Presumed combined hamartoma of the retina and retinal pigment epithelium with preretinal neovascularization

PURPOSE: To describe a case of presumed combined hamartoma of the retina and retinal pigment epithelium associated with preretinal neovascularization. DESIGN: Observational case report. METHODS: We report clinical and angiographic findings of a 26-year-old woman. RESULTS: The patient presented with mild vitreous hemorrhage and slowly decreasing vision in the right eye. A combined hamartoma of the midperipheral retina and retinal pigment epithelium with an epiretinal membrane causing traction to the macula was found. Fluorescein angiography showed areas of capillary nonperfusion and a large preretinal neovascularization peripheral to the hamartoma. CONCLUSIONS: A combined hamartoma may be associated with retinal capillary nonperfusion and preretinal neovascularization, suggesting that significant retinal ischemia can occur with a combined hamartoma.     

Proliferative sarcoid retinopathy

Sarcoidosis is a well-established cause of ocular neovascularization. A review of the literature, however; shows that it has been implicated as the cause for retinal neovascularization in only a limited number of patients. In this article, the authors report the clinical features of proliferative sarcoid retinopathy in seven additional patients (11 eyes). All 11 eyes displayed retinal neovascularization. In addition, two of the eyes had optic disc neovascularization, whereas iris neovascularization developed in one. In all cases, the new retinal vessels were associated with concomitant peripheral retinal capillary nonperfusion. These findings support the theory that in sarcoidosis, capillary nonperfusion secondary to microvascular shutdown, rather than a direct effect of inflammation, is the stimulus for the formation of retinal neovascularization.     

Disk and peripheral retinal neovascularization secondary to talc and cornstarch emboli.

A 30-year-old man was examined because of blurred vision. The symptoms had occurred immediately after the patient had injected himself intravenously with crushed, unfiltered, methylphenidate HCl (Ritalin) tablets. Fine yellow-white crystals were scattered throughout the retin of both eyes. The crystals were presumed to be talc emboli. Disk and peripheral retinal neovascularization were present. Fluorescein angiography revealed widespread retinal capillary nonperfusion. This is the first reported case, to the best of my knowledge, of disk neovascularization occurring after intravenously injected, crushed, unfiltered, methylphenidate HCl tablets.     

Classification of proliferative diabetic retinopathy

Using panretinal fluorescein angiography, three patterns (A, B, C) of capillary nonperfusion were identified in 308 eyes with proliferative diabetic retinopathy. Statistical analysis showed that there was a significant association with different retinal complications and clinical parameters. Pattern A (83.7%: midperipheral location of capillary nonperfusion) occurs in type I and II diabetes and is associated with early retinal neovascularization and focal macular edema. Pattern B (8.1%: capillary exclusions disseminated on the whole retina) is typical of young type-I diabetics and is complicated by early disc new vessels and ischemic maculopathy. Pattern C (8.1 %: capillary nonperfusion confined to the peripheral retina) is observed in type-I diabetic females and associated with multiple, retinal new vessels, without maculopathy. This study also demonstrated that eyes with pattern B retinal ischemia respond less well to laser treatment than eyes with other pattern types. Various pathogenetic factors could lead to these three distinct types of proliferative diabetic retinopathy.Presented at the XVth Meeting of the Club Jules Gonin, Copenhagen, 10-15 August 1986     

Characteristics of the Hemispheric Retinal Vein Occlusion

Hemispheric retinal vein occlusions involve the venous return from approximately one-half of the retina and have characteristics of both central retinal vein occlusions and branch retinal vein occlusions. One hundred six such occlusions were studied in 104 patients whose average age was 66 years. The site of the hemispheric vein occlusion was located in a branch retinal vein in 90% of the eyes, and in 10% of the eyes it was located in one of the dual intraneural trunks of the central retinal vein. Of these eyes, 11 developed neovascularization of the disc, 9% developed neovascularization of the retina elsewhere, 9% developed neovascularization of the iris, and 3% developed neovascular glaucoma. Neovascularization was positively correlated with an increasing percentage of capillary nonperfusion (ischemic index) within the area of occlusion. Prophylactic argon laser treatment was effective in reducing the incidence of neovascularization in eyes with significant capillary nonperfusion.     

