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Indian Journal of Otolaryngology and... Jun 2024Ophthalmic vein thrombosis is a severe clinical entity with proptosis, eyelid swelling, orbital pain and reduction of visual acuity; its incidence is rare with 3-4 cases...
Ophthalmic vein thrombosis is a severe clinical entity with proptosis, eyelid swelling, orbital pain and reduction of visual acuity; its incidence is rare with 3-4 cases /million /year. Clinical manifestations result from venous congestion caused by septic (orbital cellulitis) or aseptic aetiologies (coagulopathies, trauma) and in some cases it could be associated with cavernous sinus thrombosis. In this paper, we describe a case report unique in the literature, of bilateral cavernous sinus and ophthalmic veins thrombosis due to both septic and aseptic causes characterized by unilateral sphenoid sinusitis sustained by infection. is an opportunistic animal pathogen, and its infections occur in both domestic and wild animals worldwide but are rare in humans; this is the first instance of human infection in the head and neck with an unknown hypercoagulable state.
PubMed: 38883471
DOI: 10.1007/s12070-024-04505-1 -
BMC Ophthalmology Jun 2024Echinococcosis, commonly known as hydatid disease, is a zoonotic infection resulting from the tapeworm Echinococcus granulosus. The occurrence of hydatid cysts in the... (Review)
Review
BACKGROUND
Echinococcosis, commonly known as hydatid disease, is a zoonotic infection resulting from the tapeworm Echinococcus granulosus. The occurrence of hydatid cysts in the orbital region is uncommon, representing less than 1% of all reported hydatid cases. This report details a unique case of an intramuscular hydatid cyst in the orbital region that led to compressive optic neuropathy.
CASE PRESENTATION
A 22-year-old male from Kabul, Afghanistan presented with a five-month history of progressive proptosis in his left eye, associated with a gradual decrease in vision over the past three weeks. The left eye exhibited upward globe dystopia, ocular motility limitation, mild conjunctival injection, and chemosis. Diagnosis was achieved through imaging and histopathological examination. Treatment involves surgical removal of the cyst and prolonged albendazole therapy. The postoperative course showed significant improvement in the patient's condition and restoration of his vision.
CONCLUSIONS
Despite its rarity, this case underscores the importance of awareness and knowledge of hydatid disease among physicians, especially those working in endemic areas. It emphasizes the importance of including hydatid disease in the differential diagnosis of orbital masses, particularly in endemic regions.
Topics: Humans; Male; Echinococcosis; Young Adult; Optic Nerve Diseases; Eye Infections, Parasitic; Orbital Diseases; Magnetic Resonance Imaging; Albendazole; Tomography, X-Ray Computed
PubMed: 38877497
DOI: 10.1186/s12886-024-03502-w -
Journal of Cranio-maxillo-facial... Jun 2024The orbital cavity is a subject of interest for various specialists, and achieving optimal outcomes requires comprehensive, multidisciplinary management. This study aims...
The orbital cavity is a subject of interest for various specialists, and achieving optimal outcomes requires comprehensive, multidisciplinary management. This study aims to report 10 years of experience in the preoperative, surgical, and postoperative care of patients with orbital lesions, examining their clinical, radiological, and anatomopathological features and outcomes. A retrospective review of 125 patients who underwent surgical treatment for intraorbital masses between January 2012 and December 2021 was performed. Outcome measures included postoperative diplopia, exophthalmos, decimal visual acuity, eyeball position, ocular motility, operative time, complications, and aesthetic results. A total of 107 patients were included. All cases were discussed with a neuroradiologist to determine the best therapeutic approach based on preoperative imaging. Preoperative diplopia was linked to extraconal (p = 0.03) and anterior (p = 0.001) lesions, and exophthalmos and visual acuity deterioration were associated with intraconal (p = 0.02; p = 0.03) and retrobulbar (p = 0.001; p = 0.02) lesions. Complications (11.2%) included diplopia, worsened visual acuity, postoperative blepharoptosis, and postoperative ectropion. Of the patients, 80.4% reported an "excellent" aesthetic outcome. This study underscores the importance of a multidisciplinary approach based on a thorough analysis of preoperative imaging. Periorbital approaches tailored to the lesion's three-dimensional location enables safe access to most intraorbital lesions, resulting in minimal complications and good aesthetic results.
