Retinal and disc neovascularization in Behçet's disease and efficacy of laser photocoagulation

Background: The vasoocclusive episodes resulting from Behçet's disease can cause capillary dropout and vascular remodeling. Retinal and disc neovascularizations, which occur as a result of occlusive vasculitis, can cause recurrent vitreal hemorrhages and neovascular glaucoma leading to severe visual impairment. Methods: 1080 eyes of 540 patients with Behçet's disease were examined between 1973 and 1993. Of the 912 eyes with posterior segment involvement, laser photocoagulation could be performed in 13 of 25 eyes with disc neovascularization (NVD), 12 of 22 eyes with retinal neovascularization (NVE), and 4 of 6 eyes with NVD and NVE. Laser was directed at areas of NVE and retinal capillary nonperfusion. In cases of NVD, panretinal photocoagulation was performed. Results: The rate of regression of NVD was significantly greater in laser-treated eyes than in the untreated group. The results were similar in cases of NVD with NVE. In eyes with NVE which underwent laser photocoagulation, the NVE regressed. None of the treated eyes developed neovascular glaucoma during the follow-up period. Vitreous hemorrhage occurred in two laser-treated eyes. Conclusion: Laser photocoagulation is successful in preventing complications of retinal and disc neovascularizations. Thus, in cases of occlusive vasculitis associated with Behçet's disease, laser photocoagulation should be considered for prevention of complications such as vitreous hemorrhage and neovascular glaucoma.Presented at the meeting of the Club Jules Gonin, Versaille, France, 8-13 September 1994     

Importance of fluorescein angiographic study in evaluating early retinal changes in Takayasu disease

PURPOSE: To determine the usefulness of fluorescein angiography in studying Takayasu disease. METHODS: We examined 31 eyes in 16 patients with Takayasu disease using indirect ophthalmoscopy, color photography, and fluorescein angiography. Ophthalmoscopic and fluorescein angiographic findings were compared. RESULTS: Fluorescein angiography revealed no additional retinal changes in 10 eyes that had no retinal vein dilatation as seen by indirect ophthalmoscopy. Seven (33%) of 21 eyes that had dilated retinal veins also had additional abnormal findings, such as microaneurysms, arteriovenous shunts, retinal neovascularization, and avascular areas. Some differences in grading the stages of retinopathy were noted with these newly found retinal changes, as compared with the classifications determined by ophthalmoscopy alone. CONCLUSIONS: In Takayasu disease, studying the fundus of patients with fluorescein angiography is particularly important in correctly classifying the stages of retinopathy when the retinal vein appears dilated in ophthalmoscopic observation.     

A new case of acute idiopathic frosted branch angiitis in Europe

CASE REPORT: A 20-year-old male with no history of any systemic or eye disease developed loss of visual acuity in both eyes. White exudates surrounding the retinal veins from the posterior pole to the periphery, retinal edema and hemorrhages in both eyes were evident on ophthalmoscopy. Fluorescein angiography showed leakage of the dye from the veins and extensive staining of the vein walls. A diagnosis of acute frosted branch angiitis was made. Systemic examination revealed axillary, submandibular and inguinal lymphadenopathies. VCA IgM antibody for Epstein-Barr virus was negative and IgG positive. Biopsy was taken of an axillary lymph node; a non-specific inflammatory reaction was found on anatomopathologic study. The patient was started on 90 mg prednisolone daily. After two weeks retinal vasculitis had improved and the lymphadenopathies soon resolved. Small, hard exudates were present in the posterior pole during absorption of the retinal edema and resolution of the vascular inflammation. Systemic prednisolone were reduced progressively and definitively withdrawn two months later. The patient's visual acuity was 20/25 in both eyes. No fibrotic scar tissue or atrophic lesions were noted in either fundus. CONCLUSIONS: We report a new case of acute frosted branch angiitis with an onset and favorable clinical course similar to previous reports. We found the additional presence of lymphadenopathies but have been unable to establish a possible causal agent. To our knowledge, apart from a recent case of frosted branch angiitis-like response in Greece, the present case is the first reported in western Europe.     

