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.
后视路病变是视交叉以后的视觉通路其本身或毗邻结构发生病变，引起视觉功能改变的一类疾病。神经眼科医生比较熟悉枕叶病变引起的对称性同侧偏盲，但枕极(纹状皮质的最后部分)的病变产生中心性对称性同向盲点，此类视野改变容易被忽略或误诊。该文报道一例老年男性患者，因双眼视觉清晰度下降、视物变形就诊。眼科检查：最佳矫正视力：右眼0.8，左眼1.0，FM-100检查提示重度色觉异常，颅脑磁共振成像(magnetic resonance imaging,MRI)提示双侧枕叶脑梗死(右侧枕极部，左侧纹状皮质前部)，24-2 Humphrey视野检查可见双眼同向暗点趋势(不典型)，10-2 Humphrey视野检查可见双眼中心视野同向偏盲(暗点)，故而确诊。后视路病变可引起多种特征性的视野改变，可伴有高级视功能异常及其他神经系统症状和体征，是神经眼科的重要组成部分。该例枕极脑梗死病变产生对称性同向性盲点伴色觉改变患者的诊治过程，提示需关注后视路病变视野改变的多样性及其他视觉功能异常，提高早期诊断率，改善患者预后。
The disease of the posterior visual pathway is a kind of lesion in which the visual pathway itselfor its adjacent structure changes after optic chiasma causes pathological changes, resulting in changes in visual function. Neuro-ophthalmologists are familiar with symmetrical ipsilateral hemianopia caused by occipital lobe lesions, but occipital tip (the last part of the striatal cortex) lesions produce central symmetrical homonymous scotomas, which can easily be overlooked or misdiagnosed. This article reported a case of an olderly male patient treated with decreased binocular visual clarity and distortion. Ophthalmology examination: best corrected visual acuity: 0.8 in the right eye, 1.0 in the left eye; FM-100 examination indicated severe dyschromatopsia; cranial magnetic resonance imaging: infarction of bilateral occipital lobe (right portion of the occipital tip and left anterior portion of striate cortex); 24-2 Humphrey field examination showed a tendency of homonymous scotoma in bilateral eyes (atypical); 10-2 Humphrey field examination showed homonymous hemianopia (scotoma) in the central visual field. These results confirm a diagnosis of the disease of the posterior visual pathway. As an important part of neuro-ophthalmology, the posterior visual pathway can cause various characteristic visual field defects, which can be accompanied by advanced visual dysfunction and other neurological symptoms and signs. The diagnosis and treatment process of this case of occipital tip cerebral infarction with symmetrical homonymous blind spot accompanied by color vision changes suggests that attention should be paid to the diversity of visual field changes and other visual functional abnormalities in the posterior visual pathway lesions, so as to improve the early diagnosis rate and prognosis of the patient s.
目的：评估与原发性慢性青光眼患者生活质量最具相关性的视觉指标。方法：回顾性病例系列研究。收集2010年3月至2010年8月就诊于首都医科大学附属北京同仁医院且符合入选及排除标准的原发性慢性闭角型青光眼和原发性开角型青光眼患者131例262只眼。采用Humphrey Field AnalyzerII 750i型视野分析仪对入选患者分别进行单眼Central 24-2程序和Esterman双眼视野检查程序(Esterman Binocular Visual Field Test，EBVFT)各两次检查，以保证所得数据的可靠性。使用LogMAR视力表检查并记录受试者日常单眼生活视力和日常双眼生活视力、非接触式眼压计测量眼压、直接眼底镜检查视神经的杯盘比。记录病史及目前所使用抗青光眼药物，角膜及晶状体状态。应用视功能相关生活质量量表-25(25-Item National Eye Institute Visual Function Questionnaire，VFQ-25)对患者进行生活质量评估，并将双眼日常生活视力、较好眼生活视力、较差眼视野MD(mean defect)值、较好眼视野MD值、(Esterman Visual Field Test，EVFT)效用值和VFQ-25得分进行相关性分析。结果：共有131例受试者符合入选条件。在患者生存质量的统计中，自我评价视力、近距离活动、远距离活动、社会功能、依赖程度、色觉、视野这7项得分较好，即受试者完成该7项的能力较高。VFQ-25总分与EBVFT效用值成正相关(r=0.24998，P=0.004<0.05)，与双眼日常生活视力成负相关(r=?0.37778，P<0.0001)，与较差眼视野MD值成正相关(r=0.22917，P=0.0187<0.05)，与较好眼生活视力、较好眼视野、较差眼生活视力无明显相关关系。结论：原发性慢性青光眼患者双眼视觉(双眼视野和双眼日常生活视力)和VFQ-25有良好的相关性，可用于评估慢性青光眼患者的生活质量。
