Proptosis

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  • 文章类型: Journal Article
    IgG4相关疾病(IgG4-RD)是一种自身免疫性纤维炎症,以IgG4阳性淋巴浆细胞浸润和受累器官广泛纤维化为特征。它通常会影响胰腺,胆道和唾液腺。中国轨道的参与是一个相对罕见的表现。受累器官存在广泛的纤维化。活检通常是诊断性的,它显示广泛的坏死和淋巴浆细胞浸润。他们对类固醇疗法表现出戏剧性的反应。在这里,我们介绍了三例涉及眼眶和鼻窦的IgG4-RD疾病,他们在三级护理教学医院接受了2年的评估和治疗。
    IgG4 related disease (IgG4-RD) is an auto immune fibro-inflammatory condition, characterised by presence of IgG4 positive lymphoplasmacytic infiltrates and extensive fibrosis of the involved organ. It commonly affects pancreas, biliary tract and salivary glands. Sino-orbital involvement is a relatively rare presentation. There is extensive fibrosis of the involved organ. Biopsy is often diagnostic and it shows extensive necrosis and lymphoplasmocytic infiltrates. They show dramatic response to steroid therapy. Here we present three cases of IgG4-RD disease involving orbit and para nasal sinuses who were evaluated and treated in a tertiary care teaching hospital over a period of 2 years.
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  • 文章类型: Journal Article
    甲状腺眼病(TED)是一种使人衰弱的自身免疫性疾病,通常与甲状腺功能障碍有关,导致显著的眼和眼眶发病率。这篇综述探讨了TED管理的最新进展,专注于医疗和外科创新。Teprotumumab的介绍,FDA批准的第一个专门用于TED的药物,标志着医学治疗的关键发展。Teprotumumab靶向胰岛素样生长因子-1受体(IGF-1R),有效减少炎症和组织重塑。临床试验证明其在减少眼球突出和改善生活质量方面的功效,使其成为治疗活跃的基石,中度至重度TED。对于患有慢性TED或对药物治疗无反应的患者,手术管理仍然至关重要。眼眶减压手术的进展,包括图像引导和微创技术,提供改善的结果和减少的并发症。眼睑和斜视手术的创新提高了功能和美容效果,进一步提高患者满意度。TED的管理需要涉及内分泌学家的多学科方法,眼科医生,眼整形外科医生,放射科医生,和其他专家。这种协作策略确保了全面的护理,解决TED的各个方面,从甲状腺功能障碍到眼部健康和心理健康。TED治疗的未来方向包括针对疾病病理生理学不同方面的新兴药物治疗和旨在提高精确度和安全性的先进外科技术。这篇评论强调了个性化的重要性,管理TED的多学科方法,强调当前的进步,并探索潜在的未来创新,以改善患者的预后和生活质量。
    Thyroid Eye Disease (TED) is a debilitating autoimmune condition often associated with thyroid dysfunction, leading to significant ocular and orbital morbidity. This review explores recent advancements in the management of TED, focusing on both medical and surgical innovations. The introduction of Teprotumumab, the first FDA-approved drug specifically for TED, marks a pivotal development in medical therapy. Teprotumumab targets the insulin-like growth factor-1 receptor (IGF-1R), effectively reducing inflammation and tissue remodeling. Clinical trials demonstrate its efficacy in reducing proptosis and improving quality of life, making it a cornerstone in the treatment of active, moderate-to-severe TED. Surgical management remains critical for patients with chronic TED or those unresponsive to medical therapy. Advancements in orbital decompression surgery, including image-guided and minimally invasive techniques, offer improved outcomes and reduced complications. Innovations in eyelid and strabismus surgery enhance functional and cosmetic results, further improving patient satisfaction. The management of TED necessitates a multidisciplinary approach involving endocrinologists, ophthalmologists, oculoplastic surgeons, radiologists, and other specialists. This collaborative strategy ensures comprehensive care, addressing the diverse aspects of TED from thyroid dysfunction to ocular health and psychological well-being. Future directions in TED treatment include emerging pharmacological therapies targeting different aspects of the disease\'s pathophysiology and advanced surgical techniques aimed at enhancing precision and safety. This review underscores the importance of a personalized, multidisciplinary approach in managing TED, highlighting current advancements, and exploring potential future innovations to improve patient outcomes and quality of life.
