Hemorrhagic occlusive retinal vasculitis leading to the diagnosis of ciliary body melanoma

Hemorrhagic occlusive retinal vasculitis (HORV) is a condition associated with intraocular vancomycin during surgical intervention, most frequently following bilateral sequential cataract surgery. Because of the high rate of ophthalmic vascular complications in this condition, gonioscopic evaluation for identification of neovascularization of the angle and iris is essential. Careful examination can reveal previously asymptomatic and unassociated lesions of the iris or ciliary body. We present the case of a 71-year-old female who was diagnosed with a ciliary body melanoma secondary to complete ophthalmic examination associated with HORV. She reported decreased vision to light perception in the left eye (OS) following sequential, bilateral cataract surgery. Fundus examination OS demonstrated diffuse retinal vasculitis, retinal ischemia, and extensive hemorrhage. Evaluation included inflammatory and coagulopathy laboratory evaluation, carotid ultrasonography and magnetic resonance imaging (MRI) of the brain and orbits, all of which proved unrevealing, except for an enhancing mass OS on MRI. Further evaluation revealed a pigmented mass with features of melanoma in the anterior chamber angle extending into the ciliary body. Fine needle aspiration biopsy revealed high risk cytogenetic characteristics, and plaque radiotherapy was successfully employed.     

PCR-positive tubercular retinal vasculitis: clinical characteristics and management

BACKGROUND: Inflammation of retinal vessels is a known association of systemic tuberculosis. Patients with retinal vasculitis are subjected to extensive but unrewarding systemic workup. Polymerase chain reaction (PCR) is now commonly used to detect DNA of infective organisms including Mycobacterium tuberculosis. This study was undertaken to characterize the clinical characteristics of PCR-positive tubercular retinal vasculitis, so as to determine the clinical presentation, associated systemic features, management, and course of this form of vasculitis. METHODS: The clinical records of 13 patients seen between 1997 and 1999 with the diagnosis of PCR-positive tubercular retinal vasculitis from the aqueous or vitreous humor were reviewed. Recorded data included age, sex, race, visual acuity, anterior and posterior segment findings, and results of diagnostic evaluations. All received antituberculosis therapy with or without concomitant corticosteroids. Laser scatter photocoagulation was done in eyes with neovascularization. One eye with vitreous hemorrhage was subjected to pars plana vitrectomy. RESULTS: There were 9 (69.2%) male and 4 (30.7%) female patients with a median age of 20 years. The disease was bilateral in seven. The most consistent finding was the presence of vitritis in all the eyes followed by vitreous snowball opacities in 17 eyes (89.4%), neovascularization in 11 eyes (57.8%), retinal hemorrhages in 10 eyes (52.6%), neuroretinitis in 10 eyes (52.6%), focal choroiditis in 9 eyes (47.3%), vitreous/preretinal hemorrhage in 5 eyes (26.3%), and serous retinal detachment in 3 eyes (15.7%). Over a median follow-up of 12 months, all showed resolution of vasculitis with no recurrences. CONCLUSIONS: Polymerase chain reaction-positive tubercular retinal vasculitis had varied associated fundus findings. Its recognition is important so as to order only relevant diagnostic tests.     

Primary retinal vasculitis

CASE REPORT: We present the case of a 22-year-old Caucasian female who consulted because of a brief episode of blurred vision in her right eye. Funduscopic findings in right eye included marked venous tortuousness and roundish paramacular haemorrhages. A slight venous tortuousness in left eye. Fluorescein angiography showed a segmentary retinal vasculitis affecting medium calibre vessels of the posterior pole. Complementary examinations ruled out any systemic involvement. After therapy with oral corticoesteroids the case evolves to remission. DISCUSSION: The diagnosis of retinal vasculitis is achieved by the performance of ophthalmoscopy and fluorescein angiography. Prior studies have ruled out the need for complementary examinations to find any systemic involvement in patients.     