Objective: To assess the correlation between the Esterman binocular visual field (EBVF) and the visual function quality of life (VFQ) in primary chronic glaucoma patients. Methods: It is a retrospective case series study.One hundred and thirty-one patients, that is to say, two hundreds and sixty-two eyes, with primary chronic glaucoma satisfying the methodological criteria were recruited for this study, who were chosen from Beijing Tongren Hospital, Capital Medical University from March 2010 to August 2010. The Humphrey Field Analyzer Model II 750i (Humphrey Instruments, Zeiss Company) was used to perform an Esterman binocular visual field test (EBVFT) and bilateral monocular full-threshold central visual field testing using the 24-2 SITA Standard program. Visual acuities were examined by logarithm of the minimum angle of resolution (logMAR) scale. Intraocular pressure and cup-disc ratio were also recorded. The history with glaucoma and anti-glaucoma drugs, and the state of the cornea and crystalline lens were also remarked. All interviews were administered face to face by the same experienced interviewer, by using the 25-Item National Eye Institute Visual Function Questionnaire (VFQ-25). Agreement between the scores from these tests and the VFQ-25 was evaluated. Results: A total of 131 patients were recruited. In the statistics of the quality of life, seven domain scores of the VFQ-25 (self-assessment vision, color vision, near action, distant action, social function, mental health and peripheral vision) were better than the others, which meant the ability of completing the seven domain was higher. Substantial agreement was found between the composite score of the VFQ-25 and the Esterman test (r=0.24998, P=0.004<0.05), strong negative correlation was found between the composite score of the VFQ-25 and the binocular visual acuity of daily life (r=?0.37778, P<0.0001), and positive correlation was found between the composite score of the VFQ-25 and the MD of the worse eye (r=0.22917, P=0.0187<0.05).Conclusion: In this sample of clinic-based patients with primary chronic glaucoma, the efficiency score of the binocular visual field tests correlated well with the composite score of the VFQ-25. Binocular visual function can be well used in evaluating the quality of life of the glaucoma patients.
目的：探讨原发性慢性闭角型青光眼(chronic primary angle-closure glaucoma，CPACG)患者的视网膜血流密度(vessel density，VD)与视野缺损程度的相关性。方法：光学相干断层血管成像技术(optical coherence tomography angiography，OCTA)测量89例(112眼)视野缺损的CPACG患者的黄斑区VD、视盘旁VD，分析VD与视野缺损程度的相关性。结果：视盘旁VD与视野缺损程度成负相关(r>–0.728，P<0.05)。黄斑浅层总VD的受试者工作曲线(receiver operating characteristic，ROC)及曲线下面积(area under the curve，AUC)为0.874。在控制年龄、眼压及视力的情况下，黄斑总浅层VD每降低1%，视野平均缺损(mean deviation，MD)值增加–0.639 dB。结论：CPACG患者VD与视野缺损呈线性负相关，OCTA可以方便无创地观察青光眼患者眼底血流情况，在视野缺损前发现视网膜VD降低，从而可以作为CPACG早期诊断的参考指标。
Objective: To investigate the correlation between the retinal vessel density (VD) and the degree of visual field loss in chronic primary angle-closure glaucoma (CPACG). Methods: Eighty-nine CPACG patients (112 eyes)with different degrees of visual field loss were measured with optical coherence tomography angiography (OCTA) for macular VD and para-optic microcirculation VD, and the correlation between them and the degree of visual field defect were analyzed. Results: There was a negative correlation between the VD of the microcirculation in each zone next to the optic disc and the degree of visual field loss (r>–0.728, P<0.05). The receiver operating characteristic (ROC) and area under the curve (AUC) of the total VD of the superficial macula is 0.874. Under the condition of controlling age, intraocular pressure and vision, for every 1% decrease in the total superficial macular VD, the average visual field defect mean deviation (MD) value increases –0.639 dB. Conclusion: The VD of CPACG patients is linearly negatively correlated with visual field defects. OCTA can conveniently and non-invasively observe the blood flow of the fundus in patients with glaucoma. It is found that the retinal VD is reduced before visual field defects, which can be used as a reference index for early diagnosis of CPACG.
