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.

Macular hemorrhage (MH) is one of the most severe complications of high myopia, posing a significant threat to vision. MH can occur with or without choroidal neovascularization (CNV), with the CNV-associated form being the most prevalent. CNV-related MH may develop secondary to conditions such as pathological myopia, and punctate inner choroidopathy. Conversely, MH without CNV is often linked to factors like lacquer cracks, trauma, ocular surgery. While the exact mechanisms of CNV in high myopia are still not fully understood, anti-VEGF injections have been shown to be effective in improving visual function in patients with CNV. This review summarizes the clinical characteristics of various causes of MH and their respective treatments, providing valuable insights to help clinicians make informed diagnostic and therapeutic decisions.

Macular hemorrhage (MH) is one of the most severe complications of high myopia, posing a significant threat to vision. MH can occur with or without choroidal neovascularization (CNV), with the CNV-associated form being the most prevalent. CNV-related MH may develop secondary to conditions such as pathological myopia, and punctate inner choroidopathy. Conversely, MH without CNV is often linked to factors like lacquer cracks, trauma, ocular surgery. While the exact mechanisms of CNV in high myopia are still not fully understood, anti-VEGF injections have been shown to be effective in improving visual function in patients with CNV. This review summarizes the clinical characteristics of various causes of MH and their respective treatments, providing valuable insights to help clinicians make informed diagnostic and therapeutic decisions.

目的:研究增殖性糖尿病视网膜病变患者玻璃体基质细胞衍生因子(Strmalcell-derivedfactor-1, SDF-1)和血管内皮生长因子(Vascular endothelial growth factor, VECF)的浓度,及其相互作用关系。

方法:酶联免疫吸附法(Enzyme-linked immunosorbent assay, ELISA)检测玻璃体内 SDF-1 和 VEGF 的含量,每个标本重复3次。实验组为增性糖尿病视网膜病变(Proliferalive diabeticretinopathy, PDR)的住院患者30例,对照组为同期行玻璃体切除术的特发性黄斑裂孔患者12例。

结果: PDR 患者玻璃体 VECF 的平均浓度为(2865.87+387.85) pg/ml,明显高于特发性黄斑裂孔组[(142.42+21.03) pg/ml，P < 0.0001]。增殖性糖尿病视网膜病变患者玻璃体 SDF-1的含量平均为(298.40+24.57) pg/ml,对照组为(86.91+15.89) pg/ml,两组的差异具有统计学意义(P < 0.0001)。在30例PDR患者玻璃体内 VEGF 和 SDF-1 的含量表现为正相关(Peanson相关系数 r=0.62，P < 0.001)。

结论:增殖性糖尿病患者玻璃体 SDF-1 和 VECF 的含量均高于非糖尿病患者,提示 SDF-1 和 VEGF 共同参与了增殖性糖尿病视网膜病变患者病理性新生血管的形成过程。

Purpose: To investigate the levels of stromal cell-derived factor-1(SDF-1) andvascular endothelial growth factor (VEGF) in the vitreous of patients with proliferativediabetic retinopathy.
Methods: The levels of $DF-1 and VEGF in the vitreous of 30 eyes of 30 patients withproliferative diabetic retinopathy(PDR)and 12 eyes of 12 patients with idiopathicmacular hole (MH) were measured by enzyme-linked immunosorbent assay. Vitreousfluid samples were obtained by vitrectomy.
Resuls: The vitreous concentration of VEGF was signifcantly higher in eyes with PDR(2 865.87+387.85 pg/ml) than in eyes with idiopathic macular hole (142.42+21.03 Pgml, P< 0.000 1). The vitreous level of SDF-1 was also significantly higher in eyes withPDR (298.40+24.57 pg/ml ) than in eyes with idiopathic macular hole (86.91+15.89Pg/ml, P<0.000 1 ). The vitreous concentration of SDF-1 correlated significantly with that of VEGF in eyes with PDR( [correlation coefficient]r=0.62,P<0 .001)
Conclution: Vitreous levels of both SDF-1 and VEGF in patients with PDR aresignificantly higher than those of nondiabetic patients. SDF-1 may be correlated withVEGF in angiogenesis in PDR.

玻璃体淀粉样变性是一种罕见的眼病, 可独立发病, 也可以表现为系统性淀粉样变性的眼部受累, 常有家族史。报道 1 例遗传性玻璃体淀粉样变性患者, 中年发病, 双眼先后受累,有明确的家族史。双眼均行玻璃体切除术。术后病理检查结果显示: 玻璃体呈刚果红染色阳性, 电镜下发现纤维丝状物。对患者外周血进行 DNA 抽提, PCR 扩增, 克隆、筛选及测序等一系列基因检测, 发现转甲蛋白(Transthyretin, TTR) 存在着基因突变, 突变点 Gly83Arg, 这可能是玻璃体淀粉样变性发病的一个新的突变位点。

Vitreous amyloidosis is a rare condition that mainly occurs in Familial Amyloidotic Polyneuropathy (FAP). In some cases, it may be the only symptom without systemic disorders. One case of familial vitreous amyloidosis was reported here, with white, wispy opacities in vitreous cavity in both eyes. Pars plana vitrectomy and histopathological examination of the vitreous specimens were performed. The vitreous specimens showed typical microscopic features of amyloidosis with Congo red stain and non-branching fibrils on a transmission electron microscope. Transthyretin (TTR) gene was amplified with DNA isolated from the peripheral blood cells. Bi-directional sequencing of exon 3 showed a single base-pair substitution, which results in an amino acid substitution at position83, glycine to arginine (TTR Arg-83) . TTR Arg-83 may be a new pathologic mutation in vitreous amyloidosis. 

