PIGMENTARY GLAUCOMA: A CLINICAL REVIEW OF ANTERIOR SEGMENT PIGMENT DISPERSAL SYNDROME

Sixty-eight cases (44 males, 24 females) of anterior segment pigment dispersal syndrome are reviewed. Five patients had no rise in intraocular pressure, 38 had ocular hypertension and 25 had glaucoma as shown by optic disc cupping and field loss. All cases had heavy deposition of pigment in the drainage angle together with Krukenberg spindles and/or light reflux through the iris. Other evidence of anterior segment pigment dispersal was common. The condition was commonest in young adult males, but could present at any age and in women. Medical treatment was often successful initially, but continued to control pressure in only 17 of 42 patients.
Laser trabeculoplasty helped some patients. Drainage operations of various types were successful in 19 of 23 cases. Iris angiography showed evidence of vascular hypoperfusion of the iris which probably precedes pigment dispersal and suggests that the ultimate aetiology may be a congenital deficiency of the mesodermal support tissues of the iris. Some degree of iris stromal atrophy is also common and may become marked. Pigment dispersal from the pigment epithelium of the iris occurs in susceptible individuals and may be associated with the insertion of the dilator muscle. The term anterior segment pigment dispersal seems appropriate as the condition is limited to the anterior segment.     

Fluorescein angiography of the iris in anterior segment pigment dispersal syndrome.

The results are presented of fluorescein angiography of the iris in 11 patients with anterior segment pigment dispersal syndrome. These show a general hypovascularity of the iris with fine neovascularisation at the pupil margin and the peripupillary area. Hypoplasia of the iris stroma was also present in many cases. When the condition was virtually unilateral, the vascular changes were present though less marked in the relatively unaffected eye. It is postulated that the anterior segment pigment dispersal syndrome is secondary to a congenital mesodermal deficiency of the iris stroma with hypovascularity of the iris, which forms a poor support tissue for the pigment epithelium of the iris, resulting in shedding of pigment granules particularly in the region of the attachment of the dilator muscle to the pigment epithelium. The condition may be hereditary. Because of the hypovascularity the mesodermal hypoplasia may be progressive, but pigment release may diminish in later life with treatment, with consequent diminution of pupil activity.     

Recurrent microhyphema in the pseudophakic eye

Although painless transient obscurations of vision are usually attributed to conditions affecting the posterior segment, careful slit-lamp, gonioscopic, and angiographic evaluations may establish an anterior segment origin for these symptoms. Recurrent hyphema, an uncommon late complication following routine cataract extraction, can be caused by spontaneous rupture of incisional vessels. In pseudophakic eyes, the uveitis-glaucoma-hyphema (UGH) syndrome and intermittent visual "white out" are late complications that may necessitate removal of the implant. The authors report five patients with uncomplicated extracapsular cataract extraction and implantation of a Binkhorst biplane, two-loop, capsule-fixated lens who presented with episodes of visual white out on the basis of iris bleeding. Argon laser photocoagulation to sites of iris leakage, at points of lens iris contact, resulted in prompt cessation of bleeding. Laser treatment appears to be a safe and effective alternative to removal of an otherwise satisfactory intraocular lens.     

Pigmentary glaucoma: a clinical review of anterior segment pigment dispersal syndrome

Sixty-eight cases (44 males, 24 females) of anterior segment pigment dispersal syndrome are reviewed. Five patients had no rise in intraocular pressure, 38 had ocular hypertension and 25 had glaucoma as shown by optic disc cupping and field loss. All cases had heavy deposition of pigment in the drainage angle together with Krukenberg spindles and/or light reflux through the iris. Other evidence of anterior segment pigment dispersal was common. The condition was commonest in young adult males, but could present at any age and in women. Medical treatment was often successful initially, but continued to control pressure in only 17 of 42 patients. Laser trabeculoplasty helped some patients. Drainage operations of various types were successful in 19 of 23 cases. Iris angiography showed evidence of vascular hypoperfusion of the iris which probably precedes pigment dispersal and suggests that the ultimate aetiology may be a congenital deficiency of the mesodermal support tissues of the iris. Some degree of iris stromal atrophy is also common and may become marked. Pigment dispersal from the pigment epithelium of the iris occurs in susceptible individuals and may be associated with the insertion of the dilator muscle. The term anterior segment pigment dispersal seems appropriate as the condition is limited to the anterior segment.     

