中国和印度原发性先天性青光眼患者临床特点的对比研究

目的:总结中国原发性先天性青光眼患者的临床特点,与印度原发性先天性青光眼患者进行比较,研究二者临床特点的差异。方法:对中国和印度的原发性先天性青光眼患者进行回顾性分析。复习40例中国先天性青光眼患者的病历记录,将临床特点总结为几个可定量临床参数,评价疾病的严重程度。将中国患者的量化临床特点以及病情严重度分级和文献中的印度患者比较,进行统计分析。结果:中国患者40例和印度患者43例纳入了研究。中国患者的性别比例约为2∶1,3例(7.5%)患者有家族史,1例(2.5%)患者的亲代为近亲婚配。中国患者的主要症状和体征较多,初诊时最常见的症状是眼球长大(42.5%)、视力下降(35.0%)。与印度患者比较,中国先天性青光眼患者发病较晚,诊断较迟,角膜增大和视神经损害更严重(P<0.01)。两国的眼科医生都倾向于采用小梁切开联合小梁切除术。印度患者接受联合手术的比例更高(P<0.01)。两国患者的病情严重程度分布不同,大部分中国患者的病情严重,而大部分印度患者的病情非常严重(P<0.01)。结论:中国原发性先天性青光眼患者为散发病例,非近亲婚配型。与印度原发性先天性青光眼患者比较,中国患者的发病较晚,诊断治疗相对滞后。中国先天性青光眼的防治工作尚待加强。     

华南地区与西南地区先天性青光眼临床特点的比较

目的通过对中山眼科中心与华西医院眼科中心收治的原发性先天性青光眼患者进行对比,探讨我国华南、西南地区先天性青光眼发病的异同性。方法以华西医院眼科中心（WCHOC）报道的原发性先天性青光眼的研究结果为参照,与中山眼科中心（ZOC）在同一时段（2002年1月至2004年12月）收治的原发性先天性青光眼患者进行回顾性对比分析。结果ZOC入组的原发性先天性青光眼患者有51例（102眼）,WCHOC患者40例（80眼）。两组患者均以男性为多,分别为3.25：1和2.07：1;双眼发病为主：家族史均不明显;发现年龄在两地患者中无显著性差异（P〉0.01）：但WCHOC患者就诊年龄远远大于ZOC患者（分别为36个月与9个月）;就诊时已有视功能严重损害者以WCHOC患者为多：手术治疗均以小梁切开联合小梁切除术为主。结论与中山眼科中心原发性先天性青光眼患者比较,华西眼科中心患者就诊较晚,视功能损害严重,诊断、治疗相对滞后。在一定程度上可能与地区经济水平对该病诊治的影响有关。中国先天性青光眼的防治工作仍存在很大的不平衡性,值得关注。     

A case of spontaneously resolved primary congenital glaucoma

Primary congenital glaucoma usually presents as enlarged and hazy cornea at birth or early childhood. The diagnosis is based on a thorough clinical examination under anesthesia. Most cases require surgical intervention as the definitive treatment. In very rare instances, primary congenital glaucoma may arrest and resolve spontaneously. We describe a case of spontaneously arrested and resolved primary congenital glaucoma in a 37-year-old male presenting with large cornea, Haab''s striae, and normal intraocular pressure in one eye. Such a case has not been previously described from the Indian subcontinent.     

原发性先天性青光眼临床治疗研究

目的：观察并分析外路小梁切开联合小梁切除术治疗原发性先天性青光眼的临床疗效。 方法：收集原发性先天性青光眼患者51例89眼,应用外路小梁切开联合小梁切除术进行治疗,观察术后眼压、角膜横径、杯/盘比值、滤过泡情况及手术并发症并分析其临床疗效。 结果：术后平均随访（15.21±6.50）mo,术后1,6,12mo手术成功率分别为97%,90%,90%。术后平均眼压较术前显著降低(P<0.01);杯/盘比值较术前明显减小（P<001）;角膜横径手术前后差异无显著意义（P=0.495）;手术失败者角膜横径较术前增大（P<0.05）。手术并发症主要有不同程度的前房出血和术后浅前房等。 结论：外路小梁切开联合小梁切除术是治疗原发性先天性青光眼安全有效的手术方式之一     

