视神经脊髓炎谱系疾病相关视神经炎是一种累及视神经的脱髓鞘性炎症疾病,视力损伤严重,预后差,复发率高。及时控制急性发作和有效预防复发是治疗的关键。目前治疗主要包括糖皮质激素、血浆置换、免疫吸附、免疫抑制剂、靶向单抗类药物。特别是近年来依库丽单抗、萨特利珠单抗、及依那利珠单抗取得重大进展。该文综述视神经脊髓炎谱系疾病相关视神经炎近年治疗研究进展,期望为临床决策提供有益参考。
Neuromyelitis optica spectrum disorders (NMOSD) is a central nervous system inflammatory demyelinating disease with involvement of the optic nerve and spinal cord, with poor prognosis and high recurrence rate. Timely control of acute attacks and effective prevention of recurrence are the keys to treatment. This article reviews the recent research progress in the treatment of optic neuritis associated with NMOSD , hoping to provide useful references for clinical decision-making.
目的:总结并分析视野为中心暗点的视神经病变的病因和临床特点,为临床诊治提供参考。方法:回顾性病例研究。分析2018年8月至2020年3月期间,在中山大学中山眼科中心神经眼科专科门诊就诊,视野表现为中心暗点且随访1年以上的视神经病变患者的资料。患者双眼均行最佳矫正视力、眼压、裂隙灯显微镜及前置镜、频域光学相干断层扫描、视野、颅脑和眼眶核磁共振检查,静脉采血行血常规、血生化、肝肾功能、感染指标(乙肝、丙肝、梅毒、HIV及结核T-spot)检查及Leber遗传性视神经病变的线粒体DNA和OPA1基因检测。结果:共纳入20例患者,病因诊断构成为:Leber遗传性视神经病变9例(45%),显性视神经萎缩2例(10%),乙胺丁醇中毒性视神经病变6例(30%),营养性视神经病变2例(10%)和特发性脱髓鞘性视神经病变1例(5%)。遗传性视神经病变的视力预后差,特别是Leber遗传性视神经病变,78%的随访视力(≥1年)不高于0.1。伴有mtDNA或OPA1基因突变的乙胺丁醇中毒性视神经病变患者,视力预后差。结论:视野为中心暗点表现的视神经病变,主要为遗传、中毒和营养性视神经病变。遗传性视神经病变具有不完全外显率的特点,视野为中心暗点的视神经病变需行基因检测排除遗传性视神经病变。
Objective: To summarize and analyze the etiology and clinical features of optic neuropathy with visual field defect of central scotoma as a reference for clinical diagnosis and treatment. Methods: In the retrospective case study, the data of patients admitted in Neuro-ophthalmic Department of Zhongshan Ophthalmic Center of Sun Yat-sen University from August 2018 to March 2020, who presented with visual field defect of central scotoma and were followed up for more than 1 year, were analyzed. Both eyes of all the patients underwent best corrected visual acuity, intraocular pressure, slit lamp microscope and front mirror, spectral domain optical coherence tomography, humphry visual field tests and MRI of brain and orbit. We examined the blood routine, biochemical test, renal and liver function, infection indicators (hepatitis B, hepatitis C, syphilis, HIV and tuberculosis T-spot), mitochondrial DNA and OPA1 gene detection of Leber hereditary optic neuropathy. The follow-up time of the patients in neuro-ophthalmic department was more than 1 year. Results: A total of 20 patients were recruited. Among them, the etiological diagnosis consisted of 9 patients of Leber hereditary optic neuropathy (45%), 2 of dominant optic atrophy (10%), 6 of ethambutol-induced optic neuropathy (30%), 2 of nutritional optic neuropathy (10%) and 1 of idiopathic demyelinating optic neuropathy (5%). The patients with hereditary optic neuropathy showed a poorer visual prognosis, especially Leber hereditary optic neuropathy, with 78% of follow-up visual acuity (≥1 year) not higher than 0.1. The visual prognosis of ethambutol-induced optic neuropathy patients with mtDNA or OPA1 gene was poor. Conclusions: The optic neuropathy of visual field defects with central scotoma includes mainly hereditary, toxic and nutritional optic neuropathy. Hereditary optic neuropathy is characterized by incomplete penetrance, and genetic testing is required to exclude hereditary optic neuropathy if the visual field is the central scotoma.
