient underwent endoscopic sinus surgery with excision of the orbital mass via Denkers approach under general anaesthesia. Histopathological examination confirmed the diagnosis of hyalohyphomycosis. Postoperatively the patient was treated with anti-fungal medication Itraconazole and advised regular nasal saline irrigation. Although rare in immunocompetent individuals rhino-orbital hyalohyphomycosis should be considered among the differential diagnoses for unilateral intraorbital mass or proptosis.

Dependence is an unusual, but potentially serious complication of corticosteroid use. Two cases of prednisone dependence are reviewed; the first involving a patient with inflammatory orbital pseudotumor who developed a dependent pattern of prednisone use that persisted years after the resolution of the acute episode. The second case involves a patient with factitious disorder who attempted to obtain corticosteroids from multiple clinicians, despite the absence of any clinical indication for them, manipulating elements of her medical history to influence the prescription of corticosteroids. Both patients exhibited tolerance and withdrawal symptoms, and both developed serious systemic effects from the corticosteroids, including cataracts, diabetes mellitus, and cushingoid signs. Both patients also exhibited dependence upon other substances. A MEDLINE search was conducted, and the case literature regarding corticosteroid dependence was reviewed. Twenty-six cases of potential corticosteroid dependence were identified, and 22/26 (85%), retrospectively, met criteria for DSM-IV substance dependence involving corticosteroids. Prednisone was the most frequently implicated corticosteroid, but cases involving ACTH, cortisone, and high-dose inhaled dexamethasone and beclomethasone were also identified. Asthma was the most common underlying medical condition for which the corticosteroids were prescribed, but a variety of allergic and inflammatory conditions were also reported. Corticosteroids may induce dependence based on their propensity to induce euphoria as well as a characteristic withdrawal syndrome, in addition to directly influencing reward circuitry. Clinicians should be aware of the possibility of prednisone dependence when confronted with patients who exhibit vigorous insistence on corticosteroids out of proportion to objective signs and symptoms of inflammation.

ObjectiveTo quantitatively evaluate the degree of fibrosis in Idiopathic Orbital Inflammatory Pseudotumor (IOIP) using Intravoxel Incoherent Motion Diffusion-Weighted Imaging (IVIM-DWI).MethodsTwenty eight patients (32 eyes) with idiopathic orbital inflammatory pseudotumor were pathologically diagnosed in the Affiliated Hospital of Yunnan University from August 2019 to August 2024. Routine orbital MRI plain scan and ivim-dwi scan were completed before surgery, and the true diffusion coefficient (d), pseudo diffusion coefficient (d*) and perfusion fraction (f) were obtained. According to the proportion of chronic inflammatory cells and fibrous tissue components in postoperative histopathological sections, it was defined that fibrous cells accounted for less than 10% of the total number of cells as negative (–), and > 10% as positive (+), of which 10%%25% were +, 25%%50% were + +, 50%%75% were + + +, and > 75% were + + +. Taking the proportion of fibrocytes in histopathological sections as the standard of fibrosis degree, the correlation between ivim-dwi parameters and the proportion of fibrocytes in pathological sections was analyzed. Data were analyzed by SPSS 19.0 statistical software package, and P < 0.05 was considered statistically significant.ResultsAmong 28 cases (32 eyes) with idiopathic orbital inflammatory pseudotumor, there was no statistical difference between gender, age and the degree of IOIP fibrosis (P > 0.05). There was a statistically significant difference between the proportion of IOIP fiber composition and the D value and F value in IVIM-DWI parameters (P < 0.05), and the sensitivity of F value was higher than D value in the comparison of the two parameters combined with ROC curve analysis; There was no statistical difference between the D * value and the degree of fibrosis (P > 0.05).ConclusionThe D value and F value of IVIM-DWI showed a correlation with the proportion of IOIP fiber components, and the sensitivity of F value was higher than D value. Ivim-dwi examination parameters can be used as quantitative and objective indicators to evaluate the degree of fibrosis of lesions before IOIP.

Orbital pseudotumor is a non-infectious and benign inflammatory condition of the orbit and the surrounding tissues. This case report aims to emphasize the importance of understanding the cause-effect relationship between orbital inflammation and raised intraocular pressure (IOP). An 18-year-old girl was previously diagnosed with primary angle-closure glaucoma and had a history of twice trabeculectomy surgeries done elsewhere two months before she came to our hospital. She came with left eye pain, eyelid edema, ptosis, conjunctival chemosis, ophthalmoplegia, and elevated IOP despite a positive seidel test for a leaking trabeculectomy bleb. Five months prior to her first surgery she realized an inward squint on her left eye. She also felt joint pain for a year before the ophthalmic symptoms. After comprehensive diagnostics, she was diagnosed with left eye orbital pseudotumor and secondary glaucoma with spondyloarthropathy. Daily 30 mg of oral prednisone was given to reduce the inflammation. After a week, her conditions improved with decreased IOP although there was no additional anti-glaucoma medication. The raised IOP in orbital pseudotumor is often caused by the swelling of orbital tissue and extraocular muscles, that result in choroidal circulation problems from the superior and inferior ophthalmic veins compression. The IOP was greatly reduced once the inflammation was treated Diagnosing orbital pseudotumor may be challenging since it can mimic various conditions. Secondary glaucoma should be acknowledged as a complication in patients with orbital pseudotumor. Once orbital pseudotumor is diagnosed, a systemic steroid should be given to manage the inflammatory conditions as well as resultant glaucoma.