永存原始玻璃体增生症与先天性纤维血管瞳孔膜临床特征的比较

目的：分析永存原始玻璃体增生症(PHPV)和先天性纤维血管瞳孔膜(CFPM)的临床特征异同。

方法：回顾性分析2006-03/2021-12在空军军医大学西京医院眼科接受手术治疗的PHPV(PHPV组)和CFPM患儿(CFPM组)的眼部生物测量参数、临床表现、病变的形态学特点。

结果：纳入PHPV患儿56例61眼,CFPM患儿24例25眼; PHPV和CFPM的发病年龄相似、无性别差异,均以单眼患病为主,其占比分别为91%和96%。PHPV合并白内障患眼可有多种并发症和眼发育异常,CFPM主要为不同程度的瞳孔区堵塞及形态异常。PHPV组和CFPM组单眼患病患儿患眼前房深度(ACD)均小于对侧眼,手术年龄≤24月龄患儿患眼眼轴长度(AL)均小于对侧眼(P<0.05); PHPV组单眼患病患儿患眼角膜直径(CD)小于对侧眼、眼压高于对侧眼(均P<0.05); CFPM组单眼患病患儿患眼与对侧眼CD、IOP比较均无显著差异(P>0.05)。PHPV组患儿患眼ACD小于CFPM组患眼(P<0.05)。术中发现PHPV纤维血管膜组织位于晶状体后、玻璃体腔内,而CFPM纤维血管膜位于虹膜与晶状体前囊膜之间,很少累及晶状体。

结论：PHPV和CFPM有非常相似的临床特点,提示PHPV和CFPM可能是永存胚胎血管(PFV)的不同表现形式,但PHPV病变范围更广、病情更复杂。     

Prognosis of primary ab externo surgery for primary congenital glaucoma

BACKGROUND: The strategy of pressure reducing surgery in primary congenital glaucoma has changed over the last decade. Ab externo filtering procedures--for example, trabeculectomy or trabeculotomy combined with trabeculectomy, have now been accepted even as primary intervention. METHODS: The authors reviewed 61 eyes in 35 consecutive patients with primary congenital glaucoma, who underwent different types of initial ab externo surgery between 1988 and 1996 (median follow up 36 months) to determine the efficacy of different surgical techniques and the influence of various risk factors. RESULTS: Trabeculotomy was performed in 17 eyes (27.9%), trabeculotomy with trabeculectomy in 15 eyes (24.6%), and trabeculectomy in 29 eyes (47.5%). Regarding age, preoperative intraocular pressure, corneal diameter, ocular axial length, and incidence of corneal haze the subgroups were comparable. Success rates of trabeculotomy, trabeculectomy, and a combined procedure did not significantly differ when assessed by life table analysis. Patient age under 3 months (p = 0.014) and an ocular axial length of 24 mm or more (p = 0.016) proved to be major risk factors for primary ab externo surgery failure. A second operation was necessary in 20 of 61 eyes (32.8%) during follow up. CONCLUSION: Prognosis of primary ab externo glaucoma surgery in primary congenital glaucoma seems to be governed more by the individual course and severity of the disease than by modification of surgical techniques.     

Long-term efficacy of latanoprost in primary congenital glaucoma

Purpose

To investigate the success (glaucoma control) of latanoprost therapy of primary congenital glaucoma (PCG) and factors affecting the long-term outcome.

Methods

Patients with PCG treated with latanoprost were re-examined. At study visit and from clinical charts, we evaluated: intraocular pressure, length of glaucoma control with latanoprost, need of further medication or glaucoma surgery, systemic and topical side effects. Multivariate analysis was used to test factors related to the final outcome of the treatment.

Results

Eighty-one eyes of 44 patients with PCG, and 42 eyes of 29 patients with previous glaucoma surgery, had received latanoprost therapy. In the first group, a success (glaucoma control by latanoprost therapy) was found in 24 eyes (29.6%), whereas 57 eyes (70.4%) had received surgery (45 eyes (55.6%) in the first year); among the eyes with previous surgery, a success was found in 12 eyes (28.6%), 13 eyes (31%) required an additional therapy, and 17 eyes (40.5%) had received further glaucoma surgery. No patient discontinued the treatment because of side effects. Factors related to the failure of the latanoprost treatment were: the high score of severity of glaucoma (P=0.014) and low age at PCG presentation (P=0.042).