PubMed: 38876957
DOI: 10.1016/j.jcms.2024.06.019 -
Medicine Jun 2024Graves' disease is a prevalent thyroid disorder and is the primary cause of hyperthyroidism. Significant progress has been made in understanding the epidemiology,...
BACKGROUND
Graves' disease is a prevalent thyroid disorder and is the primary cause of hyperthyroidism. Significant progress has been made in understanding the epidemiology, pathogenesis, diagnosis, treatment, and prognosis of this disease. However, bibliometric analyses on Graves' disease are lacking. We aimed to comprehensively summarize the research, progression, and focal points of Graves' disease through data mining and integrated analysis of the existing literature.
METHODS
We retrieved relevant literature on Graves' disease from 2003 to 2023 from the Web of Science database. We performed bibliometric analysis using CiteSpace and the R package Bibliometrix.
RESULTS
We identified 10,901 publications from 132 countries, with a steady rise in the number of publications over the past 5 years. The US leads in publication volume, with the University of California System being the primary contributing institution. The journal Thyroid had the highest publication output, while the Journal of Clinical Endocrinology and Metabolism was the most frequently cited. These publications involved 2305 authors, with Antonelli Alessandro and Smith Terry being the most prolific. The most frequently cited articles were the "2016 American Thyroid Association guidelines for diagnosis and management of hyperthyroidism and other causes of thyrotoxicosis" and the "Thyroid Association/European Group on Graves' orbitopathy guidelines for the management of Graves' orbitopathy." Analysis of the bursts of cited references, keywords, and their clustering revealed that research on Graves' disease predominantly centers on clinical management, thyroid-stimulating hormone receptors, thyroid hormones, autoimmunity and inflammation, Graves' ophthalmopathy, thyroid nodules, and thyroid cancer.
CONCLUSION
This is the first comprehensive bibliometric study to summarize progress and trends in Graves' disease research. These results highlight recent research hotspots and promising directions, thereby providing a valuable reference for other scholars.
Topics: Bibliometrics; Humans; Graves Disease; Biomedical Research; Global Health
PubMed: 38875401
DOI: 10.1097/MD.0000000000037963 -
American Journal of Ophthalmology Case... Sep 2024We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD).
PURPOSE
We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD).
OBSERVATIONS
ECD is a rare non-Langerhans cell histiocytosis characterized by multisystemic organ involvement and poor prognosis. Our patient had complete vision loss due to prominent orbital involvement before any systemic symptoms appeared. This case demonstrates variable clinical manifestations of ECD.
CONCLUSIONS AND IMPORTANCE
Painless bilateral proptosis with poor response to steroid treatment should prompt consideration for ECD and systemic evaluation. In addition, in the absence of typical clinical manifestations, a thorough evaluation of the biopsy can be crucial for an accurate diagnosis.
PubMed: 38872875
DOI: 10.1016/j.ajoc.2024.102087 -
Clinical Practice and Cases in... May 2024A 52-year-old female presented to the emergency department with four days of right periorbital pain, ipsilateral temporal headache, diplopia, and photophobia. Physical...
CASE PRESENTATION
A 52-year-old female presented to the emergency department with four days of right periorbital pain, ipsilateral temporal headache, diplopia, and photophobia. Physical examination of the right eye revealed painful ophthalmoplegia, cranial nerves III and VI paresis, increased intraocular pressure, and mild proptosis. Magnetic resonance venogram and magnetic resonance imaging orbits with contrast demonstrated an abnormal signal surrounding the right cavernous sinus/petrous apex. Tolosa-Hunt syndrome (THS) was diagnosed. Per neurology recommendations, the patient was placed on a steroid regimen over the course of three weeks. She was discharged on hospital day nine following resolution of symptoms. She had no recurrence of symptoms or residual deficits noted at her two-week follow-up appointment.
DISCUSSION
With an estimated annual incidence of one case per million, THS is a sinister etiology of unilateral headache, painful ophthalmoplegia, and oculomotor palsy. Tolosa-Hunt syndrome is caused by granulomatous inflammation in the cavernous sinus and is highly responsive to corticosteroids. Magnetic resonance imaging studies of the cavernous sinus and orbital apex are highly sensitive for THS and characteristically show enlargement and focal-enhancing masses within the affected cavernous sinus.
