Search Results (69)

Background and Clinical Significance: Immune checkpoint inhibitors (ICIs), a revolutionary class of oncology therapeutics that enhance T cell-mediated antitumor immunity, are associated with various immune-related adverse events (IRAEs). While destructive thyroiditis and hypothyroidism are common, ICI-induced Graves’ disease (GD) is exceedingly rare, and the occurrence of concomitant Graves’ ophthalmopathy (GO) is even rarer. Case Presentation: A 57-year-old man with bladder cancer developed GO after receiving the first dose of the programmed death 1 (PD-1) inhibitor pembrolizumab. He presented with severe proptosis, extraocular muscle enlargement, hyperthyroidism, and significantly increased thyroid-stimulating hormone receptor autoantibodies (TRAb). Following the treatment with glucocorticoids and immunosuppressive therapy, his symptoms improved markedly but relapsed upon dosage reduction. To date, we have not identified any previous reports of GO with confirmed positive thyroid-related antibodies induced by pembrolizumab. Conclusions: This case offers valuable insights into the potential IRAEs, underscoring the importance of thorough clinical evaluation and early recognition to improve patient outcomes and quality of life. A literature review of ICI-induced GO was also performed, with further discussion of the potential pathogenic mechanisms, risk factors, and management strategies. Full article

This case-based review examines the spectrum of leukemic ocular involvement, focusing on its prognostic implications. A rare case of relapsed acute myeloid leukemia (AML) in a 63-year-old man is presented, featuring simultaneous orbital proptosis, adnexal involvement, choroidal and retinal infiltration, and hemorrhagic changes affecting both the anterior and posterior segments. This constellation of findings, affecting multiple ocular structures concurrently, highlights the eye’s potential role as a sanctuary site for leukemic cells and underscores the diagnostic challenge of distinguishing direct infiltration from treatment-related or secondary vascular damage. This case, integrated with a literature review, emphasizes that multifocal ocular signs may serve as early indicators of leukemic relapse and reinforce the need for close collaboration between ophthalmologists and hematologists in guiding patient management. Full article

Acute rhinosinusitis in children is typically caused by viral or bacterial infections. However, methicillin-resistant Staphylococcus aureus (MRSA) is increasingly linked to recurrence and severe complications, including orbital involvement. We present a case of a 6-year-old boy with periorbital swelling, proptosis, and fever following upper respiratory symptoms. Imaging revealed pansinusitis with a subperiosteal orbital abscess. He was treated empirically with intravenous ceftriaxone and metronidazole, followed by endoscopic sinus surgery. Intraoperative cultures confirmed MRSA, leading to a switch to vancomycin. The patient recovered fully without complications. This case illustrates the clinical challenges posed by MRSA sinusitis and emphasises its public health implications. Integrating antimicrobial stewardship, promoting community hygiene, ensuring early diagnostics and healthcare access, and enhancing epidemiological surveillance can play a pivotal role in reducing the burden of MRSA-related complications in children. Full article

Background/Objectives: We aimed to evaluate the demographic characteristics, clinical findings, and survival outcomes of patients diagnosed with orbital metastasis, considering primary tumor type, age, and gender variables. Methods: In this observational study, demographic data, tumor localization, histopathological diagnoses, and survival times of 83 patients followed for secondary orbital metastasis at Çukurova University Ophthalmology Department between 2003 and 2023 were retrospectively reviewed. Subgroup analyses were performed according to age (<18 and ≥19), gender, and primary tumor groups. Results: The study included 83 patients (51 (61.4%) females and 32 (38.6%) males). The mean age at diagnosis was found to be 40.8 ± 24.6 years. A total of 24.1% of the cases were in the pediatric age group (mean age 5.9 years), and the most common tumor metastasizing to the orbit in this group was neuroblastoma (80%). In adult patients, the two most frequent tumors metastasizing to the orbit were breast cancer (33.3%) and lung cancer (14.3%). The most common clinical findings were proptosis (32.5%) and blurred vision (26.2%). Orbital metastases were observed more frequently in females than in males (61.4% vs. 38.6%). This ratio was similar in the pediatric age group (65.0% vs. 35.0%). The mean survival time after metastasis was calculated as 316.7 ± 68.6 days. Female patients had a significantly longer survival time after metastasis compared to males (mean 400.4 vs. 165.4 days; p = 0.037). The median survival after metastasis was 86 days for patients with breast cancer and 204 days for patients with neuroblastoma. Conclusions: The most common source of orbital metastases in females is breast cancer, while neuroblastoma is prominent in pediatric patients. Despite all available treatment options, the prognosis after orbital metastasis is poor; this highlights the importance of early diagnosis and a multidisciplinary approach. Full article