CHANGES IN THE IRIS VASCULATURE AND CORNEAL ENDOTHELIUM IN CHRONIC CYCLITIS

Fluorescein angiography of the iris vasculature and specular microscopy of the corneal endothelium were used to examine two important anterior chamber tissues in cases of heterochromic cyclitis, classical chronic cyclitis and some cases of iridocyclitis. The 13 cases of heterochromic cyclitis and eight cases of classical chronic cyclitis all showed vascular hypoperfusion of the iris, except for two young females with heterochromia. In these cyclitis eyes there was pupillary leakage of dye and all but one young female with heterochromia showed evidence of microneovascularization of the iris. Most patients showed evidence of abnormalities of the corneal endothelium, but this was most marked in elderly patients with heterochromic cychtis, probably due to the long duration of the condition. Six of the patients with chronic cyclitis had bilateral disease and five of these were elderly women with glaucoma and grey irides, the group being distinct enough to form a discrete clinical entity. Seventeen of the patients with chronic cyclitis were females with grey irides suggesting that this type of patient is more susceptible to cyclitis. Patients with acute iridocyclitis showed little abnormality on fluorescein angiography, while those with recurrent or chronic disease showed vascular hypoperfusion and microneovascularization suggesting that these changes in the iris contribute to the chronicity of the condition. It is suggested that hypoperfusion and microneovascularization of the iris and abnormal permeability are important factors in the production of chronic cyclitis, and that the integrity of the corneal endothelium is affected by these changes if they operate over a long period of time.     

Application of optical coherence tomography angiography in ocular anterior segment diseases北大核心CSCD

Optical coherence tomography angiography (OCTA) is a rapid, noninvasive new blood flow imaging technique that was first applied to retinal retinopathy and acquired retinal vascular imaging in only a few seconds. Compared with the traditional optical coherence tomography(OCT), OCTA analyze has the advantages of higher resolution, faster scanning speed, quantitative blood flow, etc. OCTA not only can more accurately and qualitatively analyze ocular vascular morphology, but also can measure blood vessels and blood flow perfusion noninvasively and quantitatively. With the application of anti angiogenesis drugs in the anterior segment, it is important to evaluate the neovascularization of the cornea and iris in order to guide the clinical medication and evaluate the therapeutic effect. This article reviewed the application of OCTA in ocular anterior segment diseases, such as cornea and iris diseases, etc. Copyright © 2018 by the Chinese Medical Association.     

Scanning laser ophthalmoscopy of choroidal neovascularisation using indocyanine green

Background: Choroidal neovascularisation is an important cause of visual loss in age-related macular degeneration. Visualisation of these choroidal abnormalities with fluorescein angiography may be difficult, particularly if there is haemorrhage, excessive pigment, or excessive fluorescence. We report on our experience with indocyanine green (ICG) angiography in demonstrating choroidal neovascularisation.
Method: Patients with suspected choroidal neovascularisation were assessed with indocyanine green angiography using the Rodenstock Scanning Laser Ophthalmoscope and results recorded on a videotape. Fluorescein angiograms were also obtained in a similar fashion. The angiograms were then compared.
Results: In some instances ICG angiography was able to demonstrate the margins and structure of choroidal neovascular membranes more clearly than fluorescein, particularly in the presence of haemorrphage, excessive fluorescence and blocked fluorescence. However, there were instances where it was difficult to interpret the choroidal abnormalities found. Some illustrative cases are discussed.
Conclusion: We conclude that ICG angiography is a useful adjunct to fluorescein angiography in detection and delineation of choroidal neovascularisation, but that further study is needed.     

Vascular changes in the iris in chronic anterior uveitis.