特发性视网膜血管炎综合治疗的疗效分析

目的分析特发性视网膜血管炎的治疗效果。方法对126例（197眼）确诊为特发性视网膜血管炎的患者进行药物、激光或玻璃体手术治疗进行回顾性分析,包括1年内的视力、光学相干断层扫描（OCT）检查、荧光素眼底血管造影（FFA）结果。结果早期药物治疗110眼,其中视力改善者61眼（55．5％）,需激光或手术治疗者36眼（32．7％）。激光治疗累计71例（119眼）,视力改善者88跟（73．9％）。20眼接受玻璃体手术,视力改善者9眼（45％）。接受激光与玻璃体手术治疗的疗效比较差异有统计学意义（x^2=6．80,P〈0．05）。非玻璃体出血与玻璃体出血者激光治疗后视力改善眼数的差异有统计学意义（x^2=6．53,P〈0．05）。对视力改善者,OCT和FFA均提示黄斑水肿明显改善。结论视网膜激光光凝对特发性视网膜血管炎是重要的治疗手段,不同治疗方法适应证的选择和治疗期间的密切随访对于改善治疗效果至关重要。     

视网膜血管炎的临床分析

目的探讨视网膜血管炎的患病因素及治疗效果。方法回顾性分析视网膜血管炎患者14例（16只眼）的临床表现、检查、诊治和随访结果。结果所有患者眼后段均有不同程度的炎性改变,经过药物治疗、激光和手术治疗后,最终视力提高共10只眼（62．5％）。结论本病眼底表现复杂,以血管白鞘、视网膜出血和黄斑水肿最为多见,病因尚不明确,诊断以临床表现为主,早期、足量的抗病毒、皮质类固醇激素的应用,以及预防性视网膜光凝、必要联合玻璃体手术是控制病变的关键。     

Interferon and retinal vasculitis

The treatment of noninfectious posterior uveitis can lead to severe vision loss, and the first-line conventional treatment includes systemic steroids. When the prednisone doses necessary to control intraocular inflammation are above 0.3mg/day, a therapeutic association is proposed in order to lower the daily prednisone dose. The combined drugs are immunosuppressive or immunomodulative. The side effects of immunosuppressive drugs are oncogenic, infectious, and hematological, and can involve reproductive troubles, associated with specific toxic effects depending on the drug used. Recently adding polyclonal or monoclonal antibodies and the interferons to immunomodulative drugs has been suggested. Interferon alpha has been shown to be effective in Beh?et's disease. The efficacy of interferon needs to be evaluated in other etiologies of retinal vasculitis through randomized studies.     

视网膜血管炎的研究进展

视网膜血管炎主要累及青壮年视网膜静脉,动脉累及较少,致盲率较高.积极防治此病意义重大.其发生可能与感染、免疫机制、氧化反应、全身性疾病等有关,通过荧光素眼底血管造影、超声、实验室检查可提高诊断正确率.针对不同的病程,采用药物、激光、玻璃体视网膜手术等治疗措施可改善视功能.就此病的定义、病因、分类、基础研究、诊断及治疗等方面的最新进展进行综述.     

Antibody affinity to retinal S-antigen in patients with retinal vasculitis.

Using a modified enzyme-linked immunosorbent antibody method that included dissociation of antigen antibody complexes with sodium thiocyanate, we examined the functional affinity of antibody to retinal S-antigen in 48 patients with retinal vasculitis and 46 age-matched healthy control subjects. Antibody affinity was markedly lower in patients with retinal vasculitis than in healthy subjects. Low-affinity antibody was more prevalent in acute retinal vasculitis and in patients with normal levels of circulating immune complexes. We found distinct differences between the antiretinal antibodies found in patients with retinal vasculitis and those in control subjects. The association of low-affinity antibody with normal levels of circulating immune complexes may suggest defective regulation of antiretinal autoimmunity and have important pathogenic implications.     

A new approach to the problem of retinal neovascularization

Tumor cells introduced into the rabbit vitreous produced a form of retinal neovascularization, but only when the tumor cells were in direct contact with the vascularized retina. This contrasted with the production of neovascularization at a distance of several millimeters in other angiogenesis models. An extract of adult rabbit vitreous was found to inhibit the growth of limbal neovascularization induced by tumors implanted in the rabbit corneal stroma. The experimental model of retrolental fibroplasia provides a bioassay to investigate further the apparent inhibitory substances in the vitreous.     

激光扫描眼底荧光血管造影在特发性视网膜血管炎诊断中的应用

病变检查范围扩大到远周边;二者结合,有助于全面、细致观察IRV病变的程度和范围,降低病变漏诊率,对IRV的诊断和治疗提供更多帮助.[眼科新进展 2009;29(9):688-690,693]     

A longitudinal study of clinical and immunological findings in 52 patients with relapsing retinal vasculitis.

