Hallermann-Streiff 综合征继发青光眼:病例系列报告与文献回顾

Glaucoma in Hallermann-Streiff syndrome: case series report and literature review

 
目的:分析Hallermann-Streiff综合征(Hallermann-Streiff syndrome,HSS)继发性青光眼的临床表现,探讨其治疗方法。方法:采用病例系列研究与文献回顾方法,记录3例确诊为HSS继发性青光眼患者的视力、眼压、裂隙灯、超声生物显微镜、相干光断层扫描、角膜地形图、A超、B超、X线眼眶大小测量等检查结果。随访患者药物治疗、周边虹膜切除术、小梁切除术或青光眼阀植入术的疗效。结果:3例患者年龄分别为9、29和47岁,其中女性2例、男性1例。最佳矫正视力为0.04-0.5,平均屈光度为+12.1D,平均眼压为37.7 mmHg,平均角膜直径为9.1 mm,平均中央前房深度为2.43 mm,平均眼轴长度为18.13 mm,角膜地形图示平均K1值为56.97 D,平均K2值为60.65 D。眼眶水平径为28.86~31.40 mm,垂直径为30.16~32.90 mm。2例年轻患者为无晶状体眼,伴葡萄膜炎、瞳孔区纤维膜、视盘旁脉络膜萎缩弧。年长患者表现为蓝色巩膜、白内障、房角关闭,眼底表现为青光眼性视杯凹陷。3例患者平均身高143 cm,伴头发及眉毛稀疏、额头前凸、鼻子呈喙状、牙齿发育不全、下颌发育不全。术后平均随访47.7个月(范围:11~84个月),眼压控制,视力与术前一致,无治疗相关并发症出现。结论:HSS继发性青光眼的眼部病变可表现为小眼眶、小眼球、小角膜、蓝色巩膜、无晶状体、瞳孔区纤维膜、葡萄膜炎、继发性青光眼及视盘旁脉络膜萎缩。对HSS继发性青光眼的患者,个性化地选择治疗方案,可以获得较好的治疗效果。
Objective: To demonstrate the clinical characteristics and surgical effects of glaucoma in Hallermann-Streiff syndrome(HSS). Methods: Observational case series and literature review. The results of ophthalmic examinations of three patients diagnosed as glaucoma with HSS were recorded, including visual acuity, intraocular pressure (IOP), slit-lamp microscopy, ultrasound biomicroscopy, optical coherence tomography, corneal topography, A-scan and B-scan ultrasonography, and orbital size measurement by X-ray. Peripheral iridectomy, glaucoma drainage device implantation or trabeculectomy, were performed in these patients. Results: Three HSS patients were 9, 29 and 47 years old, respectively, including 2 females and 1 male. The best corrected visual acuity was 0.04-0.5. The mean spherical equivalent refraction was +12.1 D. The average IOP was 37.7 mm Hg, and the average corneal diameter was 9.1 mm. The average central anterior chamber depth was 2.43mm. The average axial length was 18.13mm. Keratometry showed average K1 of 56.97 degrees, and K2 of 60.65 degrees. Two younger patients were aphakic bilaterally with uveitis, pupillary fibrous membrane and peripapillary choroidal atrophy. The older patient showed blue sclera, cataract, and anterior chamber angle closure. The horizontal orbital diameter was 28.76-31.40 mm, and vertical orbital diameter was 30.16-32.90 mm. All patients were proportionate nanism, with an average height of 143 cm. Craniofacial manifestations included dyscephalia and “bird-like” face, hypotrichosis, dental anomalies, and mandibular hypoplasia. They were followed up for an average of 47.7 months(range:11-84 months) after surgery. The IOPs were all controlled, and the visual acuities remained unchanged. No treatment-related complications occurred. Conclusions: HSS patients with glaucoma may present as small orbit, microphthalmia, microcornea, blue sclera, aphakia, pupillary fibrous membrane, uveitis, with atrophic chorioretinal changes. For these patients, personalized treatment may help to achieve better therapeutic effects.

