剥脱性青光眼患者视网膜神经纤维层厚度变化分析

目的：比较维吾尔族假性剥脱综合征(PEX)患者、剥脱性青光眼(PEXG)患者视网膜神经纤维层(RNFL)厚度,为早期诊断剥脱性青光眼提供理论依据。

方法：回顾性病例对照研究。选取2018-04/ 2020-06在我院就诊治疗的维吾尔族假性剥脱综合征患者70例70 眼,剥脱性青光眼患者 80例80 眼,按照视野缺损分期分为早中期剥脱性青光眼患者56眼、晚期剥脱性青光眼患者24眼,选取同期本院收治的维吾尔族年龄相关性白内障病例60例60 眼作为对照组。比较四组患者视盘不同位置RNFL厚度。

结果：对照组、假性剥脱综合征组及剥脱性青光眼组患者视野缺损(MD)逐渐加重且剥脱性青光眼组患者MD显著高于假性剥脱综合征组(P<0.01); 假性剥脱综合征组、早中期剥脱性青光眼组及晚期剥脱性青光眼组视盘不同位置RNFL厚度较对照组均变薄(均P<0.01); 假性剥脱综合征组患者视盘平均RNFL、下方、上方RNFL厚度均低于对照组(均P<0.01)); 晚期剥脱性青光眼组患者视盘各个位置RNFL厚度明显低于早中期剥脱性青光眼患者(均P<0.01)。

结论：维吾尔族假性剥脱综合征患者早期RNFL厚度较未患有剥脱综合征人群开始变薄,早期对于假性剥脱综合征患者RNFL进行检测有助于剥脱性青光眼疾病诊断并进行治疗。     

剥脱综合征性青光眼的发病机制

剥脱综合征是一种常见的年龄相关性眼病,是导致青光眼的常见原因.剥脱综合征性青光眼较慢性单纯性青光眼更严重、预后更差.在此文中对近年来,剥脱综合征性青光眼发病机制的有关论著作一综述.     

剥脱性青光眼发病机制的研究进展

剥脱性青光眼是继发于剥脱综合征的一种青光眼类型。近年来, 国内外学者对剥脱性青光眼的发病机制和危险因素进行了大量研究, 发现了多种危险基因、异常表达的生长因子和细胞因子以及广泛的眼前、后节改变。剥脱性青光眼与全身疾病和环境存在一定关联。本文在对这些成果进行系统总结的基础上, 指出当前亟待进行深入研究的问题, 以期为该领域提供参考。     

剥脱性青光眼的临床和病理

前　　言剥脱综合征 exfoliation syndrome表现为高龄者的晶体前囊和虹膜瞳孔缘部出现灰白色头皮屑样物质 ,多伴发难治性青光眼。剥脱综合征伴发的青光眼称为剥脱性青光眼 exfoliation glaucoma。剥脱性青光眼基本无自觉症状 ,当发现患病时已经出现显著的视野损害 ,已不能恢复视功能。所以对本病的早期发现、早期治疗极为重要。剥脱综合征以前高发于已进入老龄化社会的北欧各国 ,在其他国家比较少见〔1〕。但是 ,最近在九州大学就诊的 5 0岁以上发生开角型青光眼的老龄患者多半归于本病〔2〕,所以对剥脱综合征和剥脱性青光眼的研究十分必要…     

剥脱综合征的遗传学

剥脱综合征(exfoliation syndrome XFS)是一种常见的年龄相关性细胞外基质代谢异常性眼病,是导致青光眼的常见原因.对剥脱综合征患者进行的全基因组关联性研究表明剥脱综合征和剥脱性青光眼(exfoliative glaucoma XFG)与赖氨酸氧化酶样基因1(lysyl oxidase-like 1 gene LOXL1)中的3个单核苷酸多态性(single nucleotide polymorphisms SNPs)密切相关.此文对近年来剥脱综合征遗传学方面的研究进展作一综述.     