PubMed: 38869347
DOI: 10.5811/cpcem.2582 -
Frontiers in Endocrinology 2024The current clinical practice lacks sufficient objective indicators for evaluating thyroid-associated ophthalmopathy (TAO). This study aims to quantitatively assess TAO...
BACKGROUND
The current clinical practice lacks sufficient objective indicators for evaluating thyroid-associated ophthalmopathy (TAO). This study aims to quantitatively assess TAO by evaluating levator palpebrae superioris (LPS) using Dixon-T2WI.
METHODS
The retrospective study included 231 eyes (119 patients) in the TAO group and 78 eyes (39 volunteers) in the normal group. Dixon-T2WI provided data on maximum thickness of LPS (LPS_T) and signal intensity ratio (LPS_SIR) between the muscle and ipsilateral brain white matter. TAO diagnosis and assessment of its activity and severity were quantitatively determined using LPS_T and LPS_SIR.
RESULTS
In the TAO group, LPS_T and LPS_SIR were higher than those in the normal group ( < 2.2e-16). The upper lid retraction (ULR) ≥ 2 mm group exhibited higher LPS_T and LPS_SIR compared to the ULR < 2 mm and normal groups. Optimal diagnostic performance was achieved with an AUC of 0.91 for LPS_T (cutoff: 1.505 mm) and 0.81 for LPS_SIR (cutoff: 1.170). LPS_T ( = 2.8e-07) and LPS_SIR ( = 3.9e-12) in the active phase were higher than in the inactive phase. LPS_T and LPS_SIR showed differences among the mild, moderate-to-severe, and sight-threatening groups ( < 0.05). ROC showed an AUC of 0.70 for LPS_T (cutoff: 2.095 mm) in judging the active phase, and 0.78 for LPS_SIR (cutoff: 1.129). For judging the moderate-to-severe and above, AUC was 0.76 for LPS_T (cutoff: 2.095 mm) and 0.78 for LPS_SIR (cutoff: 1.197).
CONCLUSION
The maximum thickness and SIR of LPS provide imaging indicators for assisting in the diagnosis and quantitative evaluation of TAO.
Topics: Humans; Graves Ophthalmopathy; Female; Male; Retrospective Studies; Middle Aged; Adult; Oculomotor Muscles; Magnetic Resonance Imaging; Aged; Eyelids; Case-Control Studies
PubMed: 38868741
DOI: 10.3389/fendo.2024.1387217 -
Heliyon Jun 2024The efficacy of rituximab (RTX) in treating steroid-resistant Graves' orbitopathy (GO) has been limitedly studied in Asians. Moreover, RTX has been considered even less...
BACKGROUND AND OBJECTIVES
The efficacy of rituximab (RTX) in treating steroid-resistant Graves' orbitopathy (GO) has been limitedly studied in Asians. Moreover, RTX has been considered even less for patients with steroid-resistant dysthyroid optic neuropathy (DON) who failed to undergo orbital decompression surgery for physical or financial reasons, or who responded poorly to the procedure. This study aimed to investigate the efficacy of RTX in treating steroid-resistant active moderate-to-severe and sight-threatening GO in a Chinese population.
METHODS
Data from 28 patients with steroid-resistant GO prescribed a single dose of 500 mg RTX were retrospectively retrieved. Treatment responses and contributing factors were analyzed.
RESULTS
The median follow-up time was 22 (8-34) weeks. 23 (82.1 %) patients had a positive objective outcome recommended by the European Group on Graves' Orbitopathy (EUGOGO), while 25 (92.6 %) had a decrease in 7-item clinical activity score (CAS) by at least 2. Diplopia, visual dysfunction, and MRI-detected T2 relaxation time of the involved extraocular muscles improved significantly at the last follow-up compared to baseline (81.0 % vs. 47.6 %, 38.9 % vs. 16.7 %, and 87.8 (8.64) vs. 75.8 (10.9) ms, respectively; all values < 0.05). No significant improvement was seen in terms of proptosis and eye muscle duction. Notably, a higher baseline IgG4 to IgG ratio was a predictor for RTX-induced positive EUGOGO outcomes. After RTX treatment, all 8 patients with DON demonstrated inactivation, and 4 improved in visual acuity by ≥ 1 line. No patient with DON experienced obvious deterioration.