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  • 文章类型: Case Reports
    鼻窦癌是一种罕见但侵袭性的恶性肿瘤,起源于鼻腔和鼻旁窦。我们介绍了一个40岁的女性,她有三个月的头痛史,视力减退,和突起。影像学检查显示软组织密度,左眼眶骨侵蚀和外侧延伸。组织病理学检查证实鼻腔鼻窦癌伴间变性改变。患者接受了肿瘤的手术切除,并在神经ICU接受了术后护理。她的视力在手术后有所改善,她出院了,神经状况稳定。该病例强调了在诊断和治疗鼻窦癌方面的挑战,并强调了多学科护理对最佳结果的重要性。早期诊断和干预对于预防这种侵袭性恶性肿瘤患者的并发症和取得良好的预后至关重要。
    Sinonasal carcinoma is a rare but aggressive malignancy arising from the nasal cavity and paranasal sinuses. We present a case of a 40-year-old female who presented with a three-month history of headache, diminution of vision, and proptosis. Imaging studies revealed soft tissue density with bony erosion and extraconal extension in the left orbit. Histopathological examination confirmed sinonasal carcinoma with anaplastic changes. The patient underwent surgical excision of the tumor and received post-operative care in the neuro-ICU. Her visual acuity improved post-surgery, and she was discharged with stable neurological status. This case highlights the challenges in the diagnosis and management of sinonasal carcinoma and underscores the importance of multidisciplinary care for optimal outcomes. Early diagnosis and intervention are crucial in preventing complications and achieving favorable outcomes in patients with this aggressive malignancy.
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  • 文章类型: Case Reports
    颞下窝(ITF)肿瘤在儿童中很少见,可能会出现各种症状。畸胎瘤是来源于3个胚层的肿瘤,并且大约6%至10%在头部和颈部内。我们的研究讨论了儿科患者ITF中最早报道的畸胎瘤病例之一。一名3岁女孩每月反复出现2年的左眶周肿胀并伴有发烧,皮肤变色,和痛苦。先前的发作用抗生素治疗,但分辨率不完全。影像学显示在ITF中心的囊性病变。她接受了病变的鼻内镜活检,没有并发症。病理显示成熟的畸胎瘤主要由胰腺组织组成。提供者应考虑ITF肿瘤和对典型治疗无反应的眶周水肿等肿块的鉴别。
    Infratemporal fossa (ITF) tumors are rare in children and may present with a variety of symptoms. Teratomas are neoplasms derived from the 3 germ layers and approximately 6% to 10% are within the head and neck. Our study discusses one of the first reported cases of teratoma in the ITF in a pediatric patient. A 3-year-old girl presents with 2 years of recurrent monthly left periorbital swelling accompanied by fevers, skin discoloration, and pain. Prior episodes were treated with antibiotics with incomplete resolution. Imaging revealed a cystic lesion centered in the ITF. She was taken for endoscopic endonasal biopsy of the lesion and had no complications. Pathology revealed a mature teratoma composed primarily of pancreatic tissue. Providers should consider masses such as teratoma in the differential for ITF tumors and periorbital edema unresponsive to typical treatment.
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  • 文章类型: Case Reports
    背景:眼眶转移是小细胞肺癌的一种可能的并发症,在文献中很少报道双侧侵犯眼外肌的模式。
    方法:一名46岁的白人男性,既往有吸烟史和IV期小细胞肺癌,表现为视力丧失和左眼疼痛。检查显示双侧眼球突出,左侧传入瞳孔缺损,视力是左眼的手部动作和右眼的4/10。眼眶计算机断层扫描显示心尖眼外肌之间的左视神经受压,并对新发病的压迫性视神经病变进行了外侧角切开术,剩余的视觉改善。右眼眶的眼外肌也明显增大。患者接受化疗和局部药物和手术(羊膜覆盖暴露性角膜病变)眼科治疗的姑息治疗,直到他最终在5个月后死亡。
    结论:眼外肌的双侧转移是小细胞肺癌的一种非常罕见的表现,在这些病例中姑息治疗具有挑战性。
    BACKGROUND: Orbital metastasis is a possible complication of small cell lung cancer and a pattern of bilateral invasion of the extraocular muscles has rarely been reported in literature.