光学相干断层成像(optical coherence tomography,OCT)自1991年发明以来,在生物成像尤其在眼科和心血流成像中起越来越重要的作用。OCT的发展经历了早期的时域系统及最新的频域系统。其中频域系统又分为谱域OCT(spectral domain OCT,SD-OCT)系统和扫频OCT(swept source OCT,SS-OCT)系统。随着眼科临床应用对系统速度、灵敏度及功能化要求的不断提升,眼科扫频OCT已经走向成熟并逐步商用化。本文将简介扫频OCT的原理,并归纳扫频OCT相对于时域和谱域OCT系统的优势,并展示其在眼科临床的应用。
Optical coherence tomography (OCT) has played an important role in biomedical imaging, especially in ocular and cardiovascular imaging. OCT technology has evolved to frequency domain technology from early time-domain technology due to the advantages of high sensitivity and high speed of frequency domain techniques. The swept source OCT is a type of frequency domain OCT. With the increasing requirements for system speed, sensitivity, and functionality in clinical application, swept source OCT is gradually becoming commercially available and widespread in clinical application. In this paper, the principle of swept source OCT was introduced, the advantages of swept source OCT over time domain and spectral domain OCT systems were summarized, and its clinical application in ophthalmology was demonstrated.
目的:探讨人工晶状体(IOL)预先巩膜悬吊在严重晶状体半脱位中的应用效果。方法:选取 2018年12月至2022年7月四川省人民医院收治的>180°的严重晶状体半脱位患者8例(8 眼)。术中避开脱位的晶状体,预先将IOL悬吊于玻璃体腔,再将晶状体托起置于IOL上方,必要时辅助以虹膜拉钩,稳定晶状体,确保超声乳化手术安全完成。结果:严重的晶状体半脱位患者8例,其中晶状体核N1-N3硬度的患者各1例,单独使用IOL预先巩膜悬吊于术中稳定脱位的晶状体,3例超声乳化手术均顺利完成;达N4患者3例、N5患者2例,其中4例在虹膜拉钩的辅助下安全完成超声乳化;有1例N5的患者,由于悬韧带损伤超过270°,在将晶状体托起放置于IOL之上时,坠入玻璃体腔,给予玻璃体腔超声粉碎处理。8例患者术后IOL均居中,视力有不同程度的提高,眼压正常,未见严重并发症。结论:在严重晶状体半脱位的超声乳化手术中,对于N2~N3的软核,IOL预先巩膜悬吊可以良好地稳定晶状体,确保超声乳化手术的顺利进行;对于N4~N5的硬核,IOL预先巩膜悬吊可以作为一种辅助方法,联合虹膜拉钩共同稳定晶状体,确保超声乳化手术的安全进行。
Objective: To investigate the effect of intraocular lens (IOL) pre-suspension in thetreatment of severe lens subluxation. Methods: Retrospective case study. From December 2018 to July 2022, 8 eyes of 8 patients with severe lens subluxation greater than 180 degrees admitted to our hospital were selected. During surgery, the IOL should avoid the subluxated lens and be pre-suspended in the vitreous cavity, and then the lens is lifted and placed above the IOL. If necessary, the iris hook can be used to stabilize the lens to ensure the safe completion of phacoemulsification. Results: There were 8 patients with severe subluxation of lens. Among them, the hardness of 3 patients' lens nucleus ranged from N1 to N3. In these 3 patients, we used the IOL pre-suspension alone to stabilize the subluxated lens, and phacoemulsification in these 3 patients was successfully completed. Three patients had N4 and 2 patients had N5, of which 4 patients underwent phacoemulsification safely with the assistance of iris hook. In another patient with N5, the lens fell into the vitreous cavity during surgery (the suspension ligament rapture greater than 270 degrees) when it was lifted and placed on the IOL which was crushed by the vitreous cavity ultrasound. After surgery, the IOL was centered in all 8 patients, visual acuity was improved to varying degrees, intraocular pressure was normal, and no serious complications were observed. Conclusions: In severe lens subluxation surgery, IOL presuspension in soft nuclei of N2 to N3 can stabilize the lens well and ensure the safety of phacoemulsification. For hard nuclei N4 to N5, IOL presuspension can be used as an auxiliary method in combination with iris hook to stabilize the lens and ensure the safety of phacoemulsification.
近年来随着人口老龄化的发展、人群用眼方式的改变,现有的眼科医疗资源正越来越难以满足日渐增长的医疗需求,亟需新型的诊疗模式予以补足。眼科人工智能作为眼科领域的新兴元素,在眼病的筛查诊断中发展迅速,主要表现为“眼部图像数据+人工智能”的模式。近年来,随着该模式在白内障、青光眼、糖尿病性视网膜病变(diabetic retinopathy,DR)等常见病中研究的深入,相关技术日渐成熟,表现出了较大的应用优势与应用前景,部分技术甚至成功转化并被逐渐应用于临床。眼科诊疗向智慧医学模式的过渡,有望缓解日益增长的医疗需求与紧缺的医疗资源之间的矛盾,从而提高整体的医疗服务水平。
The development of population aging and changes in the way people use their eyes over the recent years have increasingly challenged the existing ophthalmic medical resources to meet the growing medical needs, thus urgently calling for a novel diagnostic and treatment mode. Despite its status as an emerging sector in ophthalmology, ophthalmic artificial intelligence has developed rapidly in the screening and diagnosis of eye diseases, as can be seen in practices adopting the “eye imaging data + AI” mode. In recent years, with the intensified research on this mode with respect to common diseases such as cataract, glaucoma and diabetic retinopathy, relevant technologies have grown increasingly mature, presenting undeniable application superiority and prospects. Some of the relevant technical achievements have also been successfully transformed for practical usage, and are gradually being applied to clinical practices. Ophthalmic diagnosis and treatment are transitioning toward the era of intelligent medical services, which are expected to reduce the contradictions between the growing medical needs and the shortage of medical resources, as well as ultimately improve the overall experience of medical services.