Posterior segment involvement in ocular Behçet's disease

PURPOSE: Posterior segment involvement, which can lead to blindness with recurrent inflammatory attacks, has a very important prognostic value in ocular Beh?et's disease (BD). This study evaluated the frequency and characteristics of posterior segment involvement and the causes of visual impairment in patients with ocular BD. METHODS: We retrospectively evaluated 257 eyes of the 131 patients who were followed from 1993 to 2001 in the Uveitis and BD division of SSK Ankara Eye Hospital, Turkey. RESULTS: The mean follow-up was 49.2 +/- 27.4 months. Thirty-one of the palents were female (23.6%) and 100 male (76.4%). The mean age was 25.1 +/- 7.9. Fundus changes were observed in 213 (82.9%) eyes and were bilateral in 87%, and vitritis was present in 239 (93%) eyes. The most frequent fundus changes were vascular sheathing in 61 eyes (23.7%), optic atrophy in 46 (17.9%), macular edema in 29 (11.3%), retinal hemorrhage in 23 (9%), macular scar in 21 (8.2%), optic disc paleness in 19 (7.4%), retinal edema in 17 (6.6%), branch retinal vein occlusion in 15 (5.8%). The most common fluorescein angiography findings were diffuse vascular leakage in 98 (38%), hyperfluorescence of the optic disc in 38 (14.8%) and hyperfluorescence of the macula in 29 eyes (11.3%). Visual acuity was <1/10 in 85 (33%) of the eyes. Optic atrophy was the main cause (54.1%) of permanent visual impairment. CONCLUSIONS: Posterior segment involvement is the most serious ocular complication of BD, leading to blindness with recurrent attacks. Following patients closely, performing fluorescein angiography in all patients diagnosed as BD even they have no clinical ocular involvement, and early treatment are very important in the prognosis of the disease.     

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.     

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。     

中间葡萄膜炎的临床特征与治疗

目的 观察中间葡萄膜炎的临床表现、并发症和治疗效果。方法 回顾分析36例中间葡萄膜炎患者66只眼的临床表现、荧光素眼底血管造影（FFA）特征、治疗效果和预后。患者中男21例，女15例，年龄8～70岁，平均年龄34.8岁。双眼30例，单眼6例。结果 主要临床表现为玻璃体混浊，周边部视网膜血管病变，视盘水肿，黄斑水肿和后囊下白内障。FFA表现为周边部视网膜血管炎性病变，视盘强荧光，黄斑囊样水肿和视网膜静脉染色。经治疗后，31例患者60只眼视力提高，占90.9%；4例5只眼视力稳定，占7.6%；1例1只眼视力下降，占1.5%。导致视力损害的主要并发症有黄斑囊样水肿、后囊下白内障和玻璃体积血。结论 中间葡萄膜炎是一种较常见的双眼慢性进行性眼内炎症，主要累及前部玻璃体、睫状体扁平部和周边部视网膜血管，早期发现和及时治疗可防止发生永久性视力损害。     

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.     

Patterns of macular edema in patients with uveitis: qualitative and quantitative assessment using optical coherence tomography