剥脱综合征的遗传学

剥脱综合征(exfoliation syndrome XFS)是一种常见的年龄相关性细胞外基质代谢异常性眼病,是导致青光眼的常见原因.对剥脱综合征患者进行的全基因组关联性研究表明剥脱综合征和剥脱性青光眼(exfoliative glaucoma XFG)与赖氨酸氧化酶样基因1(lysyl oxidase-like 1 gene LOXL1)中的3个单核苷酸多态性(single nucleotide polymorphisms SNPs)密切相关.此文对近年来剥脱综合征遗传学方面的研究进展作一综述.     

剥脱综合征

剥脱综合征的特点是临床上见淡灰色或白色碎片位于瞳孔缘和晶体前囊,也出现于虹膜的两面、小带、睫状体、玻璃体、小梁和角膜内面。常合并青光眼。作者注意到单眼剥脱病人中93%需要在双眼进行抗青光眼治疗。这表明在未发生剥脱症的另一眼多有异常高眼压,并由此推测囊性青光眼可能经常与原发性开角青光眼并存。作者复查了1977年74例患单眼或双眼剥脱综合征的病人及其资料。都曾经过散瞳裂隙灯下检查、房角检查、视盘的描绘或照像和视力、视野的测量。剥脱综合征的诊断是通过对晶体、小带等处典型碎片的观察而做出的。眼压数次测量为     

剥脱综合征

一、历史背景多年来关于剥脱综合征(Exfoliation Syndrome)有许多描述和命名,Vogt(1925)首次提出老年性晶体囊膜剥脱和晶体囊膜性青光眼(Senile exfoliation of the lens capsule and glaucoma capsular),以后又有老年性炎性青光眼(Glaucoma senilis),晶体囊膜假性剥脱(Pseudoexfoliation of the lens capsule)等名称。电子显微镜研究证实除晶体囊膜外,眼的其他结构如房角小梁、睫状体、虹膜、结膜等都存在着剥脱物质。晶体摘出后,剥脱物质仍存在于玻璃体前表面,说明晶体囊膜并不是剥脱物质的唯一来源。鉴于眼的多种结构被累及,所以Sunde(1956)称之为剥脱综合征,这个名称是迄今为止最能为大家所接受的。Eagle等(1979)发现睫状体后短动脉被累及,提示剥脱综合征是一种基底膜疾病,因而命名为基底膜剥脱综合征(Basement Membrane Exfoliation Syndrome)。     

超声乳化白内障手术对剥脱综合征性青光眼的降眼压作用

目的 观察和分析剥脱综合征性青光眼患者行超声乳化白内障摘除人工晶体植入术后的眼压变化。方法 对7例伴有高眼压、明显影响视力的白内障但尚无严重的视神经、视野损害的剥脱综合征患者施行超声乳化白内障摘除人工晶体植入术，比较手术前后的眼压变化。结果 术前平均眼压31．57mmHg，术后1周时平均眼压18．19mmHg，降幅为13．38mmHg，术前术后眼压差异极显著。结论 伴有明显影响视力白内障的剥脱综合征性青光眼病人在行超声乳化白内障摘除人工晶体植入术后眼压会显著下降，对那些尚未发生明显视神经视野损害的剥脱综合征性青光眼患者，行超声乳化白内障摘除人工晶体植入术可有效降低眼压。     

