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Peripheral retinal neovascularization (Eales disease) associated with the factor V Leiden mutation.

PURPOSE: To illustrate a case of peripheral retinal neovascularization (Eales disease) in a patient who tested positive for the factor V Leiden mutation. METHODS: A 42-year-old woman had a 1-week history of blurred vision in her right eye. Her medical history was remarkable for a cerebrovascular accident. Ophthalmoscopy of the right eye disclosed a mild vitreous hemorrhage and a ridge of retinal neovascularization in the temporal periphery. The left fundus showed evidence of temporal retinal ischemia. A laboratory evaluation for hypercoagulability was positive for factor V Leiden mutation. RESULTS: Peripheral scatter laser photocoagulation was applied to the ischemic retina, and the neovascularization regressed. The patient began taking warfarin sodium to prevent further thrombotic events. CONCLUSION: A laboratory evaluation for coagulopathy, including the factor V Leiden mutation, should be added to the examination of patients with Eales disease, especially individuals with a history of a previous thrombotic event.

Adult↗

Recurrent unilateral frosted branch angiitis.

In the left eye of a 27-year-old man we found perivascular creamy sheathing of retinal veins with retinal hemorrhages and, on fluorescein angiography, delayed filling of veins with late leakage. Dramatic recovery of visual acuity and healing of retinal lesions followed intravenous corticosteroid therapy. However, the condition recurred several times within a few months. Fluorescein angiography showed delayed filling of arteries and veins and arteriovenous anastomoses with a widespread capillary nonperfusion area. Eventually, neovascular glaucoma resulted. It is suggested that frosted branch angiitis is related to vascular occlusion. Systemic corticosteroid therapy seems to affect the course of this disease.

Adult↗

A case of frosted branch angiitis with yellowish-white placoid lesions: fluorescein and indocyanine green angiography findings.

Severe, white sheathing of the retinal vessels and yellowish-white placoid lesions, suggesting frosted-branch angiitis, were found in both fundi of a 24-year-old woman. Fluorescein angiography showed hypofluorescence of the placoid lesions in the early phase and hyperfluorescence in the late phase. Indocyanine green angiography showed hypofluorescence during all phases. These findings imply an occlusion of the choriocapillaris. Fluorescein angiography also showed occlusion of the peripheral retinal vessels. With systemic corticosteroids and prostaglandin I2 derivative, the fundi recovered almost completely to normal 3 months later.

Adult↗

Eales disease--an update.

Eales disease, first described by Henry Eales in 1880, remains an enigma. The disease, observed more commonly in the Indian subcontinent than in the rest of the world, occurs in young healthy adult males, initially presenting as retinal periphlebitis and later as retinal ischemia that may lead to vascular alterations and neovascularization. Recurrent vitreous hemorrhage with or without retinal detachment is the common sequelae. In recent years, immunological, molecular biological, and biochemical studies have indicated the role of human leukocyte antigen, retinal autoimmunity, mycobacterium tuberculosis genome, and free radical mediated damage in the etiopathogenesis of this disease. However, its etiology appears to be multifactorial. The management depends on the stage of the disease and consists of medical treatment with oral corticosteroids in the active inflammatory stage and laser photocoagulation in the advanced retinal ischemia and neovascularization stages. The results of vitreoretinal surgery have been found to be satisfactory in case of vitreous hemorrhage with or without retinal detachment.

Diagnosis, Differential↗

Severe retinal vaso-occlusive disease secondary to procainamide-induced lupus.

Systemic lupus erythematosus (SLE) is known to cause various forms of ocular problems, including severe retinal vaso-occlusive disease. Procainamide is one of many drugs that may cause a lupus-like syndrome which resembles SLE but can be distinguished through clinical features and laboratory studies. Presented is a patient with severe vaso-occlusive retinopathy on high-dose procainamide therapy. Associated clinical, laboratory, and pathologic findings suggest the diagnosis of drug-induced lupus and exclude other vasculitic or inflammatory etiologies. This represents the first documented case of retinal disease attributed to procainamide-induced lupus.

Aged↗

A case report of frosted branch angiitis and its visual electrophysiology.

We reported a 5-years old boy with frosted branch angiitis in both eyes. The visual acuities of the two eyes were suddenly lost to 5/50. The fluorescein angiography, electroretinograms (ERGs) and pattern evoked potentials (PVEPs) had been tested at the acute and the recovery stage of the disease. At the onset of the disease, fluorescein angiography showed obvious dye leakage from dilated retinal veins. The PVEPs and whole field ERGs including rod, maximum, OPs and cone responses were seriously decreased and prolonged, indicating that the visual pathway's and retinal functions were greatly damaged. The patient had an excellent response to systemic prednisone. One month after the treatment, the visual acuities recovered to 60/50 in both eyes. The fluorescein angiography showed that leakage from blood vessels was diminished, but the lesions at macula and optic disc were persisted a longer time even to the end of treatment. Through 6 months medication, nearly all of the electrophysiological abnormalities had been resolved.

Administration, Oral↗

Indocyanine green angiography in a case of unilateral recurrent posterior acute multifocal placoid pigment epitheliopathy.