慢性肝病(chronic liver disease, CLD)是一种或多种损伤因素长期作用于肝脏导致的疾病总称,其影响范围广、患者人群基数大。病毒性肝炎、非酒精性脂肪性肝病和终末期肝病等慢性肝病会累及眼底,造成视网膜渗出、出血等病变;同时眼底结构改变,如脉络膜和视网膜不同层次厚度,也与慢性肝病严重程度相关。对慢性肝病患者进行眼底检查不仅用于防治相关的眼底并发症,也对肝病临床评估及监测具有潜在应用价值。文章综述不同病因、严重程度和药物治疗下的慢性肝病患者可能出现的眼底病变,以及眼底结构功能检查在慢性肝病患者中的临床应用进展,以比较不同眼底检查方法在慢性肝病患者临床实施过程中的特点及适用场景,并提示未来在慢性肝病患者中应用眼底检查的潜在新方向。
Chronic Liver Disease (CLD) is a collective term for diseases resulting from the long-term effects of one or more damaging factors on the liver. It has a broad impact and affects a large patient population. Viral hepatitis, non-alcoholic fatty liver disease, and end-stage liver disease can involve the ocular fundus, leading to retinal exudates and hemorrhages. Additionally, structural changes in the fundus, such as the thickness of the choroid and different retinal layers, are associated with the severity of chronic liver disease. Through fundus examinations in patients with chronic liver disease, not only can ocular complications related to liver disease be prevented and treated, but these examinations may also offer potential value in the clinical assessment and monitoring of liver disease. This article reviews the potential ocular fundus abnormalities in patients with chronic liver disease under different etiologies, severities, and drug treatments. It discusses the progress in the clinical application of fundus structure and function examinations in patients with chronic liver disease. It compares the characteristics and appropriate clinical scenarios of various fundus examination methods in these patients and suggests potential new directions for the future use of fundus examinations in chronic liver disease management.
The congenital cataract is one of the leading causes of treatable childhood blindness. Existing classification systems for congenital cataracts are primarily utilized for the diagnosis of the disease. However, these systems provide limited information necessary for the evaluation, formulation, and optimization of treatment plans. Furthermore, research on the classification of congenital cataracts still requires exploration to provide additional evidence supporting molecular diagnosis and syndromic disease diagnosis. This paper reviews relevant studies on the classification of congenital cataracts and discusses the prospects for future research in this area.
The congenital cataract is one of the leading causes of treatable childhood blindness. Existing classification systems for congenital cataracts are primarily utilized for the diagnosis of the disease. However, these systems provide limited information necessary for the evaluation, formulation, and optimization of treatment plans. Furthermore, research on the classification of congenital cataracts still requires exploration to provide additional evidence supporting molecular diagnosis and syndromic disease diagnosis. This paper reviews relevant studies on the classification of congenital cataracts and discusses the prospects for future research in this area.