Background: IgG4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory disorder characterized by lymphoplasmacytic infiltrates enriched in IgG4-positive plasma cells, storiform fibrosis, and frequently elevated serum IgG4 levels. Classic forms, such as pancreaticobiliary or retroperitoneal involvement, are often recognized early, whereas atypical manifestations mimic malignancy or inflammatory conditions, leading to delayed or inappropriate treatment. Case Series: A 30-year-old man presented with hyperemesis, proptosis, and gait instability. He was found to have colonic stenosis, stomach thickening, pachymeningitis, and polyserositis. Gastroenteric histology and serology confirmed IgG4-RD. Steroids were ineffective, but rituximab produced sustained clinical and radiologic improvement. A 35-year-old woman developed jaundice and cholestasis with a perihilar mass highly suggestive of cholangiocarcinoma. Histopathology revealed IgG4-RD, and rituximab therapy led to marked clinical and serological improvement. A 64-year-old woman with a submandibular mass underwent sialoadenectomy, with histology confirming IgG4-RD; she remained asymptomatic without systemic treatment. Literature Review: A literature review highlighted the diagnostic challenges of atypical IgG4-RD. Gastrointestinal involvement is rare and often misclassified as inflammatory bowel disease. Isolated biliary disease frequently mimics cholangiocarcinoma, while salivary gland involvement may be misdiagnosed as neoplasia. Serum IgG4 levels >135 mg/dL and IgG4/IgG ratio >0.21 may support clinical suspicion, but histopathology remains indispensable for definitive diagnosis and for excluding malignancy. Steroid responsiveness is a hallmark, though relapses after tapering are common, often necessitating B-cell-directed therapy. Conclusions: IgG4-RD should be considered in patients with unexplained, relapsing, or steroid-responsive conditions. Early recognition, multidisciplinary collaboration, and integration of histopathology with clinical features are essential to avoid misdiagnosis and optimize management. Full article

Background and Clinical Significance: Carcinoid tumors are rare, slow-growing neuroendocrine cell neoplasms that typically affect the gastrointestinal tract. While metastasis may occur, it most commonly occurs in the liver, and orbital metastasis is extremely rare, especially while on systemic somatostatin suppression. Case Presentation: A 57-year-old man with a history of gastrointestinal carcinoid tumor treated with lanreotide for 5 years presented with a left proptotic, red eye and double vision for several months. Clinical examination revealed left proptosis, supraduction deficit, lower lid retraction, and dilated episcleral vessels inferiorly. Magnetic resonance imaging demonstrated a 1.8 cm enhancing lesion centered within the left inferior rectus muscle. Left orbitotomy and biopsy were performed, which confirmed metastatic carcinoid tumor. He will undergo localized orbital radiation and substitution of lanreotide with systemic chemotherapy. Conclusions: Orbital metastasis of carcinoid tumor is extremely uncommon. Given its rarity, diagnosis may be challenging. In patients presenting with ocular complaints including chronic red eye, double vision, proptosis, and mass effect with a prior history of neuroendocrine cancer, a high index of suspicion for orbital metastasis is necessary with timely workup and treatment even if the disease has been otherwise well-controlled with somatostatin analogs. Full article