目的：了解原发性开角型青光眼(primary open angle glaucoma，POAG)患者视野缺损的进展情况，探讨其发生进展的相关危险因素。方法：回顾性分析2014年1月至2018年7月就诊于北京大学第三医院眼科并有至少4次视野检查的POAG患者。按照患者首次视野检查的平均偏差或平均缺损进行分期。将历次随访视野检查的平均偏差或平均缺损与时间进行线性回归分析，取其斜率(dB/年)。根据平均偏差或平均缺损的斜率将患者分为进展组与无进展组。分析患者视盘周围视网膜神经纤维层(retinal nerve fiber layer，RNFL)厚度损害位置、平均随诊间隔时间、基线视野分期等因素与青光眼视野缺损进展的关系。结果：共纳入128例患者(252只眼)，其中129眼使用Octopus视野计检查随访，基线视野缺损值为(10.91±5.76) dB；123眼使用Humphrey视野计，基线视野偏差值为(–10.62±6.89) dB。视野缺损早、中、晚期的比例分别为26.19%、36.51%和37.30%。进展组31只眼(12.30%)，无进展组221只眼(87.70%)。上下方RNFL都存在重度损害的患者，其视野缺损更易进展(P<0.001)。平均随诊间隔时间≤4个月的患眼，发生进展的比例高于平均随诊间隔时间>4个月的患眼(P=0.058)。基线视野分期、年龄、性别、总随访时间与视野缺损进展未见显著相关性。结论：青光眼患者的视功能损害出现恶化是普遍存在的。上下方RNFL均存在重度损害、随诊间隔时间短与视野缺损进展相关。视神经结构的改变与功能损害具有相关性，结构改变的方位对功能损害进展有提示功能。规律随诊对病情监测有重要意义，对于可能快速进展的患者，应缩短随诊间隔时间。
Objective: To investigate the progression of visual field defect in primary open angle glaucoma (POAG), and to explore the related risk factors for its progression. Methods: A retrospective analysis was performed on patients with POAG who had at least 4 visual field examinations in the Department of Ophthalmology, Peking University Third Hospital from January 2014 to July 2018. The visual field was staged according to the mean deviation or mean defect of the first visual field examination. Linear regression analyses of mean deviation or mean defect were performed against time, and corresponding regression slopes (in decibels per year) were calculated. Patients were divided into progressive and non-progressive groups according to the mean deviation slope or mean defect slope. The relationship between retinal nerve fiber layer (RNFL) thickness lesion location, mean follow-up interval, baseline visual field staging, and the progression of visual field defect in glaucoma were analyzed. Results: A total of 128 patients (252 eyes) were included. Among them, 129 eyes were followed up with an Octopus perimeter, and the average mean defect value of the baseline visual field was 10.91±5.76 dB; while the other 123 eyes were followed up with a Humphrey perimeter, and the average mean deviation value of the baseline visual field was –10.62±6.89 dB. The proportion of early, middle and late visual field defects was 26.19%, 36.51% and 37.30%. There were 31 eyes (12.30%) in the progressive group and 221 eyes (87.70%) in the non-progressive group. Patients with severe damage to both the upper and lower RNFLs had more visual field defects (P<0.001). Patients with an average follow-up interval ≤4 months had a higher rate of progression than those with an average follow-up interval >4 months (P=0.058).There were no significant differences in baseline visual field stage, age, gender, and total follow-up time between the progression and progression-free groups. Conclusion: Deterioration of visual function impairment is common in glaucoma patients. The progression of visual field defects is associated with severe impairments which are present both in the upper and lower RNFLs, and short follow-up intervals. Optic nerve structure changes are related to functional impairment, and the location of structural changes is suggestive of functional impairment progression.Regular follow-up visits are of great significance for disease monitoring. For patients who may progress rapidly, the follow-up interval should be shortened.