早期PRP与复方樟柳碱颞浅动脉旁穴位注射治疗RR的疗效

目的：观察早期视网膜激光光凝（PRP）联合复方樟柳碱颞浅动脉旁皮下注射治疗放射性视网膜病变（RR）的临床疗效。方法：在我院确诊为因鼻咽癌外照射后发生RR的患者21例41眼,早期行双眼视网膜激光光凝联合复方樟柳碱颞浅动脉旁穴位注射治疗,观察治疗后3mo最佳矫正视力（BCVA）、毛细血管无灌注区变化、视网膜新生血管及并发症情况。结果：治疗后观察3mo,视力提高6眼（14.6%）,视力不变31眼（75.7%）,下降4眼（9.7%）。2眼虹膜新生血管消退,1眼行睫状体光凝术;9眼（75%）视网膜新生血管消退;22眼（68.7%）原视网膜无灌注区消失,7眼（21.8%）原视网膜无灌注区（NPA）缩小〉5个DA,总有效率90.5%。1眼发生玻璃体出血,未见视盘及虹膜新生血管和新生血管性青光眼等并发症以及与治疗相关的并发症。结论：早期PRP与复方樟柳碱颞浅动脉旁穴位注射治疗是有效的、合理的中西医结合治疗方法。     

Talc emboli and retinal neovascularization in a drug abuser.

A 38-year-old male drug abuser had multiple emboli in the retinal circulation of the posterior pole of both eyes. He showed widespread peripheral retinal capillary nonperfusion and neovascular proliferation at the junction of perfused and nonperfused retina. The emboli were considered to be talc particles from the intravenous administration of suspensions of oral medications. The presumed mechanism of development of neovascularization in this case was the filtering out of the particles by the retinal vasculature with vaso-occlusion, ischemia and subsequent retinal neovascularization.     

Talc retinopathy

Twenty-three patients had bilateral intraretinal talc microemboli, which were seen on ophthalmoscopic examination. Twelve of these patients had additional retinal vascular or choroidal abnormalities in the posterior pole that were confirmed by fluorescein angiography. Six patients had peripheral retinal neovascularization associated with peripheral retinal vascular nonperfusion. Of these six patients, three had associated vitreous hemorrhage, and two had optic disk neovascularization and traction retinal detachment. The progressive fundus changes seen in talc retinopathy were documented by fluorescein angiography and compared to those seen in sickle-cell retinopathy. One eye in one patient was treated by argon laser photocoagulation for proliferative retinopathy, and three eyes in two patients with vitreous hemorrhage were treated by pars plana vitrectomy.     

Retinal fibrovascular proliferation associated with Nocardia subretinal abscess

PURPOSE: To report the development of extensive fibrovascular proliferation in association with Nocardia subretinal abscess. METHOD: Case report. RESULTS: Extensive retinal neovascularization with tractional retinal detachment developed soon after ocular involvement in a 61-year-old patient with systemic nocardiosis. Fundus fluorescein angiography showed extensive area of capillary nonperfusion and severe leakage from the neovascular complex. The Nocardia subretinal abscess responded to systemic antibiotics, and the retinal neovascularization and tractional retinal detachment stabilized after 3 months. CONCLUSIONS: Retinal ischemia and severe retinal neovascularization may complicate intraocular nocardiosis. The authors propose secondary retinal vasculitis as a contributing factor towards the development of retinal ischemia in this setting.     

Role of the vitreous in branch retinal vein occlusion

We analyzed the vitreous findings in 62 patients (62 eyes) with major branch retinal vein occlusion to determine whether vitreous examination was useful in predicting the development of retinal or disk neovascularization, or both. In 18 eyes with no or partial posterior vitreous detachment and large areas of capillary nonperfusion (5 disk diameters or more), ten eyes (55.6%) eventually developed neovascularization. Only three of the remaining 44 eyes (6.8%) developed neovascularization. The probability of this development was greater in eyes with no partial posterior vitreous detachment at the initial vitreous examination (P = .0177, Cox's regression analysis), and in those with large areas of nonperfusion (P = .0097, Cox's regression analysis).     