Graves’ orbitopathy (GO) is a debilitating autoimmune disorder that may require surgical orbital decompression in severe cases with risk of proptosis and optic neuropathy. This report presents a case treated with navigation-assisted three-wall orbital decompression, planned with preoperative imaging and assessed using postoperative analysis. Intraoperative navigation enabled precise localization of critical structures, improving osteotomy execution. Postoperatively, orbital volume increased by 3.5 cm³ (right eye) and 4.0 cm³ (left eye), while proptosis was reduced by 6 mm in both eyes. These changes correlated with intraocular pressure normalization and functional improvement. This was further supported by a postoperative Clinical Activity Score (CAS) of 0, indicating active orbital inflammation. Image-guided surgery (IGS) achieved an average proptosis reduction of 3.8 mm, slightly superior to that of non-guided techniques. Although IGS enhances precision and functional outcomes, it requires longer surgical time and incurs higher costs, highlighting the need for prospective studies on long-term efficacy This case supports the importance of integrating advanced imaging and navigation-assisted techniques in GO management to improve both functional and aesthetic outcomes. Full article

Background: Thyroid eye disease (TED) is a rare autoimmune orbital disorder predominantly associated with Graves’ disease. It is characterized by orbital inflammation, tissue remodeling, and potential visual morbidity. Conventional therapies, particularly systemic glucocorticoids, offer only partial symptomatic relief, failing to reverse chronic structural changes such as proptosis and diplopia, and are associated with substantial adverse effects. This review aims to synthesize recent developments in understandings of TED pathogenesis and to critically evaluate emerging therapeutic strategies. Methods: A systematic literature review was conducted using MEDLINE, Embase, and international clinical trial registries focusing on pivotal clinical trials and investigational therapies targeting core molecular pathways involved in TED. Results: Current evidence suggests that TED pathogenesis is primarily driven by the autoimmune activation of orbital fibroblasts (OFs) through thyrotropin receptor (TSH-R) and insulin-like growth factor-1 receptor (IGF-1R) signaling. Teprotumumab, a monoclonal IGF-1R inhibitor and the first therapy approved by the U.S. Food and Drug Administration for TED, has demonstrated substantial clinical benefit, including improvements in proptosis, diplopia, and quality of life. However, concerns remain regarding relapse rates and treatment-associated adverse events, particularly hearing impairment. Investigational therapies, including next-generation IGF-1R inhibitors, small-molecule antagonists, TSH-R inhibitors, neonatal Fc receptor (FcRn) blockers, cytokine-targeting agents, and gene-based interventions, are under development. These novel approaches aim to address both inflammatory and fibrotic components of TED. Conclusions: Teprotumumab has changed TED management but sustained control and toxicity reduction remain challenges. Future therapies should focus on targeted, mechanism-based, personalized approaches to improve long-term outcomes and patient quality of life. Full article

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder primarily affecting adults. The disease in pediatric age is unusual and preferentially affects adolescents. In contrast to adults, who commonly exhibit the involvement of multiple organs simultaneously or sequentially over time, young patients tend to present with a localized disease, typically affecting the orbits. Proptosis, ptosis, diplopia, and restricted eye movement may be observed in these patients. Symptoms are proteiform, and the disease is chronic and indolent with a relapsing–remitting course. Diagnostic criteria have been developed for adults, which may not fully capture the pediatric disease phenotype. If untreated or poorly managed, IgG4-RD can lead to progressive fibrosis and scarring of affected organs, potentially causing irreversible damage. We conducted a narrative review using the IMRAD approach, presenting a nonsystematic analysis of the literature on pediatric IgG4-RD. Original papers, case reports/series, and relevant reviews in English were selected from PubMed, EMBASE, and Web of Science up to January 2024. Keywords included “IgG4-Related Disease” and “pediatric” and, additionally, we presented two original pediatric cases. Our purpose is to offer an overview of IgG4-RD manifestations, and challenges in diagnosing and managing this rare condition in children. Full article