Conclusions

Long-term treatment with latanoprost is effective in about 30% of the eyes; factors related to failure were severe glaucomatous alterations, and young age at PCG presentation.     

Ahmed青光眼引流阀植入治疗先天性青光眼术后眼压失控疗效分析

目的：探讨Ahmed青光眼引流阀植入治疗先天性青光眼术后眼压失控的疗效与安全性。

方法：回顾分析了2011-01/2014-12因先天性青光眼术后眼压失控患者22例22眼,行青光眼引流阀植入术。主要检查指标包括手术前后眼压、角膜直径以及并发症。

结果：术前平均年龄3.74±2.24岁,距上次手术平均2.59±1.78a,术前平均眼压35.22±6.36mmHg,平均水平角膜直径12.79±0.75mm。所有眼术中使用丝裂霉素C 0.3～0.5mg/mL 3～5min,青光眼引流阀植入颞上或鼻上方巩膜赤道部。术后1wk眼压11.4±4.45mmHg,术后12mo随访眼压16.73±7.23mmHg。以眼压<21mmHg为成功标准,术后12mo 16眼(73%)眼压控制。术后6例发生浅前房,均自行恢复。所有患者未发生引流阀排斥及眼内炎、角膜失代偿等严重并发症。

结论：Ahmed青光眼引流阀植入治疗先天性青光眼术后眼压失控,是一种安全、有效的方法。     

影响原发性先天性青光眼手术后视力的原因分析

目的：探讨影响原发性先天性青光眼术后视力的原因及预防措施。方法：追踪检查５１只接受手术治疗后１０年以上的原发性先天性青光眼的眼压、视力、角膜及杯盘比值等的变化情况。结果：手术不成功的１２只眼视力全部在０．０５以下。手术成功的３９只眼视力在０．１至０．８以上,７只单眼发病者视力都在０．２以下;角膜直径大于１３ｍｍ的２７只眼中２１中眼有Ｈａａｂ线存在;杯盘比值大于０．２以上者１２只眼。结论：手术不成功     

原发性先天性青光眼临床特征的相关性

目的 分析原发性先天性青光眼的临床特征及其相关关系.方法 收集2006～2007年于北京同仁医院诊治三岁内原发性先天性青光眼患者临床资料,应用SPSS软件进行相关分析和回归分析.结果 共收集144例(259只眼),79.9%为双眼患病,男性明显多于女性(男女比=2.51:1),一岁内发病者占71.53%.有先天性青光眼家族史者占2.78%.最常见的症状为角膜混浊(41.0%)和畏光流泪(38.9%),多元回归分析发现发病时间与此相关(β=-0.191,P=0.027;β=-0.235,P=0.008).多元线性回归分析发现眼压与角膜直径(β=-0.244,P=0.038)、视乳头C/D比(β=0.293,P=0.005)之间显著相关.Logistic回归分析显示角膜直径(OR=0.403,P=0.000)与Haab纹显著相关.Pearson相关分析发现角膜直径与发病时间和病程无关.结论 角膜混浊与畏光流泪同是原发性先天性青光眼最常见的症状.检查角膜是否清亮,患儿是否怕光可作为先天性青光眼初步筛查的检查项目.眼压、角膜直径、Haab纹及视乳头C/D比均可以作为判断病情严重程度的指标.角膜直径仅与眼压相关,与发病时间和病程无关,是很好的判断疾病是否进展的指标.     

Clinical effect of improved viscocanalostomy for the treatment of primary congenital glaucoma

AIM: To evaluate the clinical effect of improved viscocanalostomy in patients with primary congenital glaucoma. METHODS: Retrospective analysis of improved viscocanalostomy was performed on 51 eyes of 42 patients with primary congenital glaucoma. The outcome evaluation included postoperative intraocular pressure(IOP), corneal diameter, cup/disc ratio and complications. All patients were followed up at week 1, month 1, 3, 6 and 12. RESULTS: The results revealed that postoperative IOP was decreased from (38.57±13.61)mmHg to (10.53±3.91)mmHg, (14.89±5.26)mmHg, (15.42±5.11)mmHg, (13.82±3.46)mmHg, (13.16±5.29)mmHg at follow-up time of 1 week, 1, 3, 6, 12 months (P<0.001). The postoperative corneal diameter was decreased significantly (P=0.002); The mean cup/dish ratio wasn’t significantly different (P=0.148) before and after the surgery, the cup/dish ratio of successful surgery was evidently decreased (preoperative 0.7±0.2, postoperative 0.6±0.3, P=0.007), but the complications like as unformed anterior chamber were not observed. The mean follow-up period was 12 months. CONCLUSION: Improved viscocanalostomy improves the clinical effects of the patients with primary congenital glaucoma, such as higher success rates, lower postoperative mean IOP and fewer complications.     

