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      Primitive Retinal Vascular Abnormalities: Tumors and Telangiectasias

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          Abstract

          Primitive retinal vascular abnormalities are benign conditions of the retinal circulation that comprise vascular tumors and telangiectasias. The principal vascular tumors of the retina include retinal capillary hemangioma, cavernous hemangioma of the retina, racemose hemangiomatosis of the retina and retinal vasoproliferative tumor, while primary retinal telangiectasias include Coats’ disease, Leber’s miliary aneurysms and idiopathic juxtafoveal telangiectasias. In most cases, these alterations result in significant visual impairment due to exudation determined by the structural abnormalities of the retinal vasculature. The aim of this review is to assess the different clinical and diagnostic features of the single pathological entities and to discuss the available treatment modalities including the onset of intravitreal antivascular endothelial growth factor therapy.

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          Clinical features and natural history of von Hippel-Lindau disease.

          J Stoker, R. Harries, Molly Maher … (1990)
          The clinical features, age at onset and survival of 152 patients with von Hippel-Lindau disease were studied. Mean age at onset was 26.3 years and 97 per cent of patients had presented by aged 60 years. Retinal angioma was the first manifestation in 65 patients (43 per cent), followed by cerebellar haemangioblastoma (n = 60, 39 per cent) and renal cell carcinoma (n = 15, 10 per cent). Overall, 89 patients (59 per cent) developed a cerebellar haemangioblastoma, 89 (59 per cent) a retinal angioma, 43 (28 per cent) renal cell carcinoma, 20 (13 per cent) spinal haemangioblastoma and 11 (7 per cent) a phaeochromocytoma. Renal, pancreatic and epididymal cysts were frequent findings but their exact incidence was not accurately assessed. Mean age at diagnosis of renal cell carcinoma (44.0 +/- 10.9 years) was significantly older than that for cerebellar haemangioblastoma (29.0 +/- 10.0 years) and retinal angioma (25.4 +/- 12.7 years). The probability of a patient with von Hippel-Lindan disease developing a cerebellar haemangioblastoma, retinal angioma or renal cell carcinoma by age 60 years was 0.84, 0.7 and 0.69, respectively. A comprehensive screening protocol for affected patients and at-risk relatives is presented, based on detailed analysis of age at onset data for each of the major complications. Median actuarial survival was 49 years, with renal cell carcinoma the leading cause of death.
          Article 0 comments Cited 140 times – based on 0 reviews     Review now
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            Corticosteroids inhibit VEGF-induced vascular leakage in a rabbit model of blood-retinal and blood-aqueous barrier breakdown.

            Jeffrey Edelman, David W. Lutz, Marisol Castro (2005)
            Recent clinical studies show that a single intravitreal injection of the corticosteroid triamcinolone acetonide (TAA) may reduce edematous retinal swelling and improve visual acuity in patients with diabetic macular edema (DME). In addition, clinical and experimental studies strongly suggest that blood-retinal barrier breakdown in diabetes is induced by vascular endothelial growth factor (VEGF). These results suggest that corticosteroids may modulate VEGF-mediated responses in vivo. To test this hypothesis directly, the current study evaluated the effects of TAA and dexamethasone (DEX) in a newly developed rabbit model of VEGF-induced blood-retinal barrier and blood-aqueous (iris) barrier breakdown. VEGF165 or vehicle was injected intravitreally in female Dutch Belt rabbits, and scanning ocular fluorophotometry was used to non-invasively measure fluorescein leakage from retinal and iris vasculature. VEGF-induced retinal vasculopathy was further assessed with fundus imaging, fluorescein angiography, and ocular coherence tomography. For pharmacologic studies, rabbits were treated with either DEX (2 mg kg(-1) daily, s.c.), TAA (2 or 4 mg, intravitreal), indomethacin (20 mg kg(-1) daily, s.c.), or vehicle (s.c. or intravitreal). Human umbilical vein endothelial cells (HUVEC) were loaded with the fluorescent Ca2+ indicator dye fluo-4 and treated with dexamethasone (0.1-10 microM) or vehicle for either 2 or 24 hr prior to stimulation with 10 ng ml(-1) VEGF165. VEGF injected intravitreally induced a time and dose-dependent breakdown of the blood-retinal and blood-aqueous barriers. Maximal vascular leakage was measured at 48 hr after intravitreal injection with a dose of 500 ng VEGF. Other effects of VEGF included prominent retinal vasodilation, vessel tortuousity, fluorescein leakage from retinal vessels, and inner retinal edema. These VEGF-mediated responses are transient and approach baseline by 1 week. VEGF-induced blood-retinal and blood-aqueous barrier breakdown was completely blocked by the corticosteroid DEX administered systemically for 3 days. In contrast, the non-steroidal anti-inflammatory drug, indomethacin, had no effect. In a separate study with VEGF injected intravitreally at six different time points over 5 months, a single intravitreal 2 mg dose of TAA completely blocked VEGF-induced retinal and iris leakage for 45 days. VEGF/VEGF receptor-2-mediated Ca2+ mobilization in endothelial cells was not affected by 2 or 24 hr pretreatment with dexamethasone. These results indicate that one mechanism by which corticosteroids block blood-ocular barrier breakdown and edema is via their modulation of signaling or effector proteins downstream of the VEGF receptor.
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              Treatment with intravitreal steroid reduces blood-retinal barrier breakdown due to retinal photocoagulation.

