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      Stenosis of the Lachrymal System in Rubinstein-Taybi Syndrome

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          Abstract

          In this article the authors describe a case of atypical stenosis of the lachrymal excretory system in Rubinstein-Taybi syndrome (RTS) characterised by morphologic anomalies of the lachrymal bag and by alterations of the osseous structure of the nose. A 9-year-old girl, with typical findings of RTS, was affected by recurrent acute dacryocystitis and tearing. Ocular examination revealed bilateral reflux with mucous and purulent material flowing back after digital pressing: low and bilateral nose-lachrymal duct obstruction was the main cause of the reflux as confirmed by orifice probing, lachrymal drainage system irrigation and spiral CT examination using hydrosoluble contrast medium. The multiplanar reconstruction obtained from CT scanning shows that the right lachrymal sac has an abnormal shape (‘grape-bunch’ image) and that the left one has various bulgings, or swellings, in its shape. The ‘grape-bunch’ lachrymal bags are the most unusual features of our patient’s lachrymal system and it is important to consider the difficulties that could occur during surgery because of the abnormal bag shape and because of the increased bone thickness in RTS patients. The ‘grape-bunch’ lachrymal sac is a truly unusual anatomical feature and, most probably, it could be distinctive of this syndrome.

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          Author and article information

          Journal
          OPH
          Ophthalmologica
          10.1159/issn.0030-3755
          Ophthalmologica
          S. Karger AG
          0030-3755
          1423-0267
          2002
          August 2002
          29 August 2002
          : 216
          : 4
          : 272-276
          Affiliations
          aH.M.D. ‘M.D. Barbantini’, Ophthalmic Division, Lucca, bDepartment of Oncology, Transplants and Advanced Technologies in Medicine, Institute of Diagnostic and Interventional Radiology, University of Pisa, Italy
          Article
          63837 Ophthalmologica 2002;216:272–276
          10.1159/000063837
          12207132
          © 2002 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
          Figures: 5, Tables: 1, References: 6, Pages: 5
          Categories
          Case Report · Description de cas · Fallbericht

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