PURPOSE: To describe the morphologic characteristics of uveitic macular edema by the use of optical coherence tomography (OCT) and to investigate the correlation between tomographic features and visual acuity (VA). DESIGN: Cross-sectional study. PARTICIPANTS: Seventy consecutive patients with uveitis in a university-based practice with a clinical diagnosis of macular edema in at least one eye. Inclusion criteria were: (1) verification of macular edema by OCT, (2) adequate media clarity for fundus visualization, and (3) absence of coexisting ocular disease limiting visual potential. METHODS: Complete ophthalmic examination: best-corrected Snellen VA, slit-lamp examination, fundus biomicroscopy, indirect ophthalmoscopy, and OCT. Fluorescein angiography was performed in selected cases. MAIN OUTCOME MEASURES: Best-corrected Snellen VA and tomographic features of the macula, including macular thickness measurement and correlation of macular thickness with VA. RESULTS: Eighty-four eyes of 60 patients met the inclusion criteria. The mean VA (Snellen test) was 20/36. There were 3 patterns of macular edema: diffuse macular edema (DME), cystoid macular edema (CME), and serous retinal detachment (RD). Serous retinal detachment was detected in 17 eyes (20.2%). Patients were classified into the following groups: DME (46 eyes, 54.8%), CME (21 eyes, 25%), DME and RD (5 eyes, 5.9%), CME and RD (12 eyes, 14.3%). Epiretinal membrane was detected by OCT in 34 eyes (40.5%). Eight eyes (9.5%) demonstrated vitreomacular traction. The mean retinal thickness at the central fovea was 333+/-171 microm (mean +/- standard deviation). Macular edema was located mainly in the outer retinal layers. Eyes with CME had significantly greater retinal thickness measurements than eyes with DME (P<0.001). Multivariate analysis revealed that VA was negatively correlated with increased macular thickness, presence of CME, and RD (P<0.05). CONCLUSIONS: Optical coherence tomography demonstrated 3 patterns of macular edema in patients with uveitis: DME, CME, and RD. Epiretinal membrane coexisted in a significant percentage of patients. In patients with uveitis with clear media, the morphologic features of macular edema and macular thickness correlated with VA.     

Posterior segment manifestations of Rickettsia conorii infection

PURPOSE: To characterize and analyze the posterior segment manifestations of Mediterranean spotted fever (MSF), an infectious disease caused by Rickettsia conorii. DESIGN: Prospective, noncomparative case series. PARTICIPANTS: Thirty patients (60 eyes) with serologically proven MSF at the acute stage. METHODS: Patients underwent complete ophthalmic examination, including dilated biomicroscopic fundus examination, fundus photography, and fluorescein angiography. Sequential follow-up examinations were performed in patients with evidence of posterior segment involvement. RESULTS: Of 30 patients, 25 (83.3%) had unilateral (n = 5) or bilateral (n = 20) posterior segment involvement related to MSF. Of those 25 patients, 16 (64%) had no ocular symptoms, and 9 (36%) had ocular complaints. Findings included mild vitreous inflammation (45 eyes [75%]), white retinal lesions (18 eyes [30%]), focal vascular sheathing (5 eyes [8.3%]), multiple arterial plaques (1 eye [1.7%]), intraretinal hemorrhages (14 eyes [23.3%]), white-centered retinal hemorrhages (2 eyes [3.3%]), subretinal hemorrhages (2 eyes [3.3%]), serous retinal detachment (3 eyes [5%]), macular star (2 eyes [3.3%]), cystoid macular edema (1 eye [1.7%]), optic disc edema (1 eye [1.7%]), branch retinal artery occlusion (1 eye [1.7%]), optic disc staining (30 eyes [50%]), retinal vascular leakage (27 eyes [45%]), delayed filling in a branch retinal vein (1 eye [1.7%]), and multiple hypofluorescent choroidal dots (10 eyes [16.7%]). One eye (1.7%) had retinal neovascularization at the 6-month follow-up examination. All posterior segment findings at the acute stage resolved in 3 to 10 weeks, and the final visual acuity was 20/20 in 42 of 45 affected eyes (93.3%). Retinal pigment epithelium changes developed in 9 eyes (15%), with resolved full-thickness white retinal lesions. No other abnormalities were noted in the eye with retinal neovascularization over a further follow-up of 6 months. CONCLUSION: Posterior segment involvement, frequently asymptomatic, is common in patients with acute MSF. Because the diagnosis can be easily overlooked, a careful dilated funduscopic examination, complemented by fluorescein angiography in selected cases, is recommended. Mild vitritis, retinal vasculitis, optic disc staining, white retinal lesions, retinal hemorrhages, and multiple hypofluorescent choroidal dots are the most common manifestations of MSF. Posterior segment changes in a patient with fever and/or skin rash living in or returning from a specific endemic area, especially during the spring or summer, strongly suggest R. conorii infection.