糖尿病视网膜病变(diabetes retinopathy, DR)是糖尿病常见的眼部并发症,其病理过程复杂,涉及多种细胞及炎症因子。Müller细胞作为视网膜主要支持细胞,在DR中不仅产生白介素-17(interleukin-17, IL-17),还作为其主要靶点发挥作用,通过谷氨酸代谢异常、血管内皮生长因子(vascular endothelial growth factor, VEGF)分泌增加及调控参与DR的病理过程,加重炎症反应。IL-17主要由辅助性T细胞17(T helper cell 17, Th17)分泌,通过促进多种炎症介质(如细胞因子、趋化因子和金属蛋白酶)的分泌,增强炎症反应,导致视网膜微血管损害和神经元凋亡,促进DR的发展。高糖环境下,Müller细胞功能受损,IL-17进一步加剧其功能障碍形成恶性循环。研究表明,阻断IL-17及核因子-κB激活剂1(Nuclear factor-kappa B activator 1, Act1)/肿瘤坏死因子受体关联因子6(tumor necrosis factor receptor associated factor 6, TRAF6)/核因子-κB(Nuclear factor-kappa B, NF-κB)信号通路可减轻DR的病理改变,为DR的治疗提供了新的思路。因此,深入研究IL-17与Müller细胞在DR中的相互作用机制,对于研究该疾病的发病机制及开发精准有效的治疗策略具有重要意义。
Diabetes retinopathy (DR) is a common ocular complication of diabetes, characterized by a complex pathological process involving multiple cells and inflammatory factors. Müller cells, as the primary supporting cells of the retina, not only produce interleukin-17 (IL-17) but also serve as a primary target in DR. They participate in the pathological process of DR by contributing to abnormal glutamate metabolism, increased secretion of vascular endothelial growth factor (VEGF), and regulatory functions, thereby exacerbating the inflammatory response. IL-17 is primarily secreted by T helper cell 17 (Th17) cells and enhances the inflammatory response by promoting the secretion of various inflammatory mediators (such as cytokines, chemokines, and metalloproteinases), leading to retinal microvascular damage and neuronal apoptosis, which accelerates the progression of DR. In a high-glucose environment, Müller cell function is impaired, and IL-17 further exacerbates this dysfunction, creating a vicious cycle. Studies have shown that blocking the IL-17 and Act1/TRAF6/NF-κB signaling pathways can mitigate the pathological changes associated with DR, providing new insights for the treatment of this disease. Therefore, conducting in-depth research on the interaction mechanism between IL-17 and Müller cells in DR is of great significance for exploring the pathogenesis of this disease and developing precise and effective treatment strategies.
Meibomian gland dysfunction (MGD) manifests through two main clinical presentations, characterized by the meibomian gland (MG) ductal obstruction or acinar dropout. While previous research has predominantly associated MGD pathogenesis with hyperkeratinization-related MG ductal obstruction and subsequent acinar atrophy, recent cases have shown significant functional acinar loss in the absence of apparent ductal keratinization or blockage. The deterioration of either MG obstruction or dropout exacerbates the condition of the other, suggesting an independent yet interconnected relationship that perpetuates the vicious cycle of MGD. Understanding the distinct pathological features of MG obstruction and dropout is crucial for delineating their etiology and identifying targeted therapeutic strategies. This review explores the nuanced interrelations of MG obstruction and dropout, elucidating potential pathological mechanisms to establish a foundation for early MGD diagnosis and intervention.
Meibomian gland dysfunction (MGD) manifests through two main clinical presentations, characterized by the meibomian gland (MG) ductal obstruction or acinar dropout. While previous research has predominantly associated MGD pathogenesis with hyperkeratinization-related MG ductal obstruction and subsequent acinar atrophy, recent cases have shown significant functional acinar loss in the absence of apparent ductal keratinization or blockage. The deterioration of either MG obstruction or dropout exacerbates the condition of the other, suggesting an independent yet interconnected relationship that perpetuates the vicious cycle of MGD. Understanding the distinct pathological features of MG obstruction and dropout is crucial for delineating their etiology and identifying targeted therapeutic strategies. This review explores the nuanced interrelations of MG obstruction and dropout, elucidating potential pathological mechanisms to establish a foundation for early MGD diagnosis and intervention.
前段巨眼(anterior megalophthalmos, AM)是一种罕见的双侧非进展性先天性眼前段增大疾病,表现为大角膜(直径≥ 12.5 mm)、前房极深、角膜厚度正常或轻中度变薄和睫状环扩大等。并发性白内障以及晶状体脱位是导致AM视力下降的主要原因。然而,解剖结构的异常使AM白内障手术具有很大的挑战性。文章报道了一例AM合并白内障的48岁男性患者,成功为其行手法小切口白内障摘除联合人工晶状体(intraocular lens, IOL)一期植入术,患者术后视力恢复良好,IOL位置居中,未出现较大的屈光误差。对该典型AM病例的临床特点以及手术难点的回顾总结,有助于加深广大眼科临床工作者对该疾病的认识。
Anterior megalophthalmos is a rare congenital enlargement of the anterior segment, characterized by bilateral nonprogressive megalocornea (diameter ≥12.5 mm), extremely deep anterior chamber, normal or moderate thinning of the cornea, and elongation of the ciliary ring. Cataract and lens dislocation are the main causes of decreased vision in patients with AM. However, cataract surgery on patients with AM are challenging due to the anatomical abnormalities. This case reports a 48-year-old male patient diagnosed with AM and cataract, who successfully underwent a manual small incision cataract extraction combined with intraocular lens implantation. Finally, our patient showed a good visual outcome with a well centered IOL and without obvious refractive error. In this typical AM case, we reviewed and summarized the clinical characteristics and the challenges of surgical treatment so that other ophthalmologists can learn about this disease.