Vascular changes of the iris were studied in 6 patients with chronic anterior uveitis of varying aetiology by means of fluorescein angiography. In 1 case the angiographic findings of the second eye were compared with the histopathological changes in the fellow eye enucleated earlier because of absolute glaucoma. In the milder cases abnormal superficial vessels were seen mainly in relation to the minor arterial circle of the iris, while in advanced cases a coarse vascular network covered the whole surface of the iris. Both mild and severe changes were found to differ from the arborising type of neovascularisation usually seen in vascular eye diseases. Instead, a resemblance in the vascular pattern was found between some of these cases and eyes with chronic capsular glaucoma. In 1 patient clinical rubeosis seemed to be partly due to dilatation of the pre-existing capillaries rather than true neovascularisation.     

Metastatic carcinoma of the iris.

Of three women and one man with metastatic carcinoma of the iris, the average age of the four was 50.2 years. Two of the tumors originated in the breast and two in the lungs. The average survival from time of diagnosis of the iris lesion was less than six months. Each patient presented with characteristic grayish-white translucent nodules on the iris or in the anterior chamber angle. The patients displayed other features of iris metastases such as secondary glaucoma, rubeosis iridis, iris atrophy, anterior uveitis, hypopyon, and hyphema. As an aid in the clinical evaluation, anterior segment fluorescein angiography was performed, revealing vascular details not seen clinically. Leakage of dye was a constant feature of the angiogram.     

Iris atrophy with hypoperfusion and microneovascularisation.

A series of 17 patients with stromal atrophy, hypoperfusion, and microneovascularisation of the iris investigated in the Glaucoma Investigation and Research Unit are described, and their iris angiograms were compared with those of normal irides of patients in the same age group seen in general clinics. In all but one of the 17 cases this iris atrophy was associated with glaucoma or ocular hypertension, which appeared to be secondary to the iris changes. The condition was bilateral and presented a typical slit-lamp appearance, with subtle evidence of microneovascularisation. There was neither history nor clinical evidence of previous trauma, heterochromia, or intraocular inflammation. The commonest form of iris atrophy affected the inner third of the iris stroma in a patchy manner, often with sparing above. However, diffuse atrophy occurred in two cases, and there were two cases of 'senile tears' of the iris. Some accompanying atrophy of the pigment epithelium was usual but less prominent. The changes on fluorescein angiography of the iris included the late appearance of dye with a long arteriovenous circulation time, fewer arteries than normal with sectorial hypoperfusion, leakage of dye from the pupil margin and peripupillary neovascularisation, stromal tufts, and sometimes more complex stromal microneovascularisation. An expanded prominent lesser vascular circle was a common feature of the condition. The condition is bilateral and distinct from other forms of iris atrophy. In all cases the iris changes appeared to be secondary to the vascular hypoperfusion and were not consistently associated with evidence of gross vascular disease. All patients had grey (blue) irides, and this may be an aetiological factor. The condition appears common enough to form a significant group of glaucoma patients and to be a separate clinical entity.     

三种不同造影方式在糖尿病性视网膜病变诊断中的应用对比

目的：比较分析虹膜荧光血管造影(iris fluorescein angiography,IFA)联合眼底荧光血管造影(fundus fluorescein angiography,FFA)、吲哚菁绿血管造影(indocyanine green angiogrsphy,ICGA)和FFA在早期糖尿病性视网膜病变(diabetic retinitis,DR)中的诊断价值。

方法：选择2015-08/2016-08期间我院收治的DR患者70例136眼,全部患者分别接受ICGA、FFA、IFA+FFA检查,分析三种造影方式的检出结果。

结果：FFA检出病变120眼(88.2%),ICGA检出病变124眼(91.2%),IFA+FFA检出病变130眼(95.6%),三种检查方法病变检出率比较,差异无统计学意义(P>0.05); FFA检出48眼出现新生血管,18眼玻璃体积血,38眼黄斑水肿,16眼无灌注区; ICGA检出49眼新生血管,38眼黄斑水肿,17眼玻璃体积血,20眼无灌注区; IFA+FFA检出17眼增殖性糖尿病性虹膜病变(DI),22眼非增殖性DI,5眼NVG,92眼无DI。

结论：在DR诊断中,IFA+FFA、FFA、ICGA均具有不错的诊断价值,但IFA+FFA可及时发现合并的糖尿病虹膜病变,为及时治疗提供帮助。     

Anterior Segment Ischemia in Viper Bite

Purpose: To describe the clinical and histopathological features of post viper bite anterior segment ischemia.