息肉状脉络膜血管病变(polypoidal choroidal vasculopathy，PCV)是亚洲人中常见的眼底致盲性疾病，当PCV合并视网膜下出血或玻璃体积血(vitreous hemorrhage，VH)时，患者视力骤然下降，视力预后差异大。但目前聚焦于PCV合并VH的相关文献较少，因此研究和阐明PCV继发VH的治疗方法及预后具有重要的临床意义。目前临床上常选择手术干预，玻璃体切除术(pars plana vitrectomy，PPV)是临床上最常选择的一种术式。其他治疗方式包括玻璃体内注射抗血管内皮生长因子(vascular endothelial growth factor，VEGF)、眼内气体或硅油填充、眼内注射组织纤溶酶原激活剂(tissue plasminogen activator，tPA)和光动力疗法(photodynamic therapy，PDT)。PCV合并VH患者的视力预后决定因素是黄斑视功能的保留程度，也与年龄、术前视力、PCV病变部位、视网膜下出血量、视网膜脱离范围、基线黄斑中心厚度(central macular thickness，CMT)、是否出现术后并发症以及是否形成视网膜瘢痕等因素相关，目前也有研究发现视力预后与单核苷酸多态性(single nucleotide polymorphisms，SNP)相关。本文就PCV继发VH的临床特点、治疗及预后进行综述。

Polypoid choroidal vasculopathy (PCV) is a common fundus blinding disease in Asians. When PCV is associated with subretinal hemorrhage or vitreous hemorrhage (VH), patient's visual acuity decreases suddenly and the visual prognosis varies greatly. There are few relevant literatures focusing on VH secondary to PCV, so it is of great clinical significance to study and clarify the treatment methods and prognosis of VH secondary to PCV. At present, surgical intervention is often selected in clinical practice. Vitrectomy is the most commonly selected surgical procedure in clinical practice. The other treatment modalities include intravitreal injection of antivascular endothelial growth factor (VEGF), intraocular gas or silicone oil filling, intraocular injection of tissue plasminogen activator (tPA) and photodynamic therapy. The prognostic determinant of visual acuity in PCV

patients with VH is the degree of preservation of macular visual function. The prognostic is also related to age, preoperative visual acuity, PCV lesion location, amount of subretinal hemorrhage, extent of retinal detachment, baseline central macular thickness (CMT), postoperative complications and retinal scars. Recent studies also find that the prognosis of visual acuity is related to single nucleotide polymorphisms. This article reviews the clinical characteristics, treatment and visual prognosis of PCV associated with VH.

目的：分析高度近视有晶状体眼后房型人工晶状体植入术后孔源性视网膜脱离的临床特征及预后。方法：回顾分析2012年4月至2021年6月中山眼科中心收治的9例(9只眼)行后房型人工晶状体植入术后孔源性视网膜脱离患者的临床特征、手术方式及疗效，随访(4.96±4.78)个月。结果：患者年龄(30.44±20.11)岁，屈光手术至发病时间(32.10±17.80)个月。4例(44.4%)马蹄形裂孔，1例(11.1%)萎缩性裂孔，4例(44.4%)巨大裂孔；9眼裂孔均位于赤道部前，除2眼(22.2%)为单个巨大裂孔，1眼(11.1%)单个马蹄孔，余6眼(66.7%)均有视网膜周边变性区存在；视网膜脱离范围(3.0±1.12)个象限，8例累及黄斑；增殖性玻璃体视网膜病变C级以上4眼。视网膜初始复位率为77.8%，最终视网膜复位率100%。末次随访最佳矫正视力优于术前(P<0.05)。随访期间，2例硅油填充眼发生并发性白内障，4眼发生术后早期高眼压。结论：有晶状体眼后房型人工晶状体植入术前存在的视网膜变性或术后玻璃体牵引的存在可能是孔源性视网膜脱离发生的危险因素。

Objective: To analyze the clinical presentation, surgical management, and outcomes of rhegmatogenous retinal detachment (RRD) in patients with high-myopia corrected by posterior chamber phakic (PCP) intraocular lens (IOL) implantation. Methods: Nine eyes of 9 patients in whom RRD developed after PCPIOL implantation from April 2012 to June 2021 in Zhongshan Ophthalmic Center were retrospectively studied. Mean follow-up after retinal detachment surgery was (4.96±4.78)months. Results: Mean patient age was (30.44±20.11) years old. RRD occurred (32.10±17.80) months after PCPIOL implantation. Four (44.4%) breaks were horseshoe tear, 1 (11.1%) was atrophic hole and 4 participants (44.4%) had a giant retinal tear. Nine cases had causative breaks located anterior to the equator while peripheral retina lattice degeneration was found in 6 eyes. RRD extended from 1 to 4 quadrants (3.0±1.12 quadrants) and 8 cases were macula-off retinal detachments. Four eyes’ proliferative vitreoretinopathy were more severe than level C. Initial reattachment rate was 77.80%. Final retinal reattachment was 100%. Final follow-up BCVA was significantly better than baseline (P<0.05). Furthermore, concurrent cataract occurred in 2 eyes in which silicone oil was used as tamponade. Ocular hypertension was detected in 4 eyes after surgery. Conclusion: The existed lattice degeneration and postoperative vitreous traction may be risk factors for RRD after PCPIOL implantation.