先天性视盘发育异常的临床特征及鉴别诊断

目的 对先天性视盘发育异常的类型及临床特征进行分析评价.方法 单中心病例回顾性分析.纳入2013年5～10月在复旦大学附属眼耳鼻喉科医院神经眼科就诊后诊断为先天性视盘发育异常的患者,收集其人口学特征及临床特征进行回顾性分析.采用Humphrey静态自动视野、Goldmann视野、频域相干光断层扫描、眼底自发荧光、头颅磁共振扫描、头颅断层扫描和眼部超声进行辅助检查.结果 诊断为先天性视盘发育异常的患者共25例,其中男性13例;年龄中位数21岁（1～51岁）.受累眼共40只,双眼受累15例、单眼10例.视力从正常至无光感.诊断包括：视神经发育不良3例,节段性视神经发育不良6例,倾斜视盘6例,牵牛花综合征2例,视盘小凹2例,视盘玻璃疣3例,有髓神经纤维2例,视盘缺如合并视盘小凹及黄斑发育不良1例.常见误诊诊断：视神经炎5例,正常眼压青光眼4例,鞍区占位3例,颅高压3例,视盘血管炎2例.结论 先天性视盘发育异常病种繁多,常累及儿童及青年人,视功能损害差异较大.一些隐匿性视神经发育不良误诊率高.正确识别不同类型的视盘先天发育异常有助于临床解读患者的症状、体征和辅助检查结果,避免过度诊疗.     

原发性开角型青光眼视神经损伤不同阶段血清细胞因子水平分析及临床意义

目的：探讨原发性开角型青光眼（ POAG）视神经损伤不同阶段患者血清中各细胞因子的水平，并分析其临床意义。方法选择2012年8月至2014年8月在我院接受治疗的POAG患者97例（172只眼），作为观察组。再选择同期在我院眼科接受治疗的白内障患者50例，作为对照组。对比两组眼压及视力情况，对相关细胞因子水平进行对比分析。然后根据MD值分组得到A、B、C组，并对比三组间的眼压、视力、细胞因子水平等。结果观察组眼压、近视患者所占比例以及白细胞介素-4（ IL-4）水平显著高于对照组，而IL-6及IL-12水平低于对照组。观察组中根据不同视神经损伤程度分为A、B、C组发现A、B、C组眼压呈上升趋势，并且三组间眼压显著差异。并且A组近视比例低于C组，A组、B组IL-12水平显著高于C组，差异均有统计学意义（均P ＜0．05）。其他水平不存在显著差异（ P ＞0．05）。结论 POAG出现眼压升高、视力下降的可能性增大，其中IL-4会对视神经造成一定程度的损伤，相反IL-6以及L-12具有保护作用。此外，在POAG视神经不同损伤阶段仅有IL-12水平波动显著，说明相关的免疫应答反应在视神经损伤过程中的作用显著。     

Congenital anomalies of the optic disc

Although anomalies affecting the optic nerve head are usually clinically innocuous, they can sometimes cause significant symptoms and lead to visual loss. It is important to be able to recognize even the relatively benign lesions in order to differentiate them from other more threatening lesions or disease processes which they may clinically resemble. An awareness of the clinical appearance of disc anomalies is especially important in the differential diagnosis of optic nerve glaucomatous changes. Some anomalies cause various types of visual field loss which, if the actual disc lesion is not recognized, may lead to unnecessary neurologic evaluation or even to intracranial surgery. The optic nerve changes in acquired myopia and in the congenital tilted disc syndrome should be clearly defined and differentiated: high (pathologic) myopia may be highly progressive with many dangerous secondary sequelae, while the latter anomaly is stationary. Finally, there is a group of conditions, collectively termed “elevated anomalies of the disc,” which must be considered in the differential diagnosis of papilledema and potentially dangerous intraocular tumors, particularly retinoblastoma. This review provides a clinicopathologic correlation comparing the characteristics of the normal optic disc to those of the most important congenital anomalies of the disc.     