Methods: Seven patients with ocular complications following viper bite referred to uveitis clinic had slit-lamp examination, intraocular pressure (IOP) measurement, and fundus evaluation. Iris and fundus fluorescein angiography was performed on 2 patients. Histopathological examination was performed on iris tissues collected during cataract surgery.

Results: Strikingly similar clinical findings were noted, including circumpupillary superficial iris atrophy, mid dilated fixed pupil, marked pigment dispersion, low IOP, and cataract. All clinical signs were noted only in the anterior segment; the posterior segment was normal. Histopathology of iris revealed atrophy of iris stroma, necrotic iris pigment epithelium, and infiltration of T lymphocytes and fibrous membrane. Poor visual outcome was noted in patients with low IOP.

Conclusion: Viper bite victims presented with clinical and histopathological signs of anterior segment ischemia and secondary inflammatory signs mimicking uveitis.     

Deconstructing the mechanisms of angle closure with anterior segment optical coherence tomography

Background: To illustrate the common mechanisms of angle closure by observing the changes following a laser iridotomy and then cataract surgery with anterior segment optical coherence tomography. Design: Retrospective interventional case series. Participants: Four patients with iridotrabecular contact. Methods: The anterior chamber was imaged in different lighting conditions prior to and following a laser iridotomy and then cataract surgery. The images were superimposed on each other, using the interscleral spur line as a common baseline. Main Outcome Measures: Qualitative and quantitative analysis of the changes of the iris and the drainage angle. Results: Iridotrabecular contact was seen in dark conditions in all patients. Iridotrabecular contact related to pupil block was abolished after a laser iridotomy. Persistent iridotrabecular contact following a laser iridotomy, due to a lens‐induced mechanism, was abolished after cataract surgery. Iridotrabecular contact that persisted after a laser iridotomy and cataract surgery was due to a ‘pure’ plateau iris syndrome or peripheral anterior synechiae. These treatments lowered the height of the iris plane in the region posterior to the trabecular meshwork. Conclusion: Following a laser iridotomy and cataract surgery, there is posterior movement of the iris plane away from the trabecular meshwork, but iridotrabecular contact can persist due to the ciliary processes or peripheral anterior synechiae. These changes show that the risk of iridotrabecular contact depends on the height of the iris plane relative to the trabecular meshwork and the degree of physiologic pupil dilation, and that angle closure can be a multi‐mechanism disease rather than a pure one.     

虹膜荧光素血管造影对具有棕色虹膜的中国人前葡萄膜炎的诊断价值

背景 前葡萄膜炎的炎症性损伤与血-房水屏障的破坏有关,裂隙灯下对其病情的检查有一定的限制,而荧光素血管造影能客观显示病情的变化.但目前国内对虹膜荧光素血管造影(IFA)的应用研究较少.目的 观察中国人棕色虹膜葡萄膜炎患者IFA图像,探讨IFA在葡萄膜炎诊断和治疗中的临床应用价值.方法 对正常中国人40人40眼和前葡萄膜炎、全葡萄膜炎累及眼前段的患者13例21眼进行裂隙灯、虹膜彩色照相和IFA检查,探讨前葡萄膜炎患眼IFA表现并与正常人进行对照.结果 正常对照眼IFA不显影,呈色素遮蔽荧光,＞60岁正常老年人4例4眼瞳孔缘可见轻微荧光素渗漏.本组葡萄膜炎患眼IFA均有异常荧光素渗漏,按照前葡萄膜炎的严重程度表现为:(1)瞳孔缘和基质层放射状虹膜血管荧光素渗漏.(2)虹膜弥漫性或局限性萎缩,表现为放射状血管透见荧光.(3)虹膜新生血管(NVI),表现为细线状或花团状强荧光,随造影时间延长,有不同程度的荧光素渗漏.结论 IFA可显示裂隙灯检查不能够显示的虹膜炎症性损害病灶并间接反映血-房水屏障和虹膜血管的损害,IFA表现取决于炎症类型、部位以及炎症活动性的严重程度,有助于判断前葡萄膜炎的病情和监测治疗过程.     

