色素播散综合征的临床和病理机制

色素播散综合征和色素性青光眼是以虹膜色素上皮受损,色素颗粒播散并沉积到眼前段为特征的一种较少见疾病。容易与其它眼前段色素播散的疾病混淆,其治疗与原发性青光眼不尽相同。本文就近20年来它的临床表现、病理机制、鉴别诊断和治疗方面的研究进行系统回顾和总结。     

色素播散综合征的临床和病理机制

色素播散综合征和色素性青光眼是以虹膜色素上皮受损，色素颗粒播散并沉积到眼前段为特征的一种较少见疾病。容易与其它眼前段色素播散的疾病混淆，其治疗与原发性青光眼不尽相同。本就近20年来它的临床表现、病理机制、鉴别诊断和治疗方面的研究进行系统回顾和总结。     

色素性青光眼合并睫状体脱离1例

色素播散综合征（pigment dispersion syndrome,PDS）[1]是以虹膜色素上皮受损,色素颗粒播散并沉着于眼前段为特征的一种疾病。当PDS伴有眼压升高才称为色素性青光眼（pigment glaucoma,PG）[2]。PG合并睫状体脱离的临床报告较为罕见,现我院报告1例。     

色素性青光眼误诊分析

色素播散综合征(pigment dispersion syndromePDS)是以虹膜色素上皮受损,色素颗粒播散并沉着于眼前段为特征的一种常染色体显性遗传性疾病[1].PDS伴眼压升高为色素性青光眼(pigmentaryglaucoma PG).该病东方人罕见,易于高眼压症、开角型青光眼等疾病相混淆.     

色素性青光眼误诊分析

色素播散综合征(pigment dispersion syndromePDS)是以虹膜色素上皮受损,色素颗粒播散并沉着于眼前段为特征的一种常染色体显性遗传性疾病[1].PDS伴眼压升高为色素性青光眼(pigmentaryglaucoma PG).该病东方人罕见,易于高眼压症、开角型青光眼等疾病相混淆.     

色素播散综合征和色素性青光眼

色素播散综合征和色素性青光眼是一类在不同人种中具有不同临床表现的眼病,患病率在不同人种间差别亦较大。本文对色素播散综合征和色素性青光眼的发病机制、临床表现、临床病理研究、鉴别诊断、治疗及预后等作一综述。（眼科,2013,22：10-13）     

高度近视白内障术后继发色素性青光眼二例

色素性青光眼(pigmentary glaucoma,PG)是指虹膜色素上皮损伤脱失,色素颗粒沉着眼前段各部分而引起的继发性青光眼,多见于白色人种,有色人种少见,而继发于手术后者更鲜见报道[1].本文总结了2例(2眼)继发于高度近视白内障术后PG的临床特征及治疗体会如下.     

色素性青光眼发病机理的研究进展

色素性青光眼的确切发病机理尚不清楚。研究表明,色素播散综合症和色素性青光眼的虹膜色素播散与虹膜向后弯曲增大、虹膜止端在睫状体上的位置较正常人明显靠后、逆向性瞳孔阻滞、虹膜血管低灌流等有关。房水流出管道的形态测量表明,色素性青光眼房水流出受阻与房水流出管道和盲管表面积减少有关。小梁网的小梁细胞密度减少和坏死与小梁细胞大量吞噬和消化色素有关。本文从色素播散和房水流出受阻的机理等方面综述了色素性青光眼发病机理的研究进展。     

虹膜血管造影在青光眼研究中的应用

虹膜血管造影是将眼底血管造影技术应用于虹膜的一种检查方法,对阐释虹膜病变具有重要价值.本文综述了虹膜血管造影在青光眼研究中的应用,包括原发性开角型青光眼、原发性闭角型青光眼、假性剥脱综合征、新生血管性青光眼、眼前段色素播散综合征等.     

色素性青光眼的临床特征及处理

目的 观察色素性青光眼的临床表现及特征,探讨其治疗方法及预后.方法 色素性青光眼患者8例16只眼,均为男性.年龄19～48岁(34.13±7.12)岁.观察眼压、裂隙灯显微镜、房角、超声生物显微镜(UBM)、眼底及视野等;9只眼行小梁切除术,7只眼行Nd:YAG激光周边虹膜切除.随访时间为12～36个月.结果 所有病例都为近视,1例为高度近视;眼前段色素播散表现为:75%(6例12只眼)的患眼有典型的Krukenberg梭.所有患眼晶状体韧带后囊附止部及晶状体韧带小束均可见色素沉着;周边虹膜后凹;小梁网上均可见宽、均匀而较致密的色素带(Ⅲ级);超声生物显微镜检查示所有患者均存在反向瞳孔阻滞现象,6例存在虹膜一睫状突接触.术后3月复查UBM显示所有患者周边虹膜形态平坦,反向瞳孔阻滞现象解除.所有患者眼压均控制平稳.为10.5～19mmHg(14.3±5.2)mmHg.Humphrey视野检查显示无进展情况.结论 色素性青光眼为一表现相对复杂的特殊类型的青光眼,需要正确认识并适时给予恰当的治疗.     