Thyroid eye disease (TED) is a complex autoimmune disorder characterized by orbital inflammation and tissue remodeling. Teprotumumab, a fully human monoclonal antibody targeting insulin-like growth factor-1 receptor (IGF-1R), represents a significant breakthrough in TED treatment. This review comprehensively analyzes the therapeutic role of teprotumumab in TED management. Mechanistically, teprotumumab inhibits the IGF-1R/TSHR signaling complex, thereby reducing orbital fibroblast differentiation and inflammatory responses. Phase II and III clinical trials have demonstrated its remarkable efficacy in reducing proptosis and improving clinical activity scores, with the benefits extending to both active and chronic TED cases. Real-world studies have validated these findings further and expanded its potential applications to various clinical scenarios, including dysthyroid optic neuropathy and steroid-resistant cases. However, several challenges remain. These include treatment-related adverse effects such as hyperglycemia and hearing impairment, with emerging evidence suggesting ethnic variations in susceptibility. The high cost of treatment poses significant accessibility barriers, while limited long-term follow-up data and potential disease recurrence necessitate further investigation. This review synthesizes the current evidence to inform clinical decision-making and highlights areas requiring additional research to optimize teprotumumab’s therapeutic application in TED management. Full article

Eye disease in dogs is a common problem that can affect both the health and well-being of the animals. The aim of the study was to investigate the prevalence and incidence of the most common eye diseases in a large population of dogs from two stray dog centers in northwestern Romania over a two-year period.We conducted a retrospective observational study of 208 eye diseases in two dog shelters in northwestern Romania: the “Ham Ham” Association and the “Free Life” Association in Satu Mare County. The study spanned from January 2022 to December 2023. We collected data on eye disease status from a total of 2293 dogs. The dogs were categorized into three age groups: young dogs (under 2 years), adult dogs (2 to 7 years), and senior dogs (over 7 years). Additionally, the dogs were grouped by sterilization status and gender. The statistical analysis was performed using Pearson’s chi-squared test, Student’s t-test, and binary logistic regression. The prevalence of ocular diseases in this study was 9.07%, with 208 out of 2293 dogs diagnosed with eye problems. The most common ocular diseases observed were conjunctivitis (58/208 or 27.9%), eyelid issues (50/208 or 24%), cataracts (40/208 or 19.2%), glaucoma (13/208 or 6.3%), keratopathies (10/208 or 4.8%), traumatic eye injuries (10/208 or 4.8%), foreign bodies (7/208 or 3.4%), blepharitis (5/208 or 2.4%), proptosis (3/208 or 1.4%), progressive retinal atrophy ((3/208 or 1.4%), retinal detachment ((3/208 or 1.4%), and dry eye (2/208 or 1%). Among the three age groups, puppies and young dogs (under 2 years) were the most affected, with a punctual prevalence of 50.96% at the “Free Life” center and 65.51% at the “Ham Ham” center. Compared with older dogs, the younger dogs were 4.91 times more likely to develop conjunctivitis (OR = 4.91, p = 0.001), 9.38 times more likely to develop eyelid problems (OR = 9.38, p < 0.000), and 0.31 times less likely to develop cataracts (OR = 0.31, p = 0.019).Our epidemiological study found that eye diseases are common among shelter dogs, with incidence rates (new cases over a specific period) varying by age. The youngest and oldest dogs were the most affected, with the highest prevalence observed in the youngest group. The study also highlighted the significant impact of factors such as the shelter center (p < 0.000), age (p < 0.000), and sterilization status (p < 0.000) on the prevalence of eye disorders. These findings underscore the need for enhanced awareness about eye diseases and their risk factors to facilitate early diagnosis and treatment. Full article