The differential diagnosis of malignant melanoma of the iris. A clinical study of 200 patients

The records of 200 consecutive patients referred to the Ocular Oncology Service of Wills Eye Hospital because of an iris lesion suspected of being an iris melanoma were reviewed. On clinical evaluation, 24% of the lesions met the criteria for the diagnosis of iris melanoma and 76% were diagnosed as simulating lesions (pseudomelanomas). The most common pseudomelanomas included primary iris cyst (38%), iris nevus (31%), essential iris atrophy (5.7%), iris foreign body (4.5%), peripheral anterior synechia (2.5%), and iris metastasis (2.5%). Less frequently encountered pseudomelanomas included aphakic iris cysts, leiomyoma, melanocytoma, reactive lymphoid hyperplasia, adenoma of iris pigment epithelium, iridoschisis, and other miscellaneous entities. The clinical features that are helpful in differentiating the more common iris pseudomelanomas from true melanomas are discussed. Correct clinical identification of these simulating lesions may prevent unnecessary surgery or other treatment.     

Fluorescein iris angiography in nodular sarcoid iritis

The value of fluorescein iris angiography in the diagnosis and follow-up of nodular sarcoid iritis was studied in five patients with nodular iritis and histologically verified generalized sarcoidosis. On the angiograms the sarcoid nodules appeared as hyperfluorescent patches which always exceeded the number of nodules seen by biomicroscopy. Fresh iris nodules were characterized by mild diffuse fluorescence and dilatation and leakage of the adjacent vessels whereas old granulomas were covered by tortuous neovascular vessels which in the active stage of the disease leaked fluorescein profusely. After recovery or between recurrent attacks the amount of leakage decreased or stopped although the neovascular network did not disappear. At recurrences new areas of fluorescence due to fresh nodules were observed in addition to intense leakage from some of the previous lesions. Most of the small nodules were situated in the pupillary part of the iris resulting in dilatation and abnormal permeability of all the peripupillary vessels. The weakness of absence or diffuse leakage from the radial vessels of the iris agrees with the proliferative nature of sarcoid nodular iritis and explains the low-grade symptoms of the patients.     

The complex study of the iris in transformed light

Complex investigations of the iris in a transformed light by methods of iridochromoscopy, iridochromophotography, examination in a polarized light, transillumination in red light and fluorescence angiography was conducted in 25 eyes of patients with open-angle glaucoma, 10 eyes of patients with anterior flaccid uveitis and in 23 healthy eyes (a control group). It was found that in patients with open-angle glaucoma and anterior flaccid uveitis trophic and vascular changes in the iris predominated, as compared with control. The most typical for open-angle glaucoma changes in the iris were atrophy of the stroma with predominant involvement of the pupillary zone, exogenic pigmentation of the pupillary and ciliary zones, destruction of the pigment margin of the pupil, thickening of the anterior border layer, pseudoexfoliation of the pupillary margin, defects in the posterior pigment layer, being more extensive in the pupillary zone, as well as disturbances in permeability of the iris in a combination with hypoperfusion and rare bundles of microneovascularization in the area of a lesser arterial circle and the ciliary zone. In case of flaccid uveitis, the most frequent changes were diffuse atrophy of the iris, efflorescence of the pupillary margin, destruction of the pigment rim of the pupil, multiple defects in the posterior pigment layer along its whole length, increased permeability of vessel, microneovascularization of the iris stroma in a form of a thin vascular network.     

