眼附属器淋巴组织增生性疾病作为一类疾病的总称，包括了良性淋巴组织增生、非典型性淋巴组织增生、IgG4相关眼病以及多种恶性淋巴瘤在内的数十种疾病类型。临床诊断此类疾病应将患者眼部体征、影像学检查与病理学检查紧密结合。随着免疫表型及分子病理等检测技术的进步，此类疾病之间的鉴别诊断正逐渐清晰。本文就眼附属器淋巴组织增生性疾病进行系统性描述，并重点探讨该类疾病的病理鉴别诊断。

Ocular adnexal lymphoproliferative disease, as a general term, contains reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, IgG4 related ocular disease and malignant lymphoma. The clinical diagnosis of this kind of disease should integrate patient’s symptoms, imaging features and pathology characteristics. Development of immunophenotyping, molecular pathology and other detection technology will help with the differential diagnosis of ocular adnexal lymphoproliferative disease. This article is going to discuss the etiology, epidemiology,diagnosis and treatment of ocular adnexal lymphoproliferative disease, with a focus on the clinicopathological differential diagnosis of such disease.

线状皮脂腺痣综合征(linear nevus sebaceous syndrome，LNSS)是一种以皮脂腺痣(nevus sebaceous，NS)为特征性改变，同时合并癫痫、智力迟钝、神经缺陷或骨骼畸形等病变的疾病。本文报道1例经病理组织学检查确诊的LNSS患者，同时伴有双眼脉络膜骨瘤和脑部先天发育异常。由于线状皮脂腺综合征伴双眼多发异常较为少见，本文将总结该例患者的临床和病理表现，旨在为临床诊疗提供一定参考资料。

Linear nevus sebaceous syndrome (LNSS) is a disease characterized by nevus sebaceous (NS) and accompanied by epilepsy, mental retardation, nerve defect or skeletal deformity. We report a case of linear sebaceous nevus syndrome diagnosed by histopathological examination with bilateral choroidal osteoma and congenital developmental abnormalities of the brain. Since linear sebaceous gland syndrome with binocular abnormalities is relatively rare, this paper will summarize the clinical and pathological manifestations of this patient, aiming to provide certain reference for clinical diagnosis and treatment.

目的：制备抗水通道蛋白4(aquaporin 4，AQP4)单克隆抗体，鉴定其免疫学性能为后期临床应用奠定基础。方法：合成AQP4优势抗原表位多肽，经过免疫小鼠、细胞融合及亚克隆筛选，制备可分泌高效价高亲和力的单克隆抗体细胞株，后期获取大量单克隆抗体通过酶联免疫吸附和免疫组织化学实验进行初步实验应用。结果：获得4株杂交瘤细胞株，均能稳定分泌高亲和力抗体，经酶联免疫吸附测定实验和免疫组织化学实验证实均能特异性识别人的AQP4蛋白。结论：成功制备了亲和力高、特异性强的抗人AQP4优势抗原表位的单克隆抗体，为小分子抗体以及抗体人源化制备奠定了基础，进而为治疗视神经脊髓炎相关疾病提供理论依据和技术支持。

Objective: To lay the foundation for clinical applications in the future, we prepare and identify the immunological properties of aquaporin 4 (AQP4) monoclonal antibodies. Methods: The dominant epitope polypeptides of AQP4 were synthesized and used to obtain the cell lines which secrete high tiler and high affinity monoclonal antibodies by immunizing BALB/c mouse cell fusion and screening of subclone. The large amount of monoclonal antibodies were obtained and used for practice via immunohistochemical staining and Enzyme-linked immunosorbent assay (ELISA). Results: Four hybridomas that can stably secret anti-AQP4 antibodies were obtained. ELISA, Western blot and Immunohistochemistry results suggested that the monoclonal antibodies specifically recognized human AQP4 protein.Conclusion: The anti-human AQP4 monoclonal antibody with high affinity and specificity was successfully generated, which thereby provides the foundation for the preparation of small molecule antibodies or humanized antibodies, and then lays a theoretical and technical basis for the treatment of neuromyelitis optica (NMO).