基于眼科前沿技术研究和发展的综述

A review of cutting-edge research and development in ophthalmology

 
数字眼科技术的蓬勃发展已经渗透于眼科各领域,并为眼科前沿技术研究带来了重大变革。文章对眼科前沿技术研究和发展进行综述,重点关注各项技术的突破和成果,聚焦国内的同时也放眼全球,阐明近年来全球数字眼科前沿技术现状和成果。如人工智能可运用于诸多疾病的精准监测、高发疾病的高效评估、远程医疗的技术支持等,提高了筛查、诊断、治疗等各环节的精确度和效率,减轻了医生的负担,展现了极大的应用潜能。其余各类技术方面,如数字成像技术也取得了飞跃式进展,在手术导航和影像诊断方面实现突破;3D建模技术和机器学习技术在手术设计和提高手术成功率方面立下汗马功劳,为世界各地的眼科疾病患者提供了更为高效、便捷的服务。此外,数字眼科技术还呈现出多元化发展的态势,与多学科协同交流,交叉运用。虽然诸多眼科前沿技术还处在发展初期,距离全覆盖实施仍有一定距离,在算法的准确性和可解释性、医疗伦理、民众接受度、医疗纠纷、临床技术挑战等问题上还存在很多不确定性。但毫无疑问的是,随着医学水平的日益提升,上述技术必会得到不断完善和普及。
The rapid evolution of digital ophthalmology technology has profoundly transformed various fields within ophthalmology. This article provides a comprehensive overview of the research and advancements in cutting-edge ophthalmology technologies, emphasizing both domestic and global breakthroughs and achievements. For instance, artificial intelligence has demonstrated remarkable potential in precise monitoring of various diseases, efficient assessment of high-prevalence conditions, and the technological support for telemedicine, thereby enhancing the accuracy and efficiency of screening, diagnosis, treatment processes, while alleviating the workload of medical professionals. Additionally, digital imaging technology has made significant strides in surgical navigation and diagnostic imaging, while 3D modeling and novel machine learning techniques have contributed to surgical planning and enhanced surgical success rates, ultimately delivering more efficient and convenient services to patients with eye diseases worldwide. Despite the diversified development trends and interdisciplinary collaborations that digital ophthalmology technology exhibits, many of these cutting-edge technology are still in their infancy, facing challenges in achieving high coverage, algorithm accuracy and interpretability, medical ethics, public acceptance, medical disputes, and clinical technical hurdles. Nonetheless, while the continuous advancement of medical standards, it is anticipated that these technologies will undergo further refinement and widespread adoption.

重视先天性白内障术后高眼压和青光眼的防治

Prioritizing Prevention and Treatment of High Intraocular Pressure and Glaucoma Following Congenital Cataract Surgery

 
先天性白内障是严重影响婴幼儿视功能的疾病。随着白内障手术和人工晶体植入手术技术的发展,先天性白内障患者术后多可获得高质量的视觉康复。然而,如何更好防治手术相关的不良事件和并发症、先天性白内障伴随的其他眼部发育不良疾病的治疗以及形觉剥夺性弱视的治疗,仍然是先天性白内障手术后需要重视的临床问题。文章对先天性白内障摘除及人工晶体植入术后高眼压和继发性青光眼的发生、相关危险因素、治疗和预防的手段进行总结,以期进一步提高对先天性白内障术后高眼压和青光眼防治的认识,减少术后并发症对视功能造成的进一步损害。
Congenital cataract is a significant condition that profoundly impacts the visual function of infants and young children. Advancements in cataract surgery and intraocular lens implantation have enabled the achievement of high-quality visual rehabilitation after congenital cataract surgery. Nevertheless, effective prevention and treatment of surgery-related adverse events and complications, as well as managing other ocular dysplasia and form deprivation amblyopia that may arise in conjunction with the surgery, continue to pose important clinical challenges following congenital cataract surgery. This article provides a comprehensive overview of the occurrence, risk factors, treatment and prevention of high intraocular pressure and secondary glaucoma after congenital cataract and intraocular lens implantation. Its aim is to enhance the comprehension of preventive and therapeutic measures for high intraocular pressure and glaucoma after congenital cataract surgery, thereby minimizing potential postoperative complications and preserving visual function.