Perimetric sensitivity with the micro perimeter 1 and retinal thickness in patients with branch retinal vein occlusion

PURPOSE: To evaluate the relationship between retinal sensitivity and thickness of the macular edema associated with branch retinal vein occlusion (BRVO). DESIGN: Prospective case series. METHODS: We prospectively examined 15 eyes of 15 patients with macular edema associated with BRVO. In each patient, retinal sensitivity in the macular area was examined with the Micro Perimeter 1 (MP-1); retinal thickness was measured by optical coherence tomography (OCT). Eyes with marked retinal hemorrhage or macular nonperfusion were excluded from the current study. RESULTS: Retinal sensitivity measured with the MP-1 was significantly correlated with retinal thickness in the fovea (R(2) = 0.460) and in all extrafoveal regions (R(2) = 0.383 to 0.794). In four eyes treated with triamcinolone acetonide, retinal sensitivity showed marked improvement in parallel with the reduction of the macular edema. CONCLUSIONS: Retinal sensitivity in the macular area correlates linearly and negatively with increased retinal thickness in eyes with BRVO.     

Retinal angiopathy and polypoidal choroidal vasculopathy

PURPOSE: To describe the clinical and angiographic features of patients with polypoidal choroidal vasculopathy, exudative detachment of the macula, and an associated retinal microangiopathy. METHODS: Case series. RESULTS: Four patients with chronic exudative detachment of the macula with a variable degree of lipid deposition are described. The retina in the detached area, but not beyond, was noted to have a microangiopathy. There was capillary telangiectasia, microaneurysm formation, patchy nonperfusion, and intraretinal leakage. In each patient, there were no other retinal vascular changes in the fundus of either eye. The fluorescein angiogram showed subretinal leakage suspicious for occult choroidal neovascularization. The indocyanine green angiogram showed the presence of underlying polypoidal choroidal neovascularization, accounting for the exudative detachment. After photocoagulation, the retinal angiopathy improved, but not completely. CONCLUSION: Retinal microangiopathy may occur in a chronic macular detachment secondary to polypoidal choroidal neovascularization. The development of these secondary retinal changes is not clearly understood; however, hypoxia from the chronic detachment, a neurotoxic effect from the lipid deposition, or a biochemically induced microvascular abnormality from secretion of vasogenic mediators are possible mechanisms. Indocyanine green angiography is helpful in making a definitive diagnosis. Clinicians should be aware that a retinal microangiopathy may occur in such eyes so that the proper diagnosis can be made and appropriate treatment administered.     

Macular infarction in thrombotic thrombocytopenic purpura

Purpose: To report a patient with transient acute generalized thrombotic thrombocytopenic purpura (TTP) who suffered severe permanent visual loss due to retinal vascular occlusions in both eyes. Methods: Case report. Results: Severe permanent visual loss occurred in a patient with acute transient TTP. Extensive soft exudates were seen at the fundus of both eyes. Fluorescein angiography demonstrated multiple retinal vascular occlusions associated with capillary nonperfusion. Conclusions: Severe and permanent visual loss due to retinal vascular occlusions demonstrated by fluorescein angiography occurred in a patient with transient thrombotic thrombocytopenic purpura.     

Peripheral circumferential retinal scatter photocoagulation for the treatment of proliferative sickle retinopathy. An update

Forty-four patients (70 eyes, 220 sea fans) with proliferative sickle retinopathy (PSR) received peripheral circumferential retinal scatter photocoagulation (PCRP) to the peripheral zones of retinal capillary nonperfusion. With an average follow-up of 3.3 years, 33% of preexisting sea fans regressed completely, 46% regressed partially, 19% remained stable, and 2% showed progression. De novo neovascularization developed after treatment in only one eye (1.4%). Ninety-five percent of patients treated had a final visual acuity of 20/30 or better and only one patient (2%) developed a nonresolving vitreous hemorrhage requiring vitrectomy surgery. These results confirm the authors' previous report and continue to compare favorably to the natural history of PSR in which de novo lesions developed in 58%, and 12% of eyes ended with a visual acuity of 20/200 or less. PCRP seems preferable to other techniques in reducing the risk of nonresolving vitreous hemorrhage and/or traction retinal detachment, although randomized controlled studies are needed to conclusively assess the safety and efficacy of this strategy in treating PSR.     