目的：分析各种病因摘除眼球的患儿中视网膜母细胞瘤(retinoblastoma，RB)的误诊率，并总结其临床和病理特点。方法：回顾性分析2003年以来在中山眼科中心因临床诊断为RB并接受眼球摘除的患者563例(577眼)，比较术前临床诊断和术后病理诊断，从中筛选出误诊病例，分析这些病例的临床和病理特点，包括患者年龄、性别、治疗过程、临床影像学、肿瘤病理分期、病理诊断以及分化程度。结果：共发现误诊病例22例(22眼)，误诊率3.91%，所有误诊病例均为单眼摘除患者。常见的临床误诊类型为：与Coats病相混淆12例(12眼)，与眼内炎症反应混淆5例(5眼)，以及与眼内其他肿瘤混淆5例(5眼)。所有患者中，>4岁者55例，其中误诊12例，误诊率21.82%。结论：与RB相关的临床误诊并不罕见，其中最常见的误诊病因是Coats病，>4岁患者是误诊的主要人群。

Objective: To investigate the misdiagnosis rate in enucleated eyes for retinoblastoma (RB), analyze the clinicopathological features and summarize differential diagnosis. Methods: Retrospective analysis was performed on 563 cases (577 eye) undergoing eyeball enucleation in Zhongshan Ophthalmic Center Sun Yat-sen University since 2003. Misdiagnosed cases were screened out by comparing the preoperative clinical diagnosis and postoperative pathological diagnosis. The clinical and pathological features of those misdiagnosed caseswere summarized, including age, gender, therapeutic process, imagological examination, pathological stage and pathological diagnosis. Results: Twenty-two cases had been misdiagnosed, which account for 3.91% of 563 enucleated eyeball cases. All of misdiagnosed cases had underwent monocular eyeball enucleation. Diseases that were easily misdiagnosed with RB were Coats disease (12/22), endophthalmitis (5/22) and other intraocular tumor (5/22). Among all patients, 55 patients were older than 4 years old, of which 12 cases were misdiagnosed,with a misdiagnosis rate of 21.82%. Conclusion: It is not unusual for clinical misdiagnosis of RB. Coats disease is the most frequent cause of RB misdiagnosis. Misdiagnosis rates is higher in patients over 4 years old.

目的：比较活体共聚焦显微镜和病理检查在角膜后部真菌感染的诊断阳性率，探讨两种检查方法在角膜后部真菌感染诊断中的价值。方法：回顾性病例对照研究。收集2009年11月至2020年12月在青岛眼科医院就诊并进行穿透性角膜移植手术治疗角膜后部真菌感染患者，术前均进行角膜刮片KOH涂片检查和活体共聚焦显微镜检查，术后病变角膜进行病理组织切片、过碘酸-Schiff法(PAS)染色和六亚甲基四胺银法(GMS)染色检查，比较不同检查方法诊断的阳性率。结果：18例角膜后部真菌感染患者角膜刮片KOH涂片均未检查到真菌菌丝，其中有16例患者经活体共聚焦显微镜检查到真菌菌丝(88.9%)，而2例患者在术前活体共聚焦显微镜检查中未查到病原体。术后病理检查PAS染色联合GMS染色，18例患者中18例均可检查到真菌菌丝，角膜后部真菌感染患者病理切片中可见角膜深基质层变性坏死，大量炎症细胞浸润，PAS染色和GMS染色可见典型真菌菌丝侵犯角膜基质深层，而角膜基质浅层及上皮层均未查见真菌菌丝。结论：活体共聚焦显微镜诊断角膜后部真菌感染具有一定的局限性，联合术后病理组织切片和特殊染色检查有助于提高角膜后部真菌感染的诊断率。