Background and Objectives: Uveitis-masked syndromes or masquerade syndromes (UMSs) are a group of ocular conditions with several systemic underlying causes, malignant or nonmalignant, that mimic the inflammatory status of the uvea. They are often difficult to detect and diagnose with traditional techniques, such as ophthalmic exams. Ocular B (bidimensional)-ultrasound (OBU) is a non-invasive, repeatable, rapid ultrasound method effective in indirect signs that lead back to systemic diseases. It is comparable in effectiveness with other imaging tools. The cause of UMSs can often be serious, and therefore early diagnosis and prompt treatment are critical. This study aimed to identify the sonographic signs of these forms, which can help physicians discover the cause underlying UMS. Materials and Methods: This was a consecutive, retrospective, nonrandomized study. This study was conducted at the University Hospital Polyclinic of Bari, Italy, from January 2022 to December 2024. A total of 186 patients were included, from 10 to 85 years old. They all underwent B-scan ultrasonography (Quantel Medical ABSolu Ocular Ultrasound). Results: All patients reported blurred vision, which could be accompanied by visual reduction (<20/40, Snellen charts), photophobia, floaters, flashes, proptosis, and redness. In all cases, we noted peculiar ultrasonographic signs, which allowed us to discriminate the underlying systemic diagnosis, such as vitreous corpuscles, choroid thickening, and primitive or metastatic solid tumors. Finally, we identified different diseases, such as primary intraocular lymphoma (PIOL), other lymphoproliferative conditions, orbital plasmacytoma, uveal melanoma, metastasis, endogenous endophthalmitis, retinal detachment, central serous retinopathy, metallic foreign bodies, ocular amyloidosis, and drug-induced UMSs. The sensitivity and specificity of ocular ultrasound compared to multimodal ocular imaging in UMSs were as follows: for primary intraocular lymphoma (PIOL) and other lymphoproliferative conditions, 0.98 (95% CI, 0.80–1) and 0.68 (90% CI, 0.40–0.92), respectively; for orbital plasmacytoma, 0.64 (92% CI, 0.52–0.86) and 0.66 (93% CI, 0.48–0.89), respectively; uveal melanoma, 1.00 (98% CI, 0.88–1.00) and 0.98 (95% CI, 0.86–0.98), respectively; metastasis, 0.75 (95% CI, 0.53–0.85) and 0.85 (95% CI, 0.48–0.98), respectively; endogenous endophthalmitis, 1.00 (95% CI, 0.50–1.00) and 0.83 (95% CI, 0.48–0.98), respectively; retinal detachment, both were 1.00 (95% CI, 0.87–1.00 and 0.84–0.97, respectively); central serous retinopathy, 0.60 (80% CI, 0.41–0.88) and 0.85 (95% CI, 0.52–0.98), respectively; metallic foreign bodies, 1.00 (95% CI, 0.78–1.00) and 0.99 (95% CI, 0.99–1.00), respectively; ocular amyloidosis, 0.77 (82% CI, 0.52–0.90) and 0.83 (80% CI, 0.49–0.88), respectively; and drug-induced UMSs, 0.64 (95% CI, 0.49–0.88) and 0.85 (95% CI, 0.52–0.98), respectively. Conclusions: Diagnosing UMS accurately can be quite challenging, and many of its different types frequently go undetected. This complexity in identification often leads to underdiagnosis, meaning it is essential to improve awareness and understanding of the condition in order to achieve better recognition and treatment. Early detection of these forms is imperative. The use of OBU can help diagnose indirect signs of these forms early and treat them promptly. It compares well with other diagnostic imaging techniques, such as MRI, but this does not mean that it replaces them; it can offer added value in multimodal imaging. Full article

Background/Aim: Among NF1-dependent tumors, the most common are optic pathway gliomas (OPGs). The objective of this study was the retrospective analysis of the course, indications for treatment, and effects of therapy for NF1-OPGs. Patients and Methods: We analyzed demographics, clinical and genetic data, imaging and ophthalmological parameters, their impact on therapeutic decisions, and the effectiveness of the therapy in 92 patients. Results: OPGs were unilateral in 55.4% of patients and bilateral in 44.6%. Post-contrast enhancement in MRI was observed in 67.4%. Oncological treatment was required in 16.3% of patients with median age 3.8 years. Factors significant in multivariate analysis contributing to the need of oncological treatment were: amblyopia and proptosis. Factors contributing to amblyopia were: strabismus, proptosis, co-occurrence of epilepsy, bilateral OPGs, and thickness of the optic nerve ≥ 8 mm. The first line of oncological treatment included vincristine + carboplatin or monotherapy with vinblastine. The use of subsequent lines of oncological treatment was necessary in 46.7% patients. Conclusions: The following conclusions, suggest modification of the approach in the management of patients with NF1-OPG, summarize the presented study: (1) perform the first MRI after the age of 1 year, (2) reduce the frequency of follow-up scans in the first year of observation in patients with isolated involvement of intraocular and/or intraorbital segments of the optic nerve, (3) refrain from administering contrast during control MRI examinations of the orbits after OPG diagnosis; (4) in patients with co-occurring psychomotor delay or treated with antiepileptic drugs, do not make decisions about oncological therapy when visual acuity deterioration is observed, without progression in optical coherence tomography (OCT), visual evoked potentials (VEP), and MRI. Full article