Multiple retinal vein occlusions in essential thrombocythemia.

PURPOSE: To report the ophthalmoscopic and angiographic evidence of both temporally and spatially independent multiple occlusions of the retinal veins in a patient with essential thrombocythemia. METHODS: Observational case report. Ophthalmic examinations, including fluorescein fundus angiogram, were performed on a 77-year-old male, who was found to have essential thrombocythemia. RESULTS: An acute impending central retinal vein occlusion in the left eye was diagnosed with coexisting old retinal vein occlusions evidenced by white vessels and capillary nonperfusion in both eyes. Peripheral retinal capillary dropout was also found angiographically in the right eye. CONCLUSION: Bilateral multiple occlusions of retinal veins in the present case suggest a prothrombotic tendency in retinal circulation in essential thrombocythemia.     

Pathologic features of vascular endothelial growth factor-induced retinopathy in the nonhuman primate

PURPOSE: Vascular endothelial growth factor (VEGF) is a potent ischemia-upregulated angiogenic protein that has been implicated in diabetic retinopathy. Intravitreal VEGF injections have not previously been shown to produce preretinal neovascularization. The purpose of this study was to further characterize the angiopathic changes that occur after intravitreal injections in a nonhuman primate and determine if preretinal neovascularization develops. DESIGN: Experimental animal study. METHODS: Vascular endothelial growth factor 165 was injected into the eyes of normal cynomolgus monkeys at regular intervals. As a control, normal eyes were injected with phosphate buffered saline. Color photography and fluorescein angiography were performed at regular intervals. The retinas were incubated for adenosine diphosphatase (ADPase) activity to visualize retinal vessels. The retinas were flat-embedded and areas of potential preretinal neovascularization were identified en bloc and serially sectioned. RESULTS: Areas of capillary nonperfusion and vessel dilation and tortuousity were seen by angiography. In serial sections, the nonperfused areas were found to be associated with endothelial cell hyperplasia in vessel lumens. Preretinal neovascularization originating only from superficial veins and venules was observed throughout peripheral retina, but was not seen in the posterior pole. Lacunae-like veins were subdivided by the process of intussusception and endothelial cell bridging. Arterioles demonstrated endothelial cell hyperplasia and microaneurysms. CONCLUSION: Intraocular injections of VEGF were sufficient to produce preretinal neovascularization in the nonhuman primate. Most vasculopathic structures were associated with endothelial cell hyperplasia. These results demonstrate that VEGF alone can produce many features of both nonproliferative and proliferative diabetic retinopathy including the previously undescribed development of preretinal neovascularization. This well-characterized VEGF-induced primate model of retinal neovascularization may be useful as a means of testing new treatments for retinal neovascularization.     