Diagnosis and Imaging of Optic Nerve Head Drusen

ABSTRACT

The presence of optic nerve swelling in pediatric patients is a frequent cause for referral to pediatric ophthalmologists and neuro-ophthalmologists because this finding can be the harbinger of serious neurologic disease including brain tumor, demyelinating disease, infiltrative disease of the optic nerve, or idiopathic intracranial hypertension. Optic nerve head drusen (ONHD) are common and can be particularly difficult to distinguish from true optic nerve swelling in pediatric patients because the ONHD are typically buried beneath the substance of the optic nerve. Correct identification of ONHD is relevant because of the visual morbidity associated with this condition and because of the need to distinguish pseudopapilledema secondary to ONHD from true optic nerve swelling. A variety of imaging modalities may be employed to evaluate for the presence of ONHD, including ultrasound, optical coherence tomography (OCT), enhanced depth imaging-OCT, fluorescein angiography, fundus autofluorescence, and optical coherence tomography angiography. To date, there is no consensus as to which of these techniques is most accurate and which should be part of a standardized evaluation for children suspected of ONHD. This review examines the recent literature analyzing these diagnostic tools and summarizes data regarding best practices for identifying ONHD.     

Fluorescein angiography in chronic simple and low-tension glaucoma.

Fluorescein angiograms were performed on a group of low-tension glaucoma and chronic simple glaucoma patients with similar extent of visual field loss, under standardised conditions, to see whether differences attributable to chronic intraocular pressure elevation could be detected. There was no evidence for difference in circulation times between these two groups. There was no evidence that hypoperfusion of the peripapillary choroid contributed to optic nerve hypoperfusion. Low-tension glaucoma patients demonstrated focal sector hypoperfusion of the optic nerve in every case, while the chronic simple glaucoma patients demonstrated a wide range of optic nerve fluorescence, suggesting both focal and diffuse optic nerve head hypoperfusion. It was concluded that, while focal hypoperfusion of the optic nerve may reflect susceptible vasculature at the nerve head with or without intraocular pressure elevation, diffuse hypoperfusion suggested that prolonged intraocular pressure elevation may simultaneously affect the whole of the optic nerve head. This could be a direct effect on blood vessels or a mechanical effect with secondary vascular changes.     

视神经损伤鼻内窥镜下视神经管减压术

目的 分析间接性视神经损伤的特点以及鼻内窥镜下视神经管减压术的手术方法。方法 介绍1998－1999年我院施行鼻内窥镜下视神经管减压术6例（6眼），比较手术前后的视力、瞳孔变化。结果 6例术后均维持和提高视力，肯定鼻内窥镜下视神经管减压术能改善间接损伤的视神经功能。结论 鼻内窥镜下视神经管减压术优于其他手术进路的视神经管减压术，避免诸多并发症及其危险。     

眶尖部及视神经肿瘤的早期诊断

目的探讨眶尖部及视神经肿瘤在眼球突出前的临床表现、影像学检查特征及临床诊断要点。方法回顾性分析22例无明显眼球突出单侧眼眶肿瘤患者的病历资料,包括主要症状、首发表现、临床诊治过程及影像学检查结果。结果22例肿瘤患者中,海绵状血管瘤6例,神经鞘瘤、视神经鞘脑膜瘤、鼻窦及鼻咽腔恶性肿瘤眶内蔓延各4例,后组筛窦黏液囊肿2例,神经纤维瘤和视神经胶质瘤各1例。以视力下降为首发症状者17例,视力下降合并复视者3例。全部患者均在CT或MRI检查后确诊。肿瘤位于眶尖部或起源于视神经。结论眶尖部及视神经肿瘤的早期症状是视功能障碍,无眼球突出表现。视力呈缓慢进行性下降,按视神经炎治疗效果差。CT或MRI检查对早期诊断起重要作用。     