    METHODS: A 46-year-old white male with a past medical history of smoking and stage IV small cell lung carcinoma presented with loss of vision and pain in the left eye. Examination revealed bilateral proptosis and left afferent pupillary defect, and visual acuity was hand motion on the left eye and 4/10 on the right eye. An orbital computed tomography scan showed a compression of the left optic nerve between the extraocular muscles at the apex, and a lateral canthotomy was performed for a new-onset compressive optic neuropathy, with residual visual improvement. There was also significant enlargement of the extraocular muscles in the right orbit. The patient was maintained in palliative treatment with both chemotherapy and local medical and surgical (amniotic membrane cover for exposure keratopathy) ophthalmological treatments until he eventually died 5 months after.
    CONCLUSIONS: Bilateral metastasis to the extraocular muscles is a very rare manifestation of small cell lung cancer and the palliative treatment in these cases is challenging.
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  • 文章类型: Case Reports
    硬脑膜窦畸形(DSM)是一种罕见的血管畸形,其特征是硬脑膜静脉窦扩张,有或没有异常的颈静脉球。它的表现与静脉反流相关的继发性突增是轶事,到目前为止,只有6起此类病例报告。我们报告了一个17个月大的男孩,该男孩在横窦和耳廓的DSM之后表现出右眼进行性突出。动静脉瘘血管内栓塞后,在6个月的随访中发现静脉湖完全血栓形成和眼球突出改善.这种罕见畸形的预后是可变的,并取决于特定的血管结构特征。
    Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features.
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  • 文章类型: Journal Article
    甲状腺眼病(TED)是一种涉及眼周和眼眶软组织的炎症性疾病,影响最常见的甲状腺功能亢进患者。用于疾病活动期的传统治疗范围从用于轻度症状的保守润滑到用于中度至重度症状的全身免疫调节药物。Teprotumumab(Tepezza)是一种对胰岛素样生长因子1具有抑制作用的单克隆抗体,并且是第一个食品和药物管理局(FDA)批准的靶向药物疗法,用于减少与TED相关的炎症体征和症状。两个大型多中心,随机化,双面蒙面,安慰剂对照试验证实了teprotumumab在活动性患者中的疗效和安全性,中度至重度TED。最近的报道和出版物也证明了teprotumumab在更广泛的患者中的功效。在这次审查中,我们总结了TED的临床特征和病理生理学,病程,传统的管理方法。我们进一步详述了teprotumumab的发展,使其获得FDA批准的创始研究,不良事件简介,以及正在进行的和未来的调查。
    Thyroid eye disease (TED) is an inflammatory condition involving the periocular and orbital soft tissues, affecting most commonly patients with hyperthyroid disorders. Traditional treatments used for the active phase of the disease range from conservative lubrication for mild symptoms to systemic immunomodulating drugs for moderate-to-severe symptoms. Teprotumumab (Tepezza) is a monoclonal antibody with an inhibitory effect on insulin-like growth factor 1 and is the first Food and Drug Administration (FDA) approved targeted medical therapy for reducing the inflammatory signs and symptoms associated with TED. Two large multicenter, randomized, double-masked, placebo-controlled trials have confirmed the efficacy and safety of teprotumumab in patients with active, moderate-to-severe TED. Recent reports and publications have also demonstrated the efficacy of teprotumumab in a wider range of patients. In this review, we summarize the clinical features and pathophysiology of TED, disease course, and traditional management methods. We further detail the development of teprotumumab, the founding studies that brought it to its FDA approval, adverse events profile, and ongoing as well as future investigations.