虹膜曲率定量测量及在可疑原发性房角关闭虹膜曲率评估中的应用

目的：探讨Rhinoceros软件定量测量虹膜前表面曲率的精确性,并用于评估可疑原发性房角关闭 （PACS）患者行激光周边虹膜切除术（LPI）前后虹膜曲率的变化。方法：前瞻性研究。选择2016年 10月至2019年1月在台州市立医院计划接受LPI治疗的PACS患者16例（16眼）作为PACS组,Allegro Oculyzer眼前节分析系统获取不同轴位的眼前节图像,采用Rhinoceros 5.0软件定量测量虹膜前表面 曲率半径,并对眼前节图像成像的一致性及测量方法的一致性进行评价。比较接受LPI治疗前后虹 膜曲率的变化,并与年龄、眼别、瞳孔直径匹配的正常人16例（16眼）（作为对照组）进行比较。分 析PACS组虹膜曲率与中央前房深度、前房容积、6 mm处周边前房深度之间的相关性,以及LPI治 疗前后虹膜曲率变化与中央前房深度变化、前房容积变化、6 mm处周边前房深度变化之间的相关 性。采用配对t检验、独立样本t检验及Pearson相关分析对数据进行统计学分析。结果：同一眼眼 前节图像成像的变异系数为3.02%,同一图像测量的变异系数为2.54%。PACS组患者虹膜曲率半 径为（7.81±1.63）mm,接受LPI治疗后虹膜曲率半径为（9.20±2.22）mm,差异具有统计学意义（t= -9.45,P<0.001）。对照组虹膜曲率半径为（9.99±4.00）mm,与PACS组治疗前相比,差异具有统计 学意义（t=-5.69,P<0.001）。PACS组在校正中央前房深度后,0°（r=0.879,P<0.001）、90°（r=0.684, P=0.005）、180°（r=0.619,P=0.014）、270°（r=0.740,P=0.002）轴位虹膜曲率半径与对应轴位6 mm 处周边前房深度之间均有相关性。在接受LPI治疗后,除下方270°（r=0.453,P=0.078）轴位方向外,0° （r=0.693,P=0.003）、90°（r=0.560,P=0.024）、180°（r=0.580,P=0.019）轴位虹膜曲率变化量与对应 轴位6 mm处周边前房深度变化量之间均有相关性。结论：该虹膜曲率定量测量方法具有较好的精 确性和可重复性,并可用于PACS的早期诊断及治疗疗效的量化评估。     

Indocyanine green anterior segment angiography for studying conjunctival vascular changes after trabeculectomy

Background : The aim of the study was to evaluate the use of indocyanine green (ICG) for angiography of the anterior segment to characterize conjunctival and episcleral vasculature changes after trabeculectomy. Methods : This was a prospective evaluation of anterior segment ICG angiography in 10 eyes of 10 patients undergoing trabeculectomy for the first time. Trabeculectomy was performed with intraoperative sponge application of 5‐ fluorouracil (5 cases) or mitomycin C (5 cases). Anterior segment ICG angiography was performed prior to surgery, then at 2 weeks and 2 months after surgery. Results : With ICG, the anterior segment vessels were well delineated, including deep episcleral veins, which have not been clearly shown in previous angiographic techniques. Late phases of the angiogram could also be studied. The vascular alterations after trabeculectomy noted included loss of vascularity over the bleb area and vascular anastomoses along the perimeter of the avascular bleb. Conclusions : Angiography using ICG has potential as an investigative tool to study the conjunctival and episcleral vasculature changes after trabeculectomy.     

血管新生性青光眼的组织学研究

对26只血管新生性青光眼的眼球标本进行了组织学研究,其共同特点是:虹膜前表面有纤维血管膜覆盖,前房角关闭,视网膜、视神经萎缩,生理凹陷扩大加深。其中16/26例虹膜的纤维血管膜有内皮细胞被覆,7/16只标本在内皮细胞下有均质的、且PAS 染色呈阳性的薄膜形成。