Objective: To compare the diagnostic rate between in vivo confocal microscopy and pathological examination in retrocorneal fungal infection. Methods: It is a retrospective study. A total of 18 patients with retrocorneal fungal infection and received PKP surgery in the Qingdao Eye Hospital from November 2009 to December 2020 were enrolled. KOH smear and in vivo confocal microscopy examination were performed before surgery, and pathological examination including periodic acid-schiff (PAS) stain and Grocott Methenamine Silver (GMS) stain were performed after surgery. Patients were diagnosed retrocorneal fungal infection based on in vivo confocal microscopy and pathological examination. The diagnostic rates of the two methods were compared. Results: None of the 18 patients with posterior corneal fungal infection were found to have fungal hyphae in the corneal smear.Sixteen patients (88.9%) were found fungal hyphae by in vivo confocal microscopy. Corneal stroma necrosis and a large number of inflammatory cells were shown by postoperative pathologic examination, and all patients were found fungal hyphae in posterior corneal stroma with PAS stain and GMS stain. Conclusion: Confocal microscopy has unique advantages such as non-invasive and rapid examination in the diagnosis of fungal keratitis.However, it needs to combine with pathological examination for diagnosing the retrocorneal fungal infection.

目的：研究泪腺腺样囊性癌不同病理组织学分型和分级间骨质破坏、神经侵犯和预后的差异。方法：回顾性分析2010年4月至2019年4月首都医科大学附属北京同仁医院眼肿瘤科收治并经病理组织学检测确诊的30例泪腺腺样囊性癌患者的完整病历资料。分析患者的一般病历资料、医学影像学资料、病理组织学检查结果以及预后情况等，并系统研究泪腺腺样囊性癌病理组织学分型和分级与患者预后的关系。结果：30例患者中，病理组织学分型为筛状型9例(30.0%)，实体型10例(33.3%)，混合型11例(36.7%)，其中实性和腺管混合型4例(13.3%)、实性和筛状混合型4例(13.3%)、筛状和腺管混合型3例(10.0%)。病理组织学分级为I级12例(40.0%)；II级4例(13.3%)；III级14例(46.7%)。不同病理组织学分型肿瘤骨质破坏发生率差异有统计学意义(P=0.046)；不同病理组织学分级肿瘤骨质破坏发生率差异无统计学意义(P=0.513)。不同病理组织学分型与分级肿瘤神经侵犯、远处转移发生均无相关性(均P>0.05)。在行手术联合放射治疗后，不同病理组织学分型肿瘤的无复发率差异有统计学意义(P<0.05)；实体型的无复发率在36个月内最低(P=0.037)；而病理学分级与手术联合放射治疗后的无复发率无关(P=0.059)。结论：泪腺腺样囊性癌病理组织学分型与骨质破坏发生率和复发率显著相关，其中实体型肿瘤较易复发。而病理学分型与神经侵犯和远处转移发生率无关。病理组织学分级与骨质破坏、神经侵犯、远处转移和复发率无显著相关性。

Objective: To investigate the differences in bone destruction, nerve invasion, and prognosis of lacrimal gland adenoid cystic carcinoma (LGACC) among different histologic types and grades. Methods: A retrospective analysis was performed on 30 cases of lacrimal gland adenoid cystic carcinoma confirmed by histopathology who were admitted to the Department of Ophthalmic Oncology, Beijing Tongren Hospital, Capital Medical University from April 2010 to April 2019. The general data, imaging findings, histological examination and prognosis were collected and analyzed, with the focus on the relationship between the histological characteristics and the prognosis of lacrimal gland adenoid cystic carcinoma. Results: Among the 30 patients, 9 cases of cribriform type (30.0%); 10 cases of solid type (33.3%); 11 cases of mixed type (36.7%), including 4 cases of solid and glandular (13.3%), 4 cases of solid and cribriform (13.3%), 3 cases of cribriform and glandular (10.0%). The histopathological grade was I in 12 cases (40.0%), II in 4 cases (13.3%) and III in 14 cases (46.7%). There was statistical difference in the incidence of bone destruction among different histological types (P=0.046). There was no significant difference in the incidence of bone destruction among different histological grades (P=0.513).There was no significant difference between different histological grades and types and the incidence of nerve invasion and distant metastasis (all P>0.05). After surgery combined with radiotherapy, there was a statistical difference in the recurrence-free rate of different histological types, and the recurrence-free rate of solid type was the lowest within 36 months (P=0.037). Histological grade was not associated with recurrence-free rate after surgery combined with radiotherapy (P=0.059). Conclusion: The histological type of adenoid cystic carcinoma of lacrimal gland was significantly correlated with the incidence of bone destruction and recurrence rate, in which solid type was more likely to relapse. And the histological type was not associated with the incidence of nerve invasion or distant metastasis. Histopathological grade was not significantly associated with the rate of bone destruction, nerve invasion, distant metastasis, and recurrence.