闭角型青光眼与剥脱综合征

多数学者认为剥脱综合征与青光眼的关系是：青光眼是继发的结果。但我科发现2例是闭角型青光眼小梁切除术后才发现有剥脱综合征的表现。现报告如下。     

Combined exfoliation and pigment dispersion syndrome

Both exfoliation syndrome and pigmentary dispersion syndrome can lead to secondary glaucoma. We treated five patients who had pigmentary dispersion and who subsequently developed exfoliation. In one patient who had bilateral pigment dispersion syndrome, the presence of exfoliation increased the difficulty of controlling intraocular pressure in the affected eye. In a monocular patient, exfoliation and pigmentary dispersion were concurrently present with characteristic defects of both pigmentary dispersion and exfoliation as seen by transillumination defects of the iris. A third patient had bilateral pigmentary dispersion and uncontrollable intraocular pressure in an eye with exfoliation. Despite argon laser trabeculoplasty, the pressure remained uncontrolled. Another patient had a filtering operation in the left eye, with gradually increasing intraocular pressure in the right eye. Exfoliation material complicated the pressure control in the unoperated on eye. Another patient had an eight-year history of glaucoma in the left eye for which filtering surgery was performed. Exfoliation was present in both eyes with controlled pressure in the filtered eye but uncontrolled pressure in the nonfiltered eye.     

Exfoliation syndrome

Exfoliation syndrome (XFS) is an age-related disorder of the extracellular matrix characterized by production and progressive accumulation of small, white deposits of a fibrillar extracellular material in many ocular tissues, most commonly seen on the pupillary border and anterior lens capsule. Characteristic signs of pigment liberation and deposition throughout the anterior segment aid in the diagnosis. Exfoliation syndrome predisposes to both open-angle and angle-closure glaucoma, and to capsular rupture, zonular dehiscence, and vitreous loss during cataract extraction. Exfoliation syndrome is associated with ocular and perhaps systemic ischemia. The exact chemical composition of exfoliation material remains unknown. Medical, laser, and surgical therapy are similar to treatment of primary open-angle glaucoma.     

Exfoliation syndrome in enucleated haemorrhagic and absolute glaucoma

Occurrence of exfoliation syndrome was studied in 172 eyes enucleated for haemorrhagic and absolute glaucoma in 1962-82, using histopathological techniques. Exfoliation was found in 33% of haemorrhagic glaucoma cases and in 22% of absolute glaucoma. In patients over 70, exfoliation was found in 53% of the haemorrhagic cases and in 46% of the absolute glaucoma cases. Capsular glaucoma may provoke haemorrhagic glaucoma and may lead to blindness and enucleation of eye with or without vascular catastrophe.     

The impact of exfoliation syndrome on therapeutic efficacy in open-angle glaucoma

Exfoliation syndrome is the most common identifiable cause leading to chronic open-angle glaucoma. It affects the treatment of glaucoma in various ways. The prognosis of capsular glaucoma (exfoliative glaucoma) is worse than that of open-angle glaucoma. Medical therapy and the long-term effect of laser treatment seem to be unsatisfactory in capsular glaucoma. Surgical intervention may be needed early, although eyes with exfoliation syndrome are prone to surgical complications. Finally, absolute glaucoma and blindness are more common in capsular glaucoma than in primary open-angle glaucoma. This article deals with the impact of exfoliation syndrome on therapeutic efficacy in open-angle glaucoma. It reviews the literature between October 1993 and September 1994; only a few studies concerning this important subject have been published during the given period.     

Increased occurrence of exfoliation in the male, Spanish American population of New Mexico.

Exfoliation syndrome was found to occur much more frequently in the male Spanish American population of New Mexico. This was based on a Veterans Administration Medical Center study in Albuquerque, New Mexico. The prevalence of exfoliation in the Spanish Americans was estimated to be from 3-6 percent and this is consistent with many other such studies performed in the United States and other countries. The Spanish American population is 5.8 times more likely to develop exfoliation than the non-Spanish American population of New Mexico. Exfoliation was found on such eye structures as the lens, iris, ciliary processes, posterior capsule, vitreous face, posterior corneal surface, and arterial chamber angle. Glaucoma capsulare was also studied and it was found that there was not a significant difference between the Spanish American and the non-Spanish American population for developing the condition. However, there was a strong association of glaucoma with the presence of exfoliation in both groups. Based on our results, patients with exfoliation are 22 times more likely to develop glaucoma than are their age-matched controls. Also, 40 percent of the exfoliation patients had glaucoma compared with only 5 percent of the age-matched controls (p less than .001). The patients with glaucoma capsulare were found to have a more medically resistant type of glaucoma, which was consistent with other reports of this condition. There are two theories we propose for the existence of exfoliation in the Spanish American population of New Mexico. The first is a geographic climatic theory that relates exfoliation to greater solar radiation levels due to a high overall altitude and sunny weather in New Mexico.(ABSTRACT TRUNCATED AT 250 WORDS)     