角膜屈光手术对角膜生物力学影响的研究进展

Research progress on the effect of corneal refractive surgery on corneal biomechanics

 
角膜屈光手术是目前屈光手术的主流术式,随着全飞秒、全激光手术方式的发展,手术变得更加安全精准,不仅角膜创伤小,术后恢复时间也进一步缩短。角膜具有屈光特性和典型的生物软组织力学特性,角膜力学特性不仅参与维持角膜形态,影响角膜手术尤其屈光手术的效果及预后,而且还与部分角膜疾病的发生和发展密切相关。近年来生物力学研究发展迅速,其在眼部疾病的诊疗中发挥着越来越重要的作用。角膜生物力学的变化与术前角膜的形态、不同手术方式的选择、术后角膜厚度的改变等多种因素相关,但手术导致的角膜自身形态改变是不可逆的,若术后角膜生物力学的变化较大,可能会引起医源性角膜扩张、继发性圆锥角膜等并发症的发生。为了规避术后角膜扩张风险和指导个性化的术式选择,了解角膜生物力学特性的影响至关重要。文章对角膜的基础结构、角膜生物力学特性、生物力学测量方法和不同术式及不同角膜瓣厚度术后生物力学变化的研究进展进行综述,为近视患者的个性化精准治疗提供理论指导。
Corneal refractive surgery is currently main stream of refractive surgery. With the development of femtosecond and laser surgery, the surgery has become safer and more accurate, resulting in less corneal trauma and a shorter postoperative recovery time. In recent years, biomechanics research has rapidly progressed, and its clinical application has gradually increased. The cornea not only possesses refractive properties but also exhibits typical biological soft tissue mechanical properties. Corneal mechanical properties not only play a role in maintaining corneal morphology but also influence the outcome and prognosis of corneal surgery, especially refractive surgery, and are closely related to the occurrence and development of some corneal diseases. Corneal refractive surgery involves cutting the cornea according to the patient's diopter, which disrupts the integrity of the cornea and inevitably affects its biomechanical stability. Changes in corneal biomechanics are associated with various factors, such as preoperative corneal morphology, the selection of different surgical methods, and postoperative changes in corneal thickness. However, the self-morphology changes caused by surgery are irreversible. If the postoperative changes in corneal biomechanics are significant, it may lead to complications such as postoperative corneal dilation and secondary keratoconus. To avoid postoperative iatrogenic corneal dilation and guide personalized surgical choice, it is crucial to understand the limits of influence of corneal biomechanical properties. This article reviews the research progress regarding corneal biomechanical properties and changes associated with corneal refractive surgery.

眼移植物抗宿主病的临床诊疗新进展

Research progress on clinical diagnosis and treatment of ocular graft-versus-host disease