Relevance Orbital involvement is typical in granulomatosis with polyangiitis (GPA) [1–5]. Determining the activity of the inflammatory process in the orbit is key to planning treatment, the purpose of which is to maintain long-term remission. Objective To study the capabilities of ultrasound in determining the stage of orbital inflammation in patients with GPA. Material and methods The study included 24 patients with diffuse orbital involvement in GPA. Among them, there were 8 men (33.3%) and 16 women (66.7%). Local disease was observed in 58.3% (14/24) of patients, and systemic disease in 41.7% (10/24). Group 1 (active stage) included 9 patients, and Group 2 (inactive stage) included 18 patients. Results When conducting an ultrasound examination in the 1st group, we identified color maps of vascular flows in the pathological focus with an active process. When constructing two-dimensional histograms in the 1st group, low acoustic density was detected, on average in the group it was 54 ± 13.1 CU. In 4 patients, the vascularization index was assessed, its value was on average 9.2%. In the 2nd group, there was no color map of the vascular flow inside the foci. A densitometric study revealed low acoustic density, on average 61.7 ± 15.2 CU. The difference in this indicator between the groups is statistically significant (p &lt; 0.05). The vascularization index was determined in 7 patients and on average amounted to 0.1%. Conclusion The ultrasound picture in orbital GPA differs depending on the stage of the inflammatory process, which allows using this non-invasive and accessible technique to assess the activity of the inflammatory process. Key words: granulomatosis with polyangiitis; orbital pseudotumor; inflammatory activity; ultrasound diagnostics

Abstract Introduction: Sarcoidosis is a multisystem disease marked by the formation of non-caseating granulomas, primarily affecting the respiratory system. Ocular involvement is common, with uveitis being the most prevalent manifestation. While optic nerve involvement is rare, occurring in 1%-5% of cases, it can lead to significant visual impairment. This case report details a patient with sarcoidosis complicated by neurosarcoidosis and pulmonary cavitary sarcoidosis. Case Presentation: A 65-year-old man with no significant medical history presented with sudden vision loss in his right eye, which progressed to blurry vision upon admission. He reported shortness of breath, a dry cough, and a 30-pound weight loss over the past year. His vital signs were stable. On neurological examination, his right eye vision was 20/70, and his left eye vision was 20/40, with a NIHSS score of 0. His CRP was 1.7 mg/dL, the rheumatologic panel was negative, and his ACE levels were normal. A brain MRI showed right retro-orbital fat stranding with adjacent edema suggestive of orbital pseudotumor. A CTA of the chest revealed multifocal infiltrates, multiple pulmonary nodules, and a large cavitary lesion in the left upper lobe (Figure 1). Furthermore, a flexible bronchoscopy with EBUS and a transbronchial biopsy of the 10L hilar node, along with a lumbar puncture, were inconclusive. A CT-guided core biopsy of the right lower lobe revealed non-necrotizing granulomatous inflammation consistent with sarcoidosis (Figure 2). The patient was started on prednisone 40 mg daily as an outpatient. During a follow-up visit six weeks later, the patient reported significant improvement in vision. Discussion: Pulmonary sarcoidosis involves an inflammatory process with lymphocytes and giant cells leading to granuloma formation. Primary cavitary sarcoidosis (PCS) is exceedingly rare, occurring in less than 0.5% of cases. In PCS, lung granulomas may undergo necrosis, forming cavities that mimic infections or malignancy. Theories explaining cavities in sarcoidosis include bullae from airway obstruction, secondary infections, extrusion of necrotic material, and cystic bronchiectasis. Ocular involvement affects 30%-50% of patients, primarily as uveitis, while optic neuritis is rare (1%-5%) and often results in poor visual outcomes. Mechanisms for optic nerve involvement include granulomatous infiltration and compression Conclusion: This case illustrates the rare combination of neurosarcoidosis and primary cavitary pulmonary involvement. A comprehensive evaluation is crucial for cavitary lung lesions, which can have various causes. Early diagnosis of optic nerve involvement in sarcoidosis is vital to prevent progressive damage and enhance outcomes. Figure: Non-caseating granulomas

Abstract Idiopathic orbital inflammation is the third most common orbital affection, characterized by a benign inflammatory process, which is neither infectious nor neoplastic and can affect any orbital tissue. A 31-year-old female patient was referred to neurosurgical evaluation due to left orbital pseudotumor, which was nonresponsive to high doses of corticosteroid therapy. During the neurological examination, we identified reduced visual acuity, complete external ophthalmoplegia, ocular pain, and severe proptosis. A neurosurgical intervention with lateral orbitotomy was performed, with great pain relief. In the present case report, we discuss the disease and its natural course and call the attention of neurosurgeons to prompt intervention in selected cases.