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  • 文章类型: Journal Article
    目的:本研究旨在确定影响蝶眶脑膜瘤(SOM)手术中眼球突出恢复的因素,并评估功能和肿瘤预后。
    方法:分析了来自32例连续SOM手术病例(2002-2021)的数据。临床,放射学,Operative,和肿瘤参数进行了检查。使用MRI或CT扫描的眼球突出指数(EI)评估眼球下垂。进行统计分析以确定突增恢复的预测因素。
    结果:75%的患者在手术后有改善(EI从1.28±0.16下降到1.20±0.13,p=0.048)。EI稳定或恶化的患者体重指数较高(28.5±7.9vs.24.1±4.7,p=0.18),辛普森等级(IV75%vs.65%,p=0.24),和蝶骨中翼震中受累(63%vs.38%,p=0.12),但与眼球突出不良结局无显著相关因素.改善组有更高的斑块形态,颞下窝侵入,和放射治疗海绵窦残留肿瘤(88%vs.75%,p=0.25;51%vs.25%,p=0.42;41%vs.25%,分别为p=0.42),但没有统计学意义。视力稳定在78%,提高了13%,在随访期间恶化了9%。4例患者中有3例(75%)手术对术前动眼神经功能障碍具有积极影响。术后观察到25%的动眼神经功能障碍,其中75%完全恢复。这种情况与眼眶肿瘤残留物的照射显着相关(p=0.04)。术后新的三叉神经感觉减退观察到47%,其中73%已恢复。所有SOM被归类为WHO1级,补充治疗实现了肿瘤控制,需要伽玛刀放射外科的53%和标准放射治疗的6%。
    结论:手术可有效改善SOM的眼球突出,虽然完整的决议是罕见的。缺乏预测因素表明是多因素的原因,包括体重指数和肿瘤切除等级。术后动眼神经功能障碍和三叉神经感觉减退很常见,但通常会恢复。伽玛刀放射外科对不断发展的肿瘤残留物保持长期的肿瘤控制。
    OBJECTIVE: This study aimed to identify factors affecting proptosis recovery in spheno-orbital meningioma (SOM) surgery and assess functional and oncological outcomes.
    METHODS: Data from 32 consecutive SOM surgery cases (2002-2021) were analyzed. Clinical, radiological, operative, and oncological parameters were examined. Proptosis was assessed using the exophthalmos index (EI) on MRI or CT scans. Statistical analyses were performed to identify predictive factors for proptosis recovery.
    RESULTS: Proptosis improved in 75% of patients post-surgery (EI decreased from 1.28 ± 0.16 to 1.20 ± 0.13, p = 0.048). Patients with stable or worsened EI had higher body mass index (28.5 ± 7.9 vs. 24.1 ± 4.7, p = 0.18), Simpson grade (IV 75% vs. 65%, p = 0.24), and middle sphenoid wing epicenter involvement (63% vs. 38%, p = 0.12), but no significant factors were associated with unfavorable exophthalmos outcomes. The improvement group had higher en plaque morphology, infratemporal fossa invasion, and radiation treatment for cavernous sinus residual tumor (88% vs. 75%, p = 0.25; 51% vs. 25%, p = 0.42; 41% vs. 25%, p = 0.42, respectively), but without statistical significance. Visual acuity remained stable in 78%, improved in 13%, and worsened in 9% during follow-up. Surgery had a positive impact on preoperative oculomotor nerve dysfunction in 3 of 4 patients (75%). Postoperative oculomotor nerve dysfunction was observed in 25%, of which 75% fully recovered. This occurrence was significantly associated with irradiation of an orbital tumor residue (p = 0.04). New postoperative trigeminal hypoesthesia was observed in 47%, of which 73% recovered. All SOMs were classified as WHO grade 1, and complementary treatments achieved oncological control, requiring gamma-knife radiosurgery in 53% and standard radiotherapy in 6%.
    CONCLUSIONS: Surgery effectively improves proptosis in SOM, though complete resolution is rare. The absence of predictive factors suggests multifactorial causes, including body mass index and tumor resection grade. Postoperative oculomotor nerve dysfunction and trigeminal hypoesthesia are common but often recover. Gamma-knife radiosurgery maintains long-term oncological control for evolving tumor residue.