Optic nerve head drusen

Optic disc drusen are congenital and developmental anomalies of the optic nerve head seen commonly in clinical practice, often as an incidental ophthalmologic finding during routine exams. Optic disc drusen are a form of calcific degeneration in some of the axons of the optic nerve. Visual acuity is often not affected but the visual fields of these patients can be abnormal and deteriorate over time. Optic disc drusen are familial and are not uncommon. They are thought to be the result of pathology at the level of the optic nerve head itself. The diagnosis can be made with clinical findings combined with B scan ultrasound and computed tomography. In addition, newer modalities using optic nerve head tomography are proving to be very useful. Since children as well as adults are affected, it is important to consider optic nerve head drusen in the differential diagnosis of papilledema or optic nerve swelling.     

Optic nerve head drusen

Optic disc drusen are congenital and developmental anomalies of the optic nerve head seen commonly in clinical practice, often as an incidental ophthalmologic finding during routine exams. Optic disc drusen are a form of calcific degeneration in some of the axons of the optic nerve. Visual acuity is often not affected but the visual fields of these patients can be abnormal and deteriorate over time. Optic disc drusen are familial and are not uncommon. They are thought to be the result of pathology at the level of the optic nerve head itself. The diagnosis can be made with clinical findings combined with B scan ultrasound and computed tomography. In addition, newer modalities using optic nerve head tomography are proving to be very useful. Since children as well as adults are affected, it is important to consider optic nerve head drusen in the differential diagnosis of papilledema or optic nerve swelling.     

外伤性视神经病变临床分析

目的 探讨外伤性视神经病变的诊断方法及治疗效果。方法 回顾32例35眼视神经损伤的诊断，手术治疗和药物治疗后的视力恢复情况。进行总结分析。结果 视神经损伤7天之内就诊者，无光感15眼仅2眼视力提高，光感以上者16眼视力均有不同程度的提高。8-20天就诊者，1例（2眼）无变化，2例行视神经管减压术视力提高。结论 外伤性视神经病变以CT检查有视神经受压迫的阳性征象者，不论有无光感均应及早手术；无阳性征象者及早药物治疗，多途径给药，效果较好。伤后立即失明者治疗效果差。     

视神经胶质瘤的诊治研究现状及进展

视神经胶质瘤（ONG）是一种好发于儿童和青少年且相对罕见的中枢神经系统肿瘤,主要病理类型为低级别的毛细胞型星形细胞瘤。其分为散发和1型神经纤维瘤病（NF-1）相关的ONG。由于ONG与视神经的紧密关系,在诊疗上存在其特殊性,其诊断主要依靠病史、症状和体征,以及磁共振成像和CT等影像检查。ONG应与神经鞘脑膜瘤、视神经炎...     

Stereoacuity testing in patients with retinal and optic nerve disorders

A study comparing the relative sensitivity for detecting abnormal stereoacuity in patients with retinal or optic nerve disease on clinically used stereoacuity tests is not available. It is also not apparent from the ophthalmic literature if optic nerve or retinal diseases are likely to have a greater impact on stereoacuity performance. We were also interested in determining a level of visual acuity loss that would likely result in an impairment of stereoacuity on these clinical tests. Forty-two patients with various retinal and optic nerve disorders and eighteen normal subjects were evaluated for stereoacuity using three tests: Titmus Stereoacuity Test (TST), Randot Stereoacuity Test (RST), and TNO Stereoacuity Test (TNO). The performance on these three stereoacuity tests was compared with the normal subjects. Additionally, TST scores from our patients were compared to predicted TST scores derived from a previously published nomogram. For patients with retinal and optic nerve disease, an abnormal score on one clinical test of stereoacuity was likely to predict an abnormality on the other tests. Performance on the TST relative to the predicted value derived from a nomogram was not significantly different for patients with retinal vs. optic nerve disease. With some exceptions, patients with visual acuities of 20/30 or worse in at least one eye were likely to show abnormal stereoacuity.This study was supported by a center grant from The Foundation Fighting Blindness, Baltimore, Maryland     

Septo-optic dysplasia: a literature review.

