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      Long-Term Follow-Up of Dominant Macular Dystrophy with Flecks (Stargardt)

      Ophthalmologica

      S. Karger AG

      Stargardt’s disease, Macular dystrophy

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          Abstract

          We present a family with dominant macular dystrophy and flecks (Stargardt) which was followed for 20 years. Twenty-three subjects out of 48 members in 4 generations underwent fundoscopic examination. Nine asymptomatic patients had a few scattered, small, whitish, drusen-like changes in the posterior pole, and an addditional patient had several clumps of increased pigmentation at the level of the retinal pigment epithelium in the macular region. Five patients had macular degeneration, with the onset of visual loss at between 24 and 30 years of age. The maculopathy started as fine, punctate, pigmentary changes, with transmission retinal pigment epithelial defects (as seen angiographically), or as flecks. The maculopathy progressed into a garland of perifoveal, subretinal flecks with small, central, areolar, chorioretinal atrophy. At the end-stage, there was a larger, central area of choroidal atrophy and a wider wreath of subretinal flecks. Visual acuity stabilized at the 20/200 level. Visual loss preceded clinically visible choroidal atrophy and was coincident with the accumulation of flecks in the foveal region.

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          Author and article information

          Journal
          OPH
          Ophthalmologica
          10.1159/issn.0030-3755
          Ophthalmologica
          S. Karger AG
          0030-3755
          1423-0267
          1992
          1992
          01 April 2010
          : 205
          : 3
          : 138-143
          Affiliations
          Department of Ophthalmology, University of Texas Medical Branch, Galveston, Tex., USA
          Article
          310329 Ophthalmologica 1992;205:138–143
          10.1159/000310329
          1475092
          © 1992 S. Karger AG, Basel

          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.

          Page count
          Pages: 6
          Categories
          Original Paper

          Vision sciences, Ophthalmology & Optometry, Pathology

          Stargardt’s disease, Macular dystrophy

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