特发性视网膜血管炎、动脉瘤、视神经视网膜炎综合征和Eales病荧光素眼底血管造影对比分析

目的 观察特发性视网膜血管炎、动脉瘤、视神经视网膜（IRVAN）综合征和Eales病荧光素眼底荧光血管造影（FFA）特征的异同。方法 回顾分析IRVAN综合征患者4例8只眼和Eales病患者43例68只眼的FFA检查资料。所有患者均行常规眼科检查,包括视力、眼压、裂隙灯显微镜、间接检眼镜眼底检查。4例Eales病患者单眼玻璃体积血眼底窥不清,行对侧眼检查,其他所有患者双眼常规彩色眼底照相、共焦激光扫描FFA检查。IRVAN综合征4例8只眼,均为双眼发病。男性1例, 女性3例;年龄16～43岁,平均年龄（27.00±12.93）岁。Eales病43例68只眼, 男性32例, 女性11例;年龄6～59岁,平均年龄（30.79±11.46）岁。双眼29例54只眼,单眼14例14只眼。两组患者眼底均可见视网膜血管白鞘或白线状改变、视网膜出血渗出、玻璃体积血。结果 FFA检查结果显示,IRVAN综合征8只眼后极部动、静脉均受累;均可见多发性视网膜大动脉瘤。周边部毛细血管闭塞形成无灌注区7只眼,占87.50%;视盘水肿荧光渗漏5只眼,占62.50%;视神经萎缩2只眼,25.00%;出血遮挡1只眼,占12.50%;视盘新生血管2只眼,占25.00%;视网膜新生血管4只眼,占50.00%;黄斑水肿4只眼,50.00%。Eales病43例68只眼中,所有患眼周边病变区视网膜静脉管壁渗漏;累及后极部静脉32只眼,占47.06%;动脉同时受累5只眼,占7.35%;周边部毛细血管闭塞形成无灌注区38只眼,占55.88%;视盘渗漏29只眼,占42.65%;视盘新生血管4只眼,占5.88%;视网膜新生血管26只眼,占38.24%;黄斑水肿15只眼,占22.06%。IRVAN综合征与Eales病患者在后极部动脉受累眼数、静脉受累眼数、动脉瘤眼数之间比较,差异有统计学意义(P均＜0.05);视盘渗漏、无灌注区、视盘和视网膜新生血管、黄斑水肿眼数之间比较,差异无统计学意义(χ2=0.479,1.449,0.068,1.676;P＞0.05)。结论 IRVAN综合征和Eales病均可发生视网膜动脉和静脉不同程度的炎性改变,并均可导致视盘水肿渗出。IRVAN综合征后极部视网膜动、静脉受累明显高于Eales病,特别是视盘旁及后极部特征性大动脉瘤样改变有助于IRVAN综合征的诊断及IRVAN综合征和Eales病的鉴别诊断。     

视网膜分支静脉阻塞和新生血管形成

分析视网膜分支静脉阻塞240例245只眼(5人为双眼)，其中主干分支阻塞208只眼，黄斑分支阻塞37只眼。79只眼产生了新生血管，占总数的32.2%，占主干分支阻塞的37.9%．玻璃体出血39只眼，占分支阻塞的15.9%，占新生血管的49.4%。新生血管的危险因素是无灌注区形成、动脉灌注不良、阻塞处静脉严重受压．新生血管的发生随病程时间延长而增高，病程5年以上者高达83.3%。分支静脉阻塞盲目者20只眼占8.2%，其中因新生血管及并发症致盲者14只眼，占盲目的70%，占分支静脉阻塞的5.7%. （中华眼底病杂志，1994，10：67-70）     

Peripheral circumferential retinal scatter photocoagulation for treatment of proliferative sickle retinopathy

Twenty-nine patients (40 eyes) with proliferative sickle retinopathy (PSR) were treated with circumferential argon laser scatter photocoagulation to zones of peripheral retinal capillary nonperfusion. Each eye was examined in a prospective fashion, with a mean follow-up of 1.4 years. Following scatter treatment, 26% of the pre-existing sea fans regressed completely, 57% regressed partially, 17% remained stable, and none showed progression. In only one eye did de novo neovascularization develop. The results compare favorably with the natural history of PSR (Condon et al, 1980), in which de novo lesions occurred in 58%, and the visual acuity was decreased to less than 20/200 in 12% of affected eyes. Severe iatrogenic complications reported following direct focal treatment of sea fans, such as vitreous hemorrhage, progressive traction retinal detachment, retinal breaks, and choroidal-retinal-vitreal neovascularization were virtually eliminated.     

Ocular manifestations in Dengue fever

PURPOSE: To report a case of Dengue fever resulting in permanent visual loss in both eyes due to retinal capillary occlusion. METHODS: Case report. RESULTS: Severe permanent visual loss occurred in a patient with Dengue fever. Dilated fundus exam showed vascular sheathing with associated retinal hemorrhages at the equator and cotton wool spots in the maculae of both eyes. Fluorescein angiography revealed areas of capillary nonperfusion at the equator and in the macula. The diagnosis of Dengue fever was confirmed by serology detecting IgM antibodies to the Dengue virus. CONCLUSION: Ocular abnormalities may be seen in patients with Dengue fever, therefore ophthalmoscopy should be performed in patients presenting with severe forms of the disease.