 
随着移植技术逐年发展,异基因造血干细胞移植患者的生存期延长,长期并发症成为影响患者预后及生活质量的主要原因。眼移植物抗宿主病是异基因造血干细胞移植术后最常见的眼部并发症,发生率可高达50%以上。根据发病时间可分为急性及慢性眼移植物抗宿主病,临床上最常以慢性炎症及眼表组织纤维化为特点,主要表现为干眼和不同程度的角结膜炎,治疗较为棘手,可不同程度影响患者视觉质量及生活质量,严重可致盲。近年来眼移植物抗宿主病越来越受到国内外学者重视,其发病机制、临床特点、诊断及治疗相关研究逐渐深入,文章针对眼移植物抗宿主病的临床诊疗新进展进行综述。总体而言,眼移植物抗宿主病早期识别仍较为困难,早期诊断策略有待进一步探索。目前治疗对眼移植物抗宿主病的效果较为有限,或缺乏充足的循证医学证据,临床上缺乏针对不同严重程度及疾病活动度的分级诊疗策略,未来有待进一步探索新的治疗靶点及疾病活动监测指标,将有助于改善患者长期预后及生活质量。
Despite advancements in allogeneic hematopoietic stem cell transplantation techniques leading to improved overall survival rates, long-term complications have emerged as the primary contributors to poor prognosis and diminished quality of life. Ocular graft-versus-host disease (oGVHD), a prevalent complication affecting over 50% of patients post-transplantation, frequently manifests as refractory dry eye, often accompanied by keratoconjunctivitis. Patients with oGVHD routinely suffer from visual impairment and a decline in their quality of life.Currently, research into the mechanisms, clinical features, diagnosis, and treatment of oGVHD has progressively deepened. This article reviews the latest advancements in the clinical diagnosis and management of oGVHD. Notably, there is a pressing need for strategies focused on early diagnosis and treatment, as early recognition of oGVHD remains challenging. Existing treatments for oGVHD either exhibit limited efficacy or lack robust clinical evidence to support their use as the best available options.Further research is imperative to develop tiered diagnostic and treatment approaches, including the exploration of novel therapeutic targets and biomarkers for disease detection. Such endeavors hold the promise of enhancing patients' long-term prognosis and quality of life.

眼中洞见:人工智能解码全身健康

Insights from the eye: artificial intelligence decodes systemic health

 
人工智能(artificial intelligence, AI)在医学领域的广泛应用为探索眼部与全身健康的关系提供了新的机遇。文章回顾了眼科AI在心血管健康、神经系统健康、肾脏健康和衰老过程中的应用。在心血管健康方面,AI能够通过分析眼底图像预测心血管疾病风险因素和未来心血管事件,并提供了简便、有效的风险分层方法。在神经系统健康方面,眼科AI在阿尔茨海默病早期诊断和帕金森病识别方面显示出潜力,尽管对未来事件预测仍具挑战性。针对多发性硬化,眼科AI在诊断和预测残疾进程上展现了良好效果。在肾脏健康中,眼科AI技术通过分析视网膜图像可预测肾功能相关指标、直接检测肾病事件,展示了其在改善肾病筛查方式和减轻医疗负担方面的潜力。在衰老过程中,AI能够利用眼部图像预测生物年龄、视网膜年龄差和晶状体年龄等参数提供了生物衰老指标,为理解衰老与眼部健康的关联提供了新视角。
The widespread application of artificial intelligence (AI) in the medical field has provided new opportunities to explore the relationship between eye and whole body health. This article reviews the application of ophthalmic AI in cardiovascular health, neurological health and aging. In terms of cardiovascular health, AI can predict cardiovascular disease risk factors and future cardiovascular events by analyzing fundus images, and provides a simple and effective risk stratification method. In terms of neurological health, ophthalmic AI shows potential in early diagnosis of Alzheimer's disease and identification of Parkinson's disease, although the prediction of future events remains challenging. For multiple sclerosis, ophthalmic AI has shown good results in diagnosing and predicting the progression of disability. In kidney health, ophthalmic AI technology can predict kidney function-related parameters and detect kidney disease events by analyzing retinal images, demonstrating its potential in improving kidney disease screening methods and reducing medical burdens. In the aging process, AI can use eye images to predict biological age. Parameters such as retinal age gap and LensAge provide biological aging indicators, providing a new perspective for understanding the relationship between aging and eye health.
其他期刊
  • 眼科学报

    主管:中华人民共和国教育部
    主办:中山大学
    承办:中山大学中山眼科中心
    主编:林浩添
    主管:中华人民共和国教育部
    主办:中山大学
    浏览
  • Eye Science

    主管:中华人民共和国教育部
    主办:中山大学
    承办:中山大学中山眼科中心
    主编:林浩添
    主管:中华人民共和国教育部
    主办:中山大学
    浏览
推荐阅读
出版者信息