Light and electron microscopy of the anterior iris surface in eyes with and without pseudo-exfoliation syndrome

Summary The anterior iris surface of 12 human eyes were studied by light and electron microscopy. Seven of these specimens were from eyes with capsular glaucoma, whereas the remaining 5 served as controls. In light microscopic sections large extracellular aggregates appeared within the anterior iris surface of the glaucomatous eyes, and in some cases such deposits formed a 25 broad zone along the anterior iris border. In the electron microscope these extracellular aggregates turned out to be pseudo-exfoliation material. It seems that the amounts of pseudo-exfoliation deposits within the anterior iris surface increase parallel with the progress of glaucoma.     

The middle-Norway eye-screening study. II. Prevalence of simple and capsular glaucoma

In this population-based screening study, dealing with 1941 persons above 64 years of age from three different municipalities, the overall open-angle glaucoma prevalence was found to be 8.3%. The prevalence in the separate areas (7.0%, 8.6%, and 9.5%) were not statistically different. Roughly 30% of the population with pseudo-exfoliation syndrome had glaucoma, and 4.2% had ocular hypertension, whereas the corresponding figures for those without pseudo-exfoliation were 4% and 0.8%, respectively. The high glaucoma rates are partly due to the high pseudo-exfoliation prevalence in the area. The prevalence of the capsular glaucoma increased towards a maximum between 75 and 79 years of age, whereafter the curve declined. This may indicate reduced survival time of glaucomatous patients.     

Gonioscopic view of the trabeculo-Descemet's membrane after deep sclerectomy

PURPOSE: To study the appearance of the trabeculo-Descemet's membrane (TDM) after deep sclerectomy with collagen implant (DSCI). METHODS: All patients who underwent a successful DSCI for open-angle glaucoma were included in a non-randomized clinical study. Gonioscopy was performed between 6 and 12 months postoperatively in all patients. RESULTS: Gonioscopy was performed in 42 eyes of 38 patients after a mean follow-up time of 8.3 +/- 2.5 months after DSCI. The diagnosis was chronic open-angle glaucoma in 16 eyes and exfoliative glaucoma in 24 eyes. One patient had pigmentary glaucoma and one had normal tension glaucoma. None of the patients were on glaucoma medication postoperatively. Synechias to the TDM were seen in five eyes. Pigment accumulation to the TDM was seen in nine eyes, four of which had capsular glaucoma, three had primary open-angle glaucoma, one had pigmentary glaucoma and one had normal tension glaucoma. Puncture holes at the TDM were seen in all eight eyes in which Nd:YAG laser goniopuncture had been performed. CONCLUSION: Postoperative changes, which may go unnoticed without routine gonioscopy, occur at the TDM after deep sclerectomy with collagen implant. Pigment accumulates relatively shortly after surgery. Whether or not these changes are progressive remains unknown. Careful follow-up after deep sclerectomy is necessary and should include routine gonioscopy.     

Long-term prognosis of visual field in glaucoma simplex and glaucoma capsular

The fate of visual fields in 42 eyes with capsular glaucoma followed for 5 to 14 years (mean 9.1 years) and 46 eyes with simple glaucoma followed for 5 to 14 years (mean 10.2 years) was studied retrospectively. In the treatment the IOP levels were around 19.5 mmHg. In both groups the visual field deteriorated in 70-80% of eyes, and 11% of patients became blind. In most cases the progression appeared during the first 4-6 years of follow-up, but in individual cases it did not occur until 8-10 years after the diagnosis. In 26 eyes disc cupping preceded the visual field loss. Five out of 42 eyes with capsular glaucoma developed retinal vein occlusion whereas this occurred in 2 out of 46 eyes with simple glaucoma. Two patients with IOP level of 9 mmHg maintained unchanged visual fields for 6 and 11 years, respectively.     

Results 1 year after trabeculoretraction by 360 degree argon laser in the treatment of open-angle glaucoma

On 210 glaucomatous patients (119 men, 91 women), 390 eyes with open angle chronic glaucoma, we have performed an argon laser trabeculoplasty according to Wise technique (360 degrees in one time). ALT was presented as an alternative to surgery. Therefore results were appreciated so that ALT allowed or not to avoid surgery. After one year, the rate of failures was: on all the 390 eyes: 15 p. cent on O.A.C.G. simplex: 6.7 p. cent on myopic glaucoma: 7 p. cent on pigmentary glaucoma: 29.5 p. cent on capsular glaucoma: 6 p. cent after trabeculectomy: 62 p. cent on aphakic glaucoma: 50 p. cent on secondary glaucoma: 33 p. cent on combined glaucoma: 0 p. cent In the group of successful results, ALT allowed to lower medical treatment in at less 70 p. cent after one year of follow-up except for pigmentary glaucoma where, in almost all cases, medication before ALT had to be continued.     