目的：探讨眼眶原发性滑膜肉瘤(synovial sarcoma，SS)的临床病理学及分子遗传学特点。方法：收集1例复旦大学附属眼耳鼻喉科医院眼科2020年10月收治并经病理学检查证实为眼眶原发性SS的病例，同时回顾性分析文献中已报道的10例眼眶原发性SS的临床及病理检查资料，包括临床表现、影像学检查、组织学特点、免疫表型及分子病理学检查结果。结果：患者女，53岁，因“复发性右眼眶内肿物13余年”收治入院。SS组织病理学：肿瘤由弥漫分布的单一短梭形细胞组成，肿瘤细胞异型性明显，胞质少，核分裂多见；肿瘤侵犯结膜下、巩膜表面、视神经鞘膜、眶内肌肉及纤维脂肪组织。免疫组织化学检查提示波形蛋白(Vimentin)、Calpolnin、CD99、Bcl-2均阳，SMARCB1(INI-1)部分阳/弱阳。荧光原位杂交(fluorescence in situ hybridization，FISH)法检测到SS18基因易位。回顾性总结文献中已报道的10例和本例(总共11例)眼眶SS患者，其中男性2例，女性9例，左眼6例，右眼5例；患者发病年龄为1~53岁，平均年龄22岁，中位年龄24岁。患者术前病程范围较广，为1周~13年。11例中，5例症状至少出现3年以上，多表现为进行性眼球突出伴眼球移位及运动受限，疼痛及视力下降。CT和MRI上多表现为分叶状或者卵圆形软组织肿块，部分因出血坏死出现囊性外观，增强扫描显示病灶呈不均匀强化。组织学上，本组11例眼眶SS中单相纤维型7例，双相型4例，单相纤维型中有2例存在分化差的成分。免疫组织化学染色显示：上皮样成分表达上皮标记(CKpan、CK7、CK19)和Vimentin；梭形细胞表达Vimentin、CD99、Bcl-2、Calpolnin、TLE1及灶性表达上皮标记。结论：眼眶原发性滑膜肉瘤罕见，形态上需要和眼眶其他软组织来源恶性肿瘤相鉴别，其具有特征性t(x:18)(p11;q11)染色体易位，产生SY T-SSX融合基因，分子病理学的检测有助于最后确诊。