BACKGROUND: Septo-optic dysplasia (SOD) is a rare disorder characterized by optic nerve hypoplasia with any combination of absent septum pellucidum and/or pituitary dysfunction. SOD may manifest as strabismus, nystagmus, decreased visual acuity, or visual impairment; as an endocrine dysfunction in isolation; or in addition to mental retardation, cerebral palsy, developmental delay, or delayed growth. METHOD: This article reviews the presenting signs and symptoms of SOD, optic nerve hypoplasia, consequences of an absent septum pellucidum, endocrine findings associated with SOD, SOD diagnosis determination, syndromes associated with SOD, and optometry's role in caring for these patients. It also examines two cases that demonstrate the variety and severity of visual and physical impairments associated with SOD. RESULTS: SOD has a multi-factorial etiology, including insult during pregnancy (e.g., viral infections, gestational diabetes); vascular disruption; or a genetic mutation. Children with SOD may manifest a variety of visual and/or physical symptoms that range from mild to severe. CONCLUSIONS: The associated vision, developmental, neurologic, and endocrine disturbances require early diagnosis and management. Optometrists need to be aware of optic nerve hypoplasia (ONH) and consider this diagnosis in patients with visual acuity loss. A comprehensive eye examination and visual-field assessment should be completed in addition to appropriate referrals for endocrine, developmental, and/or cognitive anomalies.     

Anterior ischemic optic neuropathy in association with optic nervehead drusen

Optic nerve head drusen (ONHD) are incidental ophthalmologic finding in the optic nerve. Patients with ONHD are often asymptomatic, but sometimes present with transient visual obscuration''s (TVO), the reported incidence of which is 8.6%. Optic nerve head drusen are of two types: Superficial; visible and deep. The deep-buried drusen mimic papilledema. Because of the varied presentation deep-buried drusen pose a diagnostic challenge to the ophthalmologists. In young patients, they are mistaken for papilledema as it is clinically difficult to detect a buried drusen in the optic nerve head, but are seen on the surface with aging as the retinal nerve fiber layer thins out. They are observed as pale yellow lesions more often located towards the poles. Clinical examination aided with diagnostic tests like computed tomography (CT) orbits and ultrasound B scan can help establish the diagnosis. Herein, we report a rare case of optic nerve head drusen in a young lady, who presented with loss of vision and clinical evaluation and investigations suggested ONHD with anterior ischemic optic neuropathy.     

Transvitreal optic neurotomy for nonarteritic anterior ischemic optic neuropathy

PURPOSE: To evaluate the role of transvitreal optic neurotomy in the treatment of nonarteritic anterior ischemic optic neuropathy, a scleral outlet compartment syndrome, in which neurovascular compression at the prelaminar and laminar regions of the optic nerve head may play a major role. METHODS: Seven eyes of seven selected patients with severe vision loss (<20/800) from nonarteritic anterior ischemic optic neuropathy underwent transvitreal nasal radial optic neurotomy. The study was not masked and not randomized. Visual acuity and visual fields, when possible, were measured, and fluorescein angiography was performed preoperatively and postoperatively. RESULTS: Four male and three female patients had a mean age of 52.4 years; five had bilateral disease. The mean follow-up was 13 +/- 7 weeks. Mean preoperative visual acuity was 20/2400; mean postoperative visual acuity was 20/250, with an average of 10 lines of improvement. Six of seven patients showed visual improvement. One patient had peripapillary choroidal neovascularization. In two patients with sufficient visual acuity, preoperative visual fields could be obtained; these patients showed significant improvement in postoperative perimetry. Five patients had some loss of vision, which made it impossible to obtain preoperative visual fields. CONCLUSION: Relaxation of the scleral ring of the prelaminar and laminar regions of the optic nerve head reduces constriction and may prevent necrosis of salvageable but underperfused nerve fibers. Despite improvement of visual acuity in our patients, transvitreal optic neurotomy should be considered experimental, requiring a randomized clinical trial.     