PurposeTo evaluate the clinical features, pathological findings, treatment, and prognosis of patients with orbital natural killer/T-cell lymphoma (NKTCL).MethodsThis retrospective study collected clinical data from 11 patients diagnosed with orbital NKTCL by pathological examination at Beijing Tongren Hospital, Capital Medical University, between November 2005 and March 2024. The patients were followed up to assess their prognosis.ResultsThe median age of patients with orbital NKTCL was 51 years, with a slight male predominance. All cases were unilateral, with clinical presentations of eyelid or periorbital swelling, and other symptoms including decreased vision (90.9%), conjunctival congestion (90.9%), ocular motility disorders (81.8%), proptosis (63.6%), and ptosis (36.4%). All patients had surrounding tissue involvement, with the most common sites being the eyelid (11 cases), eyeball wall (7 cases), and lacrimal gland (5 cases). Additionally, 63.6% of patients had concurrent soft tissue involvement of the nasal region, while 36.4% had isolated orbital NKTCL. Pathological examination revealed diffuse proliferation and infiltration of atypical lymphocytes, occasionally with necrosis. Immunohistochemical staining showed positive expression of GrB (11 cases), CD3 (10 cases), TIA1 (9 cases), CD56 (8 cases), and EBV/EBER (10 cases). All patients underwent ocular surgery, 7 received postoperative chemotherapy, and one received concurrent local radiotherapy. Follow-up (median duration: 120 months) revealed poor prognosis, with 5 patients lost to follow-up, 5 deaths, and only one survival without recurrence.ConclusionsOrbital NKTCL is highly aggressive, rapidly progressive, and has a poor prognosis. Its clinical symptoms are similar to orbital cellulitis or inflammatory pseudotumor. When anti-inflammatory and corticosteroid therapies prove ineffective, early biopsy for histopathological confirmation is strongly recommended. Upon diagnosis, prompt chemotherapy and radiotherapy should be initiated to improve the patient’s prognosis.

Orbital pseudotumor is a rare benign non-infective inflammatory condition of the orbit without any identifiable local or systemic causes. It has various clinical manifestations. The clinical diagnosis is based on the exclusion of orbital neoplasm, infections, and orbital manifestation of systemic illness. We report the case of a 49-year-old female presented to Popular Medical College and Hospital with the complain of progressive deteriorating symptoms of her right eye, including pain, swelling, blurring of vision, and headache without having any history of trauma or recent infection or flare up of any systemic illness. Examination of eye showed erythema, peri-orbital oedema, drooping of upper eye lid with restricted eye movement in right eye. Magnetic resonance imaging revealed orbital soft tissue enhancement. A diagnosis of orbital pseudotumor was made on the basis of inflammatory markers and Magnetic Resonance Imaging findings. She was treated with oral corticosteroids. J MEDICINE 2025; 26: 74-76

Abstract Background Ocular extraintestinal manifestations (O-EIM) of inflammatory bowel disease (IBD) are rare, with most being attributed to scleritis, episcleritis and uveitis. Orbital myositis, however, is an exceedingly rare manifestation that is described as acute or chronic inflammation of one or more extraocular muscles leading to symptoms related to the mass effect including orbital pain, swelling, ophthalmoplegia, proptosis, and diplopia. To date, 3 reports of varying ocular myositis preceding IBD diagnosis have been described in pediatrics. Aims This report describes a case of unilateral orbital myositis, which was the initial and only symptom of newly diagnosed adolescent with Crohn’s disease (CD). Methods A chart review was conducted and placed into the context of this rare presentation. Results 15-year-old female presented with acute 6th cranial nerve palsy with ipsilateral optic disc edema, raising concern for raised intracranial pressure. Magnetic resonance imaging (MRI) revealed a retro-orbital mass on the left lateral rectus muscle, consistent with orbital pseudo tumor and myositis, no intracranial tumor or vascular lesion. She was treated with 3-month course of prednisone. An inflammatory condition was suspected based on the unusual presentation of orbital myositis which responded well to prednisone. Alternative infectious or rheumatologic causes of orbital pseudotumor were excluded and the patient was treated with corticosteroids prior to gastroenterology (GI) consultation. She was found to have elevated C-reactive protein (CRP), hypoalbuminemia and anemia at presentation. Fecal calprotectin (649)) was subsequently found to be positive at GI consultation after corticosteroid treatment initiation. Given the patient did not exhibit any GI signs or symptoms at the time of the evaluation, further GI investigations were deferred to after completion of steroids. Fecal calprotectin was persistently elevated (1463) after resolution of ocular symptoms and completion of steroids. Magnetic resonance enterography (MRE) identified inflammation of the terminal ileum. A definitive diagnosis of CD was established following upper GI endoscopy and colonoscopy with biopsies. Conclusions Orbital myositis is a rare ocular manifestation of CD. Early recognition and treatment are critical as they may precede the development of overt gastrointestinal symptoms, potentially delaying diagnosis if not considered. Given the rarity of this presentation, further studies are needed to better understand the pathophysiological link between CD and orbital myositis. Funding Agencies None