PURPOSE: To report a case of unilateral recurrent acute posterior multifocal placoid pigment epitheliopathy (APMPPE) imaged by indocyanine green (ICG) angiography. METHODS: A complete ophthalmological evaluation, including fluorescein and ICG angiography, was performed. RESULTS: Acute posterior multifocal placoid pigment epitheliopathy recurred 3 months after the initial disease in a 23-year-old male patient. On ICG angiography, both new, active and healed, inactive lesions demonstrated hypofluorescence throughout the study. The healed lesions were smaller in size and irregularly shaped, whereas the new, active lesions were larger in size, round and more confluent. CONCLUSION: New, active and healed, inactive lesions in APMPPE can both be imaged and differentiated by ICG angiography.

Acute Disease↗

Vasculitides associated with infections, immunization, and antimicrobial drugs.

Many pathogenic microbes can induce a complex series of immunologic, endothelial-cell, and hemorheological activation cascades and dysfunctions and can cause interactions of various components of the vessel wall with formed and noncellular elements of the blood. These interactions can result in manifold vasculitides. This article reviews the pathogenetic, clinicopathologic, and therapeutic aspects of infection-related vasculitides; it includes a brief discussion of parasitic, drug-related, and immunization-associated vasculitides as well as of the infection-related features of vasculitides of the central nervous system, retinal vasculitides, ecthyma gangrenosum, and erythema nodosum. Viruses are mainly associated with small-vessel vasculitides, whereas bacterial infections affect vessels of all sizes, including the aorta. The vasculitides associated with fungal infection usually are characterized by erythema nodosum and involve large vessels. The search for microbes as causes of or contributors to vasculitides should continue. Infectious vasculitides may become increasingly important as the number of immunocompromised patients grows.

Anti-Bacterial Agents↗

Acute retinal necrosis syndrome treated with vitrectomy and intravenous acyclovir.

Acute retinal necrosis syndrome in a healthy 32-year-old man was treated successfully with systemic acyclovir and corticosteroids in combination with vitrectomy, 360 degrees encircling band and transscleral cryocoagulation. Herpes simplex virus antibodies were detected in the vitrectomy aspirate supporting the current view that acute retinal necrosis is caused by viruses of the herpes group.

Acute Disease↗

Campath-1H therapy in refractory ocular inflammatory disease.

BACKGROUND: Standard therapy for severe, immune mediated, ocular inflammation has significant side effects, and may fail to control the disease. T cell directed monoclonal antibody (mAb) therapy can provide long term remission of inflammatory disease in experimental models. The Campath-1H mAb was administered to patients with severe, refractory, ocular inflammation. METHODS: 10 patients with severe, refractory, non-infectious ocular inflammatory disease were treated with Campath-1H mAb. This is a fully humanised mAb which recognises the pan-lymphocyte antigen CD52. RESULTS AND DISCUSSION: Following Campath-1H therapy, all 10 patients showed an initial resolution of their ocular symptoms and signs. Long lasting remissions were achieved in eight patients, in whom baseline immunosuppression could subsequently be reduced to minimal levels. The possible mechanisms of action of Campath-1H therapy are discussed.

Adult↗

Behçet's disease: from Hippocrates to the third millennium.

Behçet's disease (BD) is characterised by recurrent episodes of orogenital aphthae, systemic vasculitis, and systemic and retinal venous thrombosis. An association between HLA-B51 and BD was first identified over 20 years ago, but recently identified gene associations implicate regions both within and without the MHC in the immunological events underlying the lesions in BD. These include allelic variants within the tumour necrosis factor gene region and within the MHC class I chain related gene region, the factor V Leiden mutation, which is associated with retinal vascular occlusion, and alleles of the intercellular adhesion molecule gene. No single causative gene for BD has emerged; the evidence indicates that the underlying immune events in BD are triggered by a microbial antigen and subsequently driven by genetic influences which control leucocyte behaviour and the coagulation pathways. Knowledge of these risk factors may permit a more accurate prognosis for a given patient, and identify new pathways for more targeted intervention than is currently available.

Behcet Syndrome↗

Antiphlogistic effect of catalase on experimental phacoanaphylactic endophthalmitis.

Oxygen-derived free radicals generated by polymorphonuclear leukocytes have recently been implicated as important pathogenic mediators of tissue damage in immune complex-mediated acute inflammatory reactions. In order to study the role of oxygen radicals in experimentally induced phacoanaphylactic endophthalmitis, rats were immunized with soluble bovine lens protein and, following surgical disruption of the anterior lens capsule, subjected to treatment with catalase. Our data show that phacoanaphylactic endophthalmitis, as determined morphologically and by measurements of retinal edema, choroidal inflammation, retinal hemorrhage and necrotizing vasculitis, is greatly diminished in catalase-treated animals. This protective effect of catalase indicates that hydrogen peroxide (H2O2) and/or conversion products of H2O2 (e.g., hydroxyl radical, hypochlorous acid) may play an important role in the development of experimental phacoanaphylactic endophthalmitis.

Animals↗

Treatment of experimental lens-induced uveitis by dimethyl thiourea.

Dimethyl thiourea is a highly specific hydroxyl radical scavenger. The antiinflammatory potential of this hydroxyl radical scavenger was studied in Lewis rats sensitized with soluble lens protein. Intraperitoneal injection of dimethyl thiourea into the experimental animals at the onset of the disease resulted in marked reduction of intraocular inflammation, hemorrhage and edema, and eliminated the retinal and choroidal necrotizing vasculitis that was characteristic in untreated animals. These findings indicate that dimethyl thiourea may act as a potent antiphlogistic agent in the treatment of experimental lens-induced uveitis.

Animals↗