Objective: To investigate the clinicopathological and molecular genetics features of synovial sarcoma (SS) of the orbit. Methods: We retrospectively reviewed 10 published cases of primary SS of the orbit, along with 1 case of primary SS of the orbit confirmed by pathology who was admitted to the ophthalmology department of Eye & ENT Hospital of Fudan University in October 2020. The clinical data, radiological findings,morphology, immunophenotype and genetic characteristics of the cases were analyzed. Results: Our case was a 53-year-old woman with an SS in the right orbit, which had recurred multiple times. Histopathologic examination showed a primitive tumor composed of spindled and ovoid cells. Focal infiltration was observed in adjacent structures, such as the sub-conjunctiva, scleral surface, optic nerve sheath, muscle, and fibro-fatty tissue. Immunohistochemistry showed positivity for vimentin, calponin, CD99, and Bcl-2 and loss of INI-1expression, which is typical of SS. Fluorescence in situ hybridization (FISH) showed the (X;18)translocation in the tumor cells. The analysis included 2 males and 9 females aged between 1 and 53 years old (mean: 22 years; median: 24 years). Among the SS cases, 6 left eyes and 5 right eyes (all monocular cases)were affected. Symptoms had been present from 1 week to 13 years in the case from our hospital, while in 5 cases, symptoms had been present for at least 3 years. Common clinical features of the patients included proptosis or globe displacement, decreased vision, and pain. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an ovoid mass with heterogenous enhancement and a cystic appearance,which was probably attributable to hemorrhage or necrosis. Of these 11 cases, 7 cases were biphasic SS,4 were monophasic fibrous SS, and 2 were poorly differentiated in monophasic SS. Immunohistochemistry revealed positivity for pan-cytokeratin (CKpan), CK7, CK19, vimentin, cluster of differentiation 99 (CD99),B-cell lymphoma 2 (Bcl-2), calponin and transducin-like enhancer protein 1 (TLE1). Conclusion: Primary SS of the orbit is extremely rare and needs to be distinguished from other spindle cell tumors of orbital soft tissue. The SS diagnosis is based on the presence of the t(X;18) (p11; q11) translocation, which results in an SYT-SSX fusion gene.

CRISPR(clustered regularly interspaced short palindromic repeats)基因编辑技术通过精准改变细胞DNA序列，控制细胞命运及表型，是有望从根本上改变疾病治疗的新技术。由于眼球独特的生理构造，基因编辑疗法在治疗眼科疾病方面的应用具有明显的优势。目前，CRISPR基因编辑疗法治疗10型Leber先天性黑矇(Leber congenital amaurosis 10，LCA10)的临床试验已经展开，治疗其他多种眼科疾病的临床试验也即将开始。随着新一代CRISPR基因编辑技术的发展，基因编辑疗法有望为眼科疾病的治疗提供新的手段。

Clustered regularly interspaced short palindromic repeats (CRISPR) genome editing is a newly developed technology to precisely modify cellular DNA sequence, which could control cell fate and phenotype and fundamentally reform disease treatment. The structure of the eye offers unique advantages as a genome editing target. Recently, a CRISPR genome editing therapy has begun to be tested in Leber congenital amaurosis 10 (LCA10) patients, and the clinical trials for more ocular diseases are about to start. The development of CRISPR/Cas genome editing tools will drive major advances in the application of gene therapies in the treatment of ophthalmic disease.

目的：分析湖南地区汉族人群中2型糖尿病患者的人口学特征及生化指标，寻找糖尿病视网膜病变的高危因素。方法：釆用病例对照研究，统计湖南地区正常人群、2型糖尿病但无视网膜病变患者、2型糖尿病视网膜病变患者的人口学特征及生化指标的相关数据，进行成组t检验及logistic回归分析，探讨分析糖尿病视网膜病发生的易感因素。所有研究对象均为汉族。结果：对照组[非糖尿病(non-diabetes mellitus，NDM)组]和2型糖尿病未合并视网膜病变[(non-diabetic retinopathy，NDR)]组之间性别分布、年龄分布、BMI、舒张压、HbA1c、总胆固醇、高密度脂蛋白(high-density lipoprotein，HDL)、尿酸及总胆红素差异无统计学意义(均P>0.05)。NDM组中腹围、收缩压、空腹血糖、三酰甘油、肌酐和低密度脂蛋白(low-density lipoprotein，LDL)值均低于NDR组，差异有统计学意义(均P<0.05)。NDM组中BMI、腹围、收缩压、舒张压、空腹血糖、HbA1c、总胆固醇、三酰甘油、肌酐和LDL值均低于2型糖尿病合并视网膜病变组(diabetic retinopathy，DR)组，差异有统计学意义(均P<0.05)。NDR组收缩压、舒张压、HbA1c、总胆固醇、三酰甘油和肌酐值均低于DR组，差异有统计学意义(均P<0.05)。结论：收缩压超过150 mmHg，舒张压超过90mmHg，糖化血红蛋白超过9%，血清肌酐超过100 μmol/L，三酰甘油超过3 mmol/L均为糖尿病患者发生视网膜病变的高危易感因素。