Long-term exposure to arsenic, a prevalent environmental contaminant, has been implicated in the pathogenesis of various hepatic conditions. Hepatic stellate cells (HSCs) are central to the development of liver fibrosis. Recently, the involvement of interleukin-17 (IL-17) and the NOD-like receptor protein 3 (NLRP3) inflammasome in hepatic pathologies has attracted significant research interest. Hepatocyte pyroptosis, a form of programmed cell death, is a critical factor in the occurrence of inflammation. The objective of this study was to investigate the specific roles of IL-17 and NLRP3 in the arsenic-induced activation of HSCs through hepatocyte pyroptosis. We pretreated MIHA cells with MCC950 (1 and 5 μM) and secukinumab (10 and 100 nM) for 4 h, then with NaAsO₂ (25 μM) for 24 h at 37 °C under 5% CO₂. After incubation, the cell-culture supernatant was collected and mixed with serum-free high-glucose DMEM medium in a 1:1 ratio to prepare the conditioned medium, which was subsequently used for the culture of LX-2 cells. The results showed that exposure to NaAsO₂ induced hepatocellular pyroptosis, which led to the release of the inflammatory cytokines IL-18 and IL-1β and subsequent activation of HSCs. Treatment with the inhibitors MCC950 and secukinumab significantly reduced the secretion of Extracellular matrix (ECM) components and attenuated HSC activation. These results demonstrate that blocking the IL-17 and NLRP3 signaling pathways significantly reduces HSC activation and attenuates hepatic fibrogenesis. These results provide novel molecular targets for the prevention and treatment of arsenic-related liver fibrosis. Full article

Background/Objectives: This case report presents a unique case of multiple postoperative complications, including sterile silicone implant extrusion, symblepharon formation, and the development of a giant cyst, following extensive multimodal chemotherapy for unilateral retinoblastoma in a pediatric patient. The case was further complicated by recurrent methicillin-resistant Staphylococcus aureus (MRSA) colonization, which persisted despite multiple eradication attempts. Methods: A 5-year-old boy presented with right-sided proptosis one year after receiving a secondary dermis-fat orbital graft. He had undergone 12 cycles of intravitreal, intra-arterial, and systemic chemotherapy as well as thermotherapy and cryotherapy due to recurrent retinoblastoma in the right eye. Following a third relapse, secondary enucleation was performed with a primary silicone orbital implant. However, extrusion of the implant occurred, and an orbital swab confirmed MRSA colonization. A secondary dermis-fat graft was harvested and implanted after ensuring MRSA clearance. A year later, the child developed rapid right-sided proptosis. Ultrasound revealed a cyst within the dermis-fat graft measured 23.6 mm in anteroposterior diameter. Surgery was postponed due to chickenpox, and the cyst enlarged reaching an anteroposterior diameter of 26.7 mm over two months. A complete excision was performed. Results: The surgery was uneventful. Intraoperative orbital swab was sterile, but MRSA was detected in a conjunctival swab, leading to treatment with local moxifloxacin drops and oral rifampicin. Conclusions: Giant cyst formation in a dermis-fat graft is an extremely rare complication. Complete excision remains the treatment of choice. However, in this case, it resulted in persistent anophthalmic socket syndrome, posing further reconstructive challenges. Full article