Exfoliation syndrome and exfoliation glaucoma

Exfoliation syndrome abnormal deposition in the anterior segment of the eye of an unknown substance thought to be related to elastic fibres and basement membrane components is associated with accelerated cataract progression. increased frequency of intraoperative and postoperative complications and increased risk for glaucoma and. therefore, is a clinically important finding. A clear association has been shown with age. The syndrome occurs worldwide but its prevalence seems to vary from country to country. The best-known sign of exfoliation syndrome is deposits of greyish-white material on the anterior lens surface. Sometimes exfoliation material can also be seen at the pupillary border, on the anterior iris surface, corneal endothelium, and on the anterior vitreous face. When clinically detected, exfoliation syndrome is somewhat more often unilateral than bilateral. According to recent investigations clinically unilateral exfoliation syndrome is probably never truly unilateral but rather asymmetric, because exfoliation material has been detected ultrastructurally and immunohistochemically around iris blood vessels of the nonexfoliative fellow eyes. Indeed, electron microscopy identifies in various organs of patients with exfoliation syndrome fibrils similar to those seen in intraocular exfoliation deposits. Other clinical signs associated with exfoliation syndrome are pigment dispersion, transillumination defects of the iris and reduced response to mydriatics. In unilateral exfoliation syndrome, intraocular pressure (IOP) of the exfoliative eye is approximately 2 mmHg higher than IOP of the nonexfoliative fellow eye. Whether elevated IOP, vascular changes or exfoliation syndrome itself is the main factor causing optic nerve head damage and conversion of an exfoliative eye to glaucomatous, is not known. Glaucoma in the exfoliation syndrome has been shown to have a more serious clinical course than in primary open-angle glaucoma (POAG). At the time of diagnosis, IOP and its diurnal variation are generally higher and visual field defects tend to be greater in exfoliation glaucoma than in POAG. Because the decrease in lOP variation and lowering of the mean IOP level has been shown to improve visual field prognosis more in exfoliation glaucoma than in POAG, the glaucomatous process is considered to be more pressure-related in exfoliation glaucoma. Furthermore, progression of optic disc damage has been shown to be similar in exfoliation glaucoma and POAG when lOPs are lowered to a comparable level by the treatment. However, vascular disturbances in the posterior segment of the eye might after all be of equal importance in these two types of glaucoma; optic disc haemorrhages and venous occlusions have been reported to be as frequent in exfoliation glaucoma as in POAG. Perhaps in exfoliation glaucoma circullatory disturbances combined with high IOP lead to a particularly relentlessly progressing form of the disease.     

Review: The role of LOXL1 in exfoliation syndrome/glaucoma

Exfoliation syndrome is a common cause of open-angle glaucoma. It is characterized by microscopic flakes of protein-rich material being deposited in both ocular and non-ocular tissues. While its mechanism is poorly understood, family- and population-based studies have established that the disorder has a strong genetic component. A further understanding of the relevant gene variants might help reveal the molecular mechanism behind exfoliation. The most-strongly associated genetic variants are found in the lysyl oxidase-like 1 (LOXL1) gene. However, two major risk alleles in the LOXL1 coding region are reversed between ethnic groups. It now appears the strong association between LOXL1 and XFS is due to non-coding variants that have not yet been identified. Such variants might alter LOXL1 expression, which is decreased in the late stages of exfoliation syndrome/glaucoma. Here we discuss LOXL1 as a risk gene for exfoliation syndrome and glaucoma.     