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  • 文章类型: Journal Article
    背景:PolygonumhydropiperL(PH)被广泛用于治疗痢疾,胃肠炎,腹泻和其他疾病。黄连(CC)具有清湿清热作用,吹扫火,和解毒。研究证实,PH中的黄酮类化合物和CC中的生物碱减轻炎症,抑制肠道炎症的发展。然而,PH-CC如何影响UC尚不清楚.因此,本研究旨在通过网络药理学和体内实验分析PH-CC对溃疡性结肠炎(UC)的作用机制。
    方法:基于相关数据库筛选PH-CC的活性成分和靶标以及UC的靶标。通过蛋白质-蛋白质相互作用网络(PPI)预测PH-CC对UC的核心靶标,然后使用注释数据库进行基因本体论-生物学过程(GO-BP)功能富集分析,可视化和集成发现(DAVID)数据库。焦亡蛋白之间的结合活性,基于分子对接技术对核心靶标和有效成分进行验证。最后,结合网络药理学和文献研究的结果,体内实验验证了PH-CC抗UC的作用机制。
    结果:PH-CC中有23种活性成分和191种潜在靶标,UC中的5275个目标,和141个共同目标。对141个共靶标的GO-BP功能分析表明,前20个生物过程与炎症和脂多糖(LPS)刺激密切相关。此外,核心靶标与相应化合物具有良好的结合活性。动物实验表明,PH-CC可有效预防UC小鼠体重下降,降低疾病活动指数(DAI)评分,保持结肠长度,抑制髓过氧化物酶(MPO)活性,抑制焦亡蛋白表达,下调IL-18和IL-1β水平,减轻肠道炎症反应。
    结论:网络药理学和动物实验结果表明,PH-CC抑制炎症反应,结肠形态恢复,并抑制UC小鼠的焦亡。因此,PH-CC可能通过调节NOD样受体蛋白结构域3(NLRP3)/Caspase-1信号通路改善UC。
    BACKGROUND: Polygonum hydropiper L (PH) was widely used to treat dysentery, gastroenteritis, diarrhea and other diseases. Coptis chinensis (CC) had the effects of clearing dampness-heat, purging fire, and detoxifying. Study confirmed that flavonoids in PH and alkaloids in CC alleviated inflammation to inhibit the development of intestinal inflammation. However, how PH-CC affects UC was unclear. Therefore, the aim of this study is to analyze the mechanism of PH-CC on ulcerative colitis (UC) through network pharmacology and in vivo experiments.
    METHODS: The active ingredients and targets of PH-CC and targets of UC were screened based on related databases. The core targets of PH-CC on UC was predicted by protein-protein interaction network (PPI), and then the Gene Ontology-biological processes (GO-BP) function enrichment analysis was conducted using the Database for Annotation, Visualization and Integrated Discovery (DAVID) database. The binding activity between pyroptosis proteins, core targets and effective ingredients were verified based on molecular docking technology. Finally, combined with the results of network pharmacology and literature research, the mechanism of PH-CC against UC was verified by in vivo experiments.
    RESULTS: There were 23 active components and 191 potential targets in PH-CC, 5275 targets in UC, and 141 co-targets. GO-BP functional analysis of 141 co-targets showed that the first 20 biological processes were closely related to inflammation and lipopolysaccharide (LPS) stimulation. Furthermore, core targets had good binding activity with the corresponding compounds. Animal experiment indicated that PH-CC effectively prevented weight loss in UC mice, reduced the disease activity index (DAI) score, maintained colon length, suppressed myeloperoxidase (MPO) activity, inhibited pyroptosis protein expression, and downregulated the levels of IL-18 and IL-1β to alleviate intestinal inflammation.
    CONCLUSIONS: The results of network pharmacology and animal experiments showed that PH-CC suppressed the inflammatory response, restored colon morphology, and inhibited pyroptosis in UC mice. Thus, PH-CC may improve UC by regulating the NOD-like receptor protein domain 3 (NLRP3)/Caspase-1 signaling pathway.
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  • 文章类型: Case Reports
    纤维骨病变包括具有不同临床和组织病理学特征的多种骨病变。
    我们报告一例17岁男性患者的骨水泥骨化病变,累及左侧筛窦和蝶窦。计算机断层扫描显示扩张性高密度病变,在左筛窦和蝶窦进入硬化的外周内膜,并延伸到左鼻腔。术前计划进行磁共振成像。经鼻内镜切除,组织病理学检查证实诊断为骨化性纤维瘤(OF)。
    筛窦受累于OF是一种罕见的疾病;因此,我们查阅了类似病例的文献,以强调可能的临床表现和治疗.内窥镜管理是安全的,有效的方法,并发症发生率低。
    UNASSIGNED: Fibro-osseous lesions include a variety of bone lesions with different clinical and histopathological features.
    UNASSIGNED: We report a case of cemento-ossifying lesion involving the left ethmoid and sphenoid sinus in a 17-year-old male patient. Computed tomography showed an expansile hyperdense lesion with sclerosed peripheral mantle epicentered on the left ethmoid and sphenoid sinus and extending into the left nasal cavity. Magnetic resonance imaging was done for preoperative planning. Transnasal endoscopic resection was performed and histopathological examination confirmed the diagnosis of ossifying fibroma (OF).
    UNASSIGNED: Involvement of the ethmoid sinus with OF is a rare condition; therefore, we examined the literature for similar cases to highlight the possible clinical presentation and management. Endoscopic management is a safe, effective approach with a low rate of complications.
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