微导管辅助的小梁切开术治疗原发性先天性青光眼的远期疗效观察

目标评估微导管辅助小梁切开术治疗原发性先天性青光眼的远期效果和安全性。设计回顾性病例系列。研究对象2014年2月至2016年12月北京同仁眼科中心微导管辅助小梁切开术治疗的原发性先天性青光眼患者65例(86眼),手术时年龄2个月~11岁,平均(3.7±2.8)岁。方法术后3、6、9个月以及1、2、3、4、5、6年进行随访。比较术前、术后末次随访时(或手术失败时)眼压及降眼压药物使用数量的变化。术后不使用降眼压药物,且眼压≤21 mmHg为绝对成功;术后需使用降眼压药物控制眼压≤21 mmHg为条件成功。主要指标眼压、抗青光眼药物使用数量、术中及术后并发症,远期手术干预。结果平均随访(46.3±9.9)个月(36~72个月)。35眼为初次手术,51眼具有抗青光眼手术史。术前平均眼压(32.5±6.4)mmHg,平均应用(2.8±0.6)种药物;末次随访平均眼压(15.6±7.9)mmHg,平均用药(0.4±1.0)种,二者较术前均显著下降(P均<0.001)。术后1、3、5年累积绝对成功率分别为90.7%、86.0%、68.8%,累积条件成功率分别为90.7%、88.3%、76.9%。随访至术后5年,累积绝对成功率既往无手术史者(94.3%)比有手术史者(60.2%)高(P=0.04)。无严重手术并发症发生。结论微导管辅助的小梁切开术治疗原发性先天性青光眼长期安全有效。抗青光眼手术史会影响远期手术成功率。     

原发性先天性青光眼分子遗传学研究进展

原发性先天性青光眼（primarycongenital aucoma,PCG）是一种发生于婴幼儿的遗传性致盲性眼病,也是先天性青光眼中最常见的类型,遗传因素为该病的主要致病原因,目前报道的与PCG相关的候选基因为CYPIBl、LTBP2和MYOC,并对这些基因的遗传方式、突变筛查及突变后的基因结构、功能改变等方面做了大量研究。然而,上述基因突变并不能解释所有PCG患者的发病机制,因此,发现新的致病基因和突变位点将成为研究工作者关注的焦点。f国际眼科纵览,2012,36：293—297）     

微导管引导的内路小梁切开术治疗原发性先天性青光眼六个月的疗效

目的 评估微导管引导的内路小梁切开术治疗原发性先天性青光眼的效果。设计 回顾性病例系列。研究对象 2018年8月至2019年2月北京同仁眼科中心原发性先天性青光眼患者27例（30眼）。方法 患者接受微导管引导的内路小梁切开术，根据术中全周切开和次全周切开，以及是否有既往手术史分组。术后随访6个月。主要指标 眼压、降眼压药物及术后并发症。结果 术前平均眼压（31.5±6.1）mmHg，用药中位数3种（1~4种）；术后末次随访眼压（15.7±3.6）mmHg，用药中位数0种（0~3种）（P均<0.001）。全周切开组（17眼）及次全周切开组（13眼）的术前眼压及术后末次眼压均无统计学差异（P均＞0.05），但全周切开组的降眼压幅度为（54.7%±10.1%），明显大于次全周切开组（37.5%±16.8%，P=0.002）。初次手术组（16眼）与多次手术组（14眼）术前眼压、术后末次眼压及降眼压幅度的差异均无统计学意义（P均＞0.05）。术后早期均有前房积血, 6眼（20%）术后一过性眼压升高。结论 短期随访结果显示， 微导管引导的内路小梁切开术治疗角膜透明的原发性先天性青光眼是可行的。     