Exfoliation syndrome in patients scheduled for cataract surgery.

A series of 305 consecutive patients 30 years of age or older scheduled for cataract surgery was examined to find out the frequency of exfoliation syndrome, the predominant type of cataract, and the intraocular pressure status. The mean age of 222 (72.8%) females and 83 (27.2%) males was 71.7 (+/- 11.1) years (range 32 to 91 years). Exfoliation was detected in 77 (25.2%) patients. It was unilateral in 37 (48.0%) patients, the affected eye being in 29 (78.4%) cases scheduled for cataract surgery, and bilateral in 40 (52.0%) patients. Exfoliation was increased steadily with age. It was most often seen on the anterior lens capsule (88.9%). Krukenberg's spindle was seen in 31 (20.4%) eyes of patients with and in 12 (2.6%) eyes of patients without exfoliation (p less than 0.001). Nuclear sclerosis predominated in eyes with (83.8%) as compared to those without (61.9%) exfoliation (p less than 0.01). Posterior subcapsular cataract was less common in eyes with exfoliation (1.5%) than in eyes (21.1%) without it (p less than 0.001). A tendency to slightly higher preoperative intraocular pressure was measured in eyes with exfoliation as compared to those without it, and glaucoma occurred much more frequently (33.8% vs 10.8%) in the former group (p less than 0.001). Exfoliation and capsular glaucoma, which must be promptly detected to avoid surgical complications, are frequent in patients scheduled for cataract surgery.     

The prevalence and type of glaucoma in geriatric patients.

A group of 100 institutionalized geriatric patients aged 69-94 years (mean 81.2 years) was studied at Koskela Helsinki Municipal Hospital. The selection of the patients was randomized by taking 100 patients having a birth-date divisible by five. Glaucoma occurred in 15% of the patients (14 women and one man). Six patients had bilateral primary open-angle glaucoma. One patient had capsular glaucoma in one eye and secondary glaucoma in the other eye. Eight patients had glaucoma only in one eye; three narrow-angle glaucoma, three primary open-angle glaucoma and two secondary glaucoma. Exfoliation occurred in 21 patients (26%, 21/80). Ten patients had bilateral exfoliation and 11 exfoliation only in one eye. IOP was measured in 75 patients, 150 eyes, with applanation tonometry, averaging 12.5 mmHg (SD 5.0), and in 22 patients, 44 eyes, with Schi?tz tonometry, averaging 16.3 mmHg (SD 5.6). Visual acuity for long distance and also the reading acuity were greater than 0.3 in 66% (54/82).     

The HNK-1 epitope in the inner connective tissue layer of the human ciliary body in exfoliation syndrome and various types of glaucoma

Possible changes in the expression of the HNK-1 carbohydrate epitope in the inner connective tissue layer of the human ciliary body, located between the ciliary epithelium and muscle, was studied using 2 formalin-fixed, paraffin-embedded eyes with exfoliation syndrome, 33 eyes with different types of glaucoma, and 21 morphologically normal control eyes. A strong immunoreaction delineating cell processes was observed in this layer with monoclonal antibodies HNK-1 and VC1.1 recognizing the HNK-1 epitope in control specimens, whereas partly granular immunoreaction was present in eyes with exfoliation syndrome. Exfoliation material was also immunoreactive. In all types of advanced glaucoma, the immunoreaction was mostly granular in nature and greatly diminished. No difference in HNK-1 immunoreactivity between control and glaucoma eyes was seen in the retina and ciliary epithelium. Elevated intraocular pressure, either directly or by decreasing blood flow to the ciliary body, may cause degenerative or metabolic changes in the inner connective tissue layer cells that bear or secrete molecules sharing the HNK-1 epitope. The partly granular immunoreactivity in eyes with exfoliation syndrome only indicates changes in this epitope even without an increase in intraocular pressure.