CONCLUSION
A single dose of 500 mg RTX seemed to be an effective and tolerable treatment for steroid-resistant GO. However, larger-scale studies with a control group are required for a more solid conclusion. The role of RTX in steroid-resistant DON management where surgery is unavailable or ineffective should be further explored.
PubMed: 38867959
DOI: 10.1016/j.heliyon.2024.e31932 -
Scientific Reports Jun 2024The primary objective of this study is to understand the regulatory role of epigenetics in thyroid-associated ophthalmopathy (TAO) using multi-omics sequencing data. We...
The primary objective of this study is to understand the regulatory role of epigenetics in thyroid-associated ophthalmopathy (TAO) using multi-omics sequencing data. We utilized tRFs sequencing data, DNA methylation sequencing data, and lncRNA/circRNA/mRNA sequencing data, as well as several RNA methylation target prediction websites, to analyze the regulatory effect of DNA methylation, non-coding RNA, and RNA methylation on TAO-associated genes. Through differential expression analysis, we identified 1019 differentially expressed genes, 985 differentially methylated genes, and 2601 non-coding RNA. Functional analysis showed that differentially expressed genes were mostly associated with the PI3K signaling pathway and the IL17 signaling pathway. Genes regulated by DNA epigenetic regulatory networks were mainly related to the Cytokine-cytokine receptor interaction pathway, whereas genes regulated by RNA epigenetic regulatory networks were primarily related to the T cell receptor signaling pathway. Finally, our integrated regulatory network analysis revealed that epigenetics mainly impacts the occurrence of TAO through its effects on key pathways such as cell killing, cytokine production, and immune response. In summary, this study is the first to reveal a new mechanism underlying the development of TAO and provides new directions for future TAO research.
Topics: Humans; Graves Ophthalmopathy; Epigenesis, Genetic; DNA Methylation; Gene Regulatory Networks; Signal Transduction; Inflammation; RNA, Long Noncoding; Gene Expression Profiling; Gene Expression Regulation
PubMed: 38867076
DOI: 10.1038/s41598-024-64415-8 -
BMJ Open Jun 2024Severe Graves' disease is a life-changing condition with poor outcomes from currently available treatments. It is caused by directly pathogenic thyroid-stimulating... (Randomized Controlled Trial)
Randomized Controlled Trial
INTRODUCTION
Severe Graves' disease is a life-changing condition with poor outcomes from currently available treatments. It is caused by directly pathogenic thyroid-stimulating hormone receptor-stimulating antibodies (TRAb), which are secreted from plasma cells. The human anti-CD38 monoclonal antibody daratumumab was developed to target plasma cells which express high levels of CD38, and is currently licensed for treatment of the plasma cell malignancy, myeloma. However, it can also deplete benign plasma cells with the potential to reduce TRAb and alter the natural history of severe Graves' disease. This study aims to establish proof of concept that daratumumab has efficacy in patients with severe Graves' disease and will provide important data to inform a choice of dosing regimen for subsequent trials.
METHODS AND ANALYSIS
The Graves-PCD trial aims to determine if daratumumab modulates the humoral immune response in patients with severe Graves' disease, and if so, over what time period, and to find an optimal dose. It is a single-blinded, randomised, dose-finding, adaptive trial using four different doses of daratumumab or placebo in 30 adult patients. Part 1 of the trial is dose-finding and, following an interim analysis, in part 2, the remaining patients will be randomised between the chosen dose(s) from the interim analysis or placebo. The primary outcome is the percentage change in serum TRAb from baseline to 12 weeks.
ETHICS AND DISSEMINATION
The trial received a favourable ethical opinion from London-Hampstead Research Ethics Committee (reference 21/LO/0449). The results of this trial will be disseminated at international meetings, in the peer-reviewed literature and through partner patient group newsletters and presentations at patient education events.
TRIAL REGISTRATION NUMBER
ISRCTN81162400.
Topics: Humans; Graves Disease; Antibodies, Monoclonal; Randomized Controlled Trials as Topic; Plasma Cells; Single-Blind Method; Adult; Male; Female; Dose-Response Relationship, Drug
PubMed: 38866568
DOI: 10.1136/bmjopen-2023-079158