Fifty-two patients with retinal vasculitis--26 with idiopathic disease and 26 with associated systemic inflammatory disease--were followed up for periods ranging from six months to 12 years. The aim of the study was to determine the relationship between relapse of uveitis, visual outcome, and the occurrence of circulating immune complexes (CIC) and antiretinal antibodies. In a total of 69 relapses, CIC were increased in one-third of patients and antiretinal antibodies in one-half. In those 34 patients who expressed antiretinal antibodies 27 (79%) of the relapses were characterised by antiretinal antibodies in the absence of raised CIC levels (p less than 0.01). These findings support our previous hypothesis that CIC may have a protective role in autoimmune retinal vasculitis and that antiretinal autoimmunity is of pathogenetic importance in relapse. In individual patients the visual outcome was not related to the number of relapses or to the CIC-autoantibody pattern, suggesting the operation of additional features which merit identification.     

ANCA associated pauci-immune retinal vasculitis

BACKGROUND: Antinuclear cytoplasmic antibodies (ANCA) are useful diagnostic serological markers for the most common forms of necrotising vasculitis. ANCA associated vasculitides represent distinctive clinicopathological categories--for example, Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and idiopathic necrotising crescentic glomerulonephritis, collectively known as the small vessel pauci-immune vasculitides. METHOD: Three cases of ANCA associated pauci-immune retinal vasculitis are described. Their systemic features are described and the clinical significance of ANCA as a diagnostic test in relation to retinal vasculitis discussed. RESULTS: These three cases represent a spectrum of clinical features associated with retinal vasculitis. Two cases have evolved into clinical recognisable entities as microscopic polyangiitis. Adherence to the international consensus statement on testing and reporting of ANCA is recommended and the authors speculate that the incidence of microscopic polyangiitis may be underestimated because of the under-recognition of systemic involvement in patients with retinal vasculitis. CONCLUSION: The receipt of a positive ANCA result should always raise the suspicion of a pauci-immune systemic vasculitis and prompt appropriate investigation. The authors emphasise the importance of the evaluation of systemic features in these patients with retinal vasculitis, enabling earlier recognition and thereby preventing significant morbidity and mortality.     

Acute multifocal hemorrhagic retinal vasculitis

The authors present a series of seven patients with acute visual loss associated with mild anterior uveitis, multifocal retinal vasculitis, retinal capillary nonperfusion, retinal hemorrhage, disc swelling, and vitreitis. Oral prednisone was of some benefit in these patients and oral acyclovir was generally ineffective. Neovascular complications including retinal, disc, choroidal, and iris new vessels were common, requiring photocoagulation in five patients. Horseshoe tears developed in two patients in zones of uninvolved retina but retinal detachment did not occur. The etiology remains unknown, although it may represent either a localized ocular form of Beh?et's disease or other systemic syndrome, infection with a herpes group virus other than zoster varicella virus, or a manifestation of an undefined infectious agent.     

Clinical picture of isolated retinal vasculitis

Clinical picture of isolated retinal vasculitis (IRV) in 43 patients (25 men and 18 women) aged 22-42 years is analyzed. IRV was bilateral in 88.4% cases and involved the veins. Two forms of disease were distinguished: peripheral (in 75%) and central-peripheral (25%). Both forms were characterized by a high hemorrhagic activity, occlusion of vessels, ischemic zones and neovascularization of the retina and optic disk, but these signs were more frequent in central peripheral form of IRV. IRV is a grave disease leading to poor vision or blindness in 18% cases. The onset is poorly manifest with floating spots and blurred vision; the main symptom is vitreous hemorrhage. Visual acuity was significantly decreased in patients with occlusive IRV in comparison with those without occlusions (0.32 +/- 0.37 vs. 0.77 +/- 0.28). The earliest changes can be detected mainly in the peripheral parts of the retina. This necessitates special attention of ophthalmologist to seemingly negligible complaints of patients and thorough examination of the fundus oculi, particularly of its peripheral sections, which can be performed only on condition of adequate mydriasis. Only timely diagnosis and early pathogenetic therapy can decrease the probability of such complications as hemophthalmos, retinal neovascularization, detachment of retina, and neovascular glaucoma, and prevent disability.