Objective: To analyze the demographic characteristics and biochemical indexes of type 2 diabetic patients in Han population in Hunan, and to find the high-risk factors of diabetic retinopathy. Methods: The data of demographic characteristics and biochemical indexes of normal population, type 2 diabetic patients but without retinopathy and type 2 diabetic retinopathy in Hunan were analyzed. Group t test and logistic regression analysis were used to analyze the susceptibility factors of diabetic retinopathy. All the subjects were Han population. Results: There were no significant differences in gender distribution, age distribution, BMI, diastolic blood pressure, HbA1c,total cholesterol, high-density lipoprotein, uric acid and total bilirubin between the control group [non-diabetes mellitus (NDM) group] and the type 2 diabetic without retinopathy group [non-diabetic retinopathy (NDR)group] (all P>0.05). The abdominal circumference, systolic blood pressure, fasting blood glucose, triglyceride,creatinine and low-density lipoprotein in NDM group were all lower than those in NDR group, and the differences were statistically significant (all P<0.05). BMI, abdominal circumference, systolic blood pressure, diastolic blood pressure, fasting blood glucose, HbA1c, total cholesterol, triglyceride, creatinine and LDL in NDM group were all lower than those in type 2 diabetic retinopathy (DR) group, and the differences were statistically significant (all P<0.05). The comparison between the NDR group and the DR group showed that the values of systolic blood pressure,diastolic blood pressure, HbA1c, total cholesterol, triglyceride and creatinine in the NDR group were all lower than those in the DR group, and the differences were statistically significant (all P<0.05). Conclusion: SBP ≥150 mmHg,DBP ≥90 mmHg, HbA1c ≥9%, serum creatinine ≥100 μmol/L, triglyceride ≥3 mmol/L are the high-risk factors of diabetic retinopathy.

青光眼是全世界范围内不容忽视的致盲性眼病，其起病隐匿，视功能损害进展迅速，晚期预后不理想。长期发展且未行治疗的青光眼患者视神经呈进行性损害，引起视力急剧下降、视野不可逆性缺损，严重降低患者的生活质量。为了适应逐渐恶化的视功能，患者注视行为发生明显变化，由此在日常活动中引起步态行为随之改变，意外事故频繁发生。故目前对于青光眼的研究引起国内外广泛重视，虚拟现实技术(virtual reality，VR)作为青光眼早期诊断及康复治疗的新手段已被现代医学所尝试。本文具体阐述了青光眼的视觉损害与注视行为及运动行为间的联系，并总结了目前国内外关于VR诊断青光眼及作为康复治疗的相关研究。

Glaucoma is a blinding eye disease that cannot be ignored worldwide. Its onset is insidious, visual impairment is progressing rapidly, and the late prognosis is not ideal. Long-term untreated glaucoma patients show progressive damage to the optic nerve, causing a sharp decline in vision, irreversible visual field defects, and severely reducing the quality of life of the patients. In order to adapt to the gradual deterioration of visual function, the patient’s gaze behavior changes significantly, which causes the gait behavior to change in daily activities, and accidents occur frequently. Therefore, the current research on glaucoma has attracted wide attention in the nation and abroad,and virtual reality (VR) technology has been tried in modern medicine as a new method for early diagnosis and rehabilitation of glaucoma. This article specifically elaborates the relationship between the visual impairment of glaucoma and the gaze behavior and movement behavior, and summarizes the current domestic and foreign research on the diagnosis of glaucoma and the rehabilitation of VR technology.