外路微导管辅助的小梁切开术治疗中晚期原发性先天性青光眼

目的:评估外路微导管辅助的小梁切开术治疗中晚期原发性先天性青光眼的疗效。方法:回顾性研究。纳入2018-08/2019-11在西安市第四医院收治的行外路微导管辅助的小梁切开术治疗中晚期原发性先天性青光眼患儿20例22眼。随访12mo,观察手术前后眼压、使用降眼压药物数量、角膜直径、视神经杯盘比、并发症。结果:平均眼压术前为33.4±9.1mmHg,末次随访时为14.4±5.2mmHg(P<0.001),术前使用降眼压药物为2(2,4)种,术后12mo为0(0,2)种(P<0.001)。角膜直径术前为13.34±0.89mm,术后12mo为13.27±0.78mm(P>0.05)。术前视神经杯盘比为0.85±0.17,术后12mo为0.84±0.16(P>0.05)。所有患者均未发生严重手术并发症。结论:外路微导管辅助的小梁切开术治疗中晚期原发性先天性青光眼安全有效。     

Launching a paradigm for first and redo-surgery in primary congenital glaucoma: institutional experience

AIM: To evaluate the outcome of the initial and the redo-surgeries for primary congenital glaucoma (PCG) correlated to its degree of severity. METHODS: A retrospective study involved patients with PCG presented between 2010 and 2018. Medical records were reviewed to assess the degree of the preoperative severity according to the intraocular pressure (IOP), corneal diameter and corneal edema. Success and failure rates were calculated for both first and redo-surgeries at 6 and 12mo respectively then correlated to the severity of the cases. RESULTS: Complete records were retrieved for 272 eyes (153 patients) with PCG: 43 eyes were mild, 136 moderate and 93 severe. Combined trabeculotomy and trabeculectomy (CTT) had the highest success rate in moderate (96.4%) and severe cases (59.3%) while trabeculotomy had the highest success rate in mild cases (96.3%). Medical records of 88 eyes (63 patients) with recurrent PCG were analyzed, most with severe presentation (59 eyes). Ahmed glaucoma valve (AGV) was used in 67 (76%) eyes and augmented trabeculectomy in 21 (24%) eyes. At 12mo, there was no statistically significant difference between both surgeries in total success rate (P=0.256). For mild cases, success rate was 100% for both surgeries. Severe cases had higher success rates following AGV (87%) than augmented trabeculectomy (20%). Preoperative severity of the disease was an independent factor affecting the failure rate in secondary trabeculectomy but not in AGV. Patients younger than 24mo had higher probabilities of failure following both redo-surgeries with hazard ratio =1.325 and 0.37 for augmented trabeculectomy and AGV respectively. CONCLUSION: Preoperative assessment of the severity of eyes with PCG helps in the selecting the optimal primary and secondary surgery. For first surgery, trabeculotomy is more effective in mild cases whereas; CTT and augmented subscleral trabeculectomy (SST) are appropriate for moderate and severe cases. AGV proved to be superior to augmented SST in severe recurrent cases.     

Identification of differentially expressed proteins in the aqueous humor of primary congenital glaucoma

Primary Congenital Glaucoma (PCG) is an autosomal recessive disease caused by an abnormal development of the anterior chamber angle. Although, PCG has been linked to several genetic loci, the role that the genes at these loci or their encoded proteins play in the pathophysiology of PCG and development of the anterior chamber is not known. To identify proteins that may be altered in PCG and that may help in understanding the underlying pathophysiology of the disease, we took a global proteomics approach. Tryptic digests of the complex mixtures of proteins in aqueous humor were analyzed using Liquid Chromatography/Mass Spectrometry (LC-MS/MS). Proteins were identified by searching the data against the human subset of the UniProt database. The proteomes of aqueous humor in PCG (n = 7) and patients undergoing cataract surgery as control (n = 4) were compared based on the scan counts of comparable proteins. Using stringent filtering criteria, Apolipoprotein A-IV (APOA-IV), Albumin and Antithrombin 3 (ANT3) were detected at significantly higher levels in PCG AH compared to control, whereas Transthyretin (TTR), Prostaglandin-H2 D-isomerase (PTGDS), Opticin (OPT) and Interphotoreceptor Retinoid Binding Protein (IRBP) were detected at significantly lower levels. Many of these proteins play a role in retinoic acid (RA) binding/transport and have been implicated in the pathogenesis of neurodegenerative diseases such as Alzheimer’s (AD). It is possible that similar to AD, the pathologic changes in PCG during development could be influenced by the availability of RA in the anterior chamber.