Pseudoexfoliation syndrome. Clinical and statistical studies

Between July 1, 1980 and December 31, 1982, out of 26 825 patients treated in both the outpatient and inpatient departments at the University Eye Clinic of Erlangen-Nürnberg, 107 (i.e., 0.4%) were found to have pseudo-exfoliation syndrome. In 51 cases only one eye was affected, in 56 cases both. Of these patients 49 were male and 58 female. They were between 54 and 89 years old, the average age being 73.2 years. In 61.3% of the cases the initial symptom was ocular hypertension. In about 75% the most important finding on slit-lamp examination, with the pupil undilated, was atrophy of the pupillary margin. A central disc was only seen in 39.3%, while the peripheral band was present in all cases. Fistulating operations had to be performed in 43 out of 100 cases diagnosed as having capsular glaucoma. This corresponds to 18.5% of all fistulating procedures performed for primary chronic open-angle glaucoma.     

Central retinal vein occlusion in pseudoexfoliation of the lens capsule

In surveying an extensive group of patients with unilateral pseudoexfoliation of the lens capsule certain interesting points emerged, particularly on the incidence of central retinal vein occlusion in this condition,and perhaps in glaucoma also. A total of 284 patients with unilateral pseudo-exfoliation of the lens capsule were examined clinically after an ophthalmic and general history were taken. Mean intraocular pressure (IOP) was 30.9 mmHg in the affected eye and 18.1 mmHg in the unaffected eye. Central retinal vein occlusion occurred in 10 affected eyes but no unaffected eyes and always in affected eyes with a rise in IOP. Average IOP in the affected eye with central retinal vein occlusion was 37.5 mmHg (range 26-54 mmHg);in the unaffected fellow eye it was 18.0 mmHg (range 11-28 mmHg). Although the process involved in producing pseudo-exfoliation of the lens capsule may contribute to the precipitation of central retinal vein occlusion, these findings suggest the rise in IOP is the more important cause. This may also be so in other forms of glaucoma.     

Pigment dispersion syndrome: a clinical study.

This study involved a group of 407 patients (799 eyes) with pigment dispersion syndrome gathered from a glaucoma population of 9200 patients. The sex distribution was equal. The majority (65%) of patients were myopic. The incidence of retinal detachment was 6.4%. No patients were black, but 5 were mulatto. Approximately one-quarter of the patients wih pigment dispersion syndrome (31% of the men, 19% of the women) had glaucoma. The average age of onset of glaucoma was 15 years less than in control patients with chronic simple glaucoma. When both eyes were affected by glaucoma, the glaucoma was consistently more severe in the eye with the more heavily pigmented angle. The degree of iris transillumination was found to be of no importance in predicting the presence of glaucoma or the severity of trabecular pigmentation. The pressure in 66% of the eyes with pigmentary glaucoma was controlled medically. A higher percentage of patients with pigmentary glaucoma required surgery than patients in the control group with chronic simple glaucoma. Men with pigmentary glaucoma required surgery at a much earlier age than women with pigmentary glaucoma.     

Functional damage at diagnosis of primary open angle glaucoma

In a well defined population, all patients with newly diagnosed primary open angle glaucoma were classified according to the degree of functional damage at diagnosis. A comparison was made between patients with simple and capsular glaucoma. No difference could be found between the 2 types of glaucoma when the severity of visual damage at diagnosis was compared. Eyes with capsular glaucoma were found to have significantly higher intraocular pressure than eyes with simple glaucoma in corresponding glaucoma stage. No obvious correlation between age and functional damage at diagnosis was found.     

Optic disc morphology in pigmentary glaucoma

AIM—To evaluate the morphology of the optic nerve head in eyes with pigmentary glaucoma.
METHODS—Colour stereo optic disc photographs of 62 patients with pigmentary glaucoma and 566 patients with primary open angle glaucoma were morphometrically evaluated. By prestudy selection, mean visual field defect and neuroretinal rim area were not significantly different between the two groups (p=0.89 and p=0.45).
RESULTS—The pigmentary glaucoma group did not vary significantly (p >0.10) from the primary open angle glaucoma group in size and shape of the optic disc, configuration of neuroretinal rim, depth of optic cup, area of alpha zone of parapapillary atrophy, diameter of retinal vessels at the disc border, and frequency of disc haemorrhages and localised retinal nerve fibre layer defects. The beta zone of parapapillary atrophy was slightly, but not statistically significantly (p=0.06), smaller in the pigmentary glaucoma group. The mean maximal intraocular pressure and mean intraocular pressure amplitude were significantly (p<0.001) higher in the pigmentary glaucoma group.
CONCLUSIONS—In contrast with the characteristic morphology of the anterior segment and despite significantly higher intraocular pressure peaks and a larger pressure amplitude, eyes with pigmentary glaucoma compared with eyes with primary open angle glaucoma do not show a pathognomonic morphology of the optic disc and retinal nerve fibre layer. The slightly smaller beta zone of parapapillary atrophy may correspond to higher intraocular pressure in pigmentary glaucoma.

Keywords: optic disc morphology; pigmentary glaucoma; secondary open angle glaucoma