外伤性视神经病变26例临床分析

目的探讨外伤性视神经病变的诊断方法及治疗效果。方法对5年间收治的26例（28只眼）视神经损伤患者的临床特点与治疗效果进行总结分析。结果无光感21只眼有5只眼视力提高,光感以上7只眼均有不同程度提高。6只眼行视神经管减压术,其中2只眼仍无光感,4只眼视力提高。结论外伤性视神经病变多见于车祸头部外伤后,应尽早用大剂量糖皮质激素和脱水剂及血管扩张剂等治疗,早期视神经管减压术效果好。伤后立即失明者效果差。     

Visual evoked potentials during the early phase of optic nerve compression in the orbital cavity

We obtained case histories and electrophysiologic recordings from four patients with transient vision impairment due to acute orbital compression. The visual evoked potentials (VEPs) displayed alterations that depended on the size and consistency of the compressing pathology and also on the duration of the compression. This study provides evidence of the utility of the VEP in the assessment of the severity and reversibility of optic nerve lesions. The case histories also emphasized the necessity to elucidate the pathologic process of compressive lesions of the optic nerve.     

Limitations of the Heidelberg Retina Tomograph.

Glaucoma is a progressive optic neuropathy where damages that occur at the optic nerve head and the retinal never fiber layer are associated with changes in the visual field. The Heidelberg Retina Tomograph (Heidelberg Engineering, Carlsbad, CA), a scanning laser ophthalmoscope, performs a three-dimensional topographic analysis of the optic nerve head and the retinal never fiber layer. The Heidelberg Retina Tomograph has been reported to be a reliable and objective method of identifying structural changes prior to the occurrence of visual field defects. In our series of patients, all had significant visual field defects but normal Heidelberg Retina Tomograph results, including rim area and Moorfield regression analysis. It is important to recognize that interpreting the Heidelberg Retina Tomograph results alone may be misleading; however, when combined with careful clinical evaluation of the optic nerve head and the visual field, the Heidelberg Retina Tomograph may provide valuable information in assisting the clinician with the diagnosis and management of glaucoma.     

Bilateral retrobulbar hemorrhage and visual loss following traumatic asphyxia

Retrobulbar hemorrhage and permanent visual loss are rare presentations following traumatic asphyxia. In this case, bilateral permanent visual disturbance developed in a woman after chest-crushing trauma without direct trauma to the orbits. A computed tomography scan confirmed bilateral retrobulbar hemorrhages. An ophthalmologic exam revealed bilateral subconjunctival hemorrhages and severe lid edema. Despite high-dose steroid therapy, visual recovery was limited, and optic nerve atrophy developed. Ischemia of the optic nerve associated with retrobulbar hemorrhage may be postulated as one of the causes of permanent visual impairment following traumatic asphyxia.     

视觉电生理技术在青光眼早期诊断中的意义

青光眼是一组威胁和损害视神经及其视觉通路,最终导致视觉功能损害的疾病。青光眼视神经损害以视网膜神经节细胞( retinal ganglion cells,RGCs)及其轴突数目进行性丢失、视盘凹陷、视野缺损为特征。静态自动视野计检查是评估青光眼病程进展的金标准,但其结果往往受到患者主观因素的限制。近年来视觉电生理技术飞速发展,虽还不能代替传统的视野检查,但作为补充,其能在青光眼视野缺损前即出现振幅及潜伏期的改变,为早期青光眼的诊断提供了新的参考思路。本文主要对三种特殊类型的视网膜电图以及多焦视觉诱发电位在青光眼早期诊断中的意义进行综述。