              B. Galán, N Ando, B Berkowitz … (1992)
              The effect of corticosteroid treatment on blood-retinal barrier breakdown caused by argon-laser panretinal photocoagulation was evaluated in the rabbit eye. One day before photocoagulation, eyes were given either a sub-Tenon (20-mg) or intravitreal (2-mg) injection of triamcinolone acetonide. The severity of blood-retinal barrier breakdown was measured after photocoagulation using rapid sequential magnetic resonance imaging following intravenous administration of gadolinium diethylenetriaminepentaacetic acid. Leakage of gadolinium diethylenetriaminepentaacetic acid into the vitreous space was significantly lower in eyes that received intravitreal triamcinolone acetonide than in control eyes (P = .007); however, sub-Tenon triamcinolone acetonide produced no significant reduction in leakage (P = .65) compared with controls. Fluorescein angiography supported the magnetic resonance imaging findings. We conclude that retinal photocoagulation in the rabbit eye produces blood-retinal barrier breakdown that is partially amenable to corticosteroid treatment.
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                Author and article information

                Journal
                Journal ID (publisher-id): OPH
                Journal ID (nlm-ta): Ophthalmologica
                Journal ID (doi): 10.1159/issn.0030-3755
                Title: Ophthalmologica
                Publisher: S. Karger AG
                ISSN (Print): 0030-3755
                ISSN (Electronic): 1423-0267
                Publication date Subscription-year: 2012
                Publication date (Print): July 2012
                Publication date (Electronic): 23 June 2012
                Volume: 228
                Issue: 2
                Pages: 67-77
                Affiliations
                Department of Ophthalmology, San Raffaele Scientific Institute, Milan, Italy
                Author notes
                *Rosangela Lattanzio, San Raffaele Scientific Institute, Via Olgettina 60, IT–20132 Milan (Italy), Tel. +39 02 2643 2648, E-Mail lattanzio.rosangela@hsr.it
                Article
                Publisher ID: 338230 Other ID: Ophthalmologica 2012;228:67–77
                DOI: 10.1159/000338230
                PubMed ID: 22738997
                SO-VID: c2824563-488c-43c3-a70f-7c68fa2a317e
                Copyright © © 2012 S. Karger AG, Basel
                License:

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Date received : 17 January 2012
                Date accepted : 06 March 2012
                Page count
                Figures: 6, Tables: 2, Pages: 11
                Categories
                Subject: EURETINA – Review

                ScienceOpen disciplines: Vision sciences,Ophthalmology & Optometry,Pathology
                Keywords: Retinal vascular tumors,Retinal angiomatosis,Retinal telangiectasias,Von Hippel-Lindau syndrome
                Data availability:
                ScienceOpen disciplines: Vision sciences, Ophthalmology & Optometry, Pathology
                Keywords: Retinal vascular tumors, Retinal angiomatosis, Retinal telangiectasias, Von Hippel-Lindau syndrome

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