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Table of Contents
Vol. 24, No. 3, 2008
Issue release date: October 2008
Section title: Paper
Fetal Diagn Ther 2008;24:225–229
(DOI:10.1159/000151343)

Survival in Primary Congenital Pulmonary Lymphangiectasia with Hydrops Fetalis

Laje P. · Wilson R.D. · Guttenberg M. · Liechty K.W.
Department of General Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pa., USA

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Article / Publication Details

First-Page Preview
Abstract of Paper

Received: 4/25/2007
Accepted: 6/30/2007
Published online: 8/28/2008

Number of Print Pages: 5
Number of Figures: 4
Number of Tables: 0

ISSN: 1015-3837 (Print)
eISSN: 1421-9964 (Online)

For additional information: http://www.karger.com/FDT

Abstract

The recent advances in neonatal and pediatric intensive care have changed the outcome of patients with congenital pulmonary lymphangiectasia of different types, including those cases with early neonatal symptoms. However, the patients who present the most severe form of the disease, manifested by in utero hydrops (including those treated by in utero thoracoamniotic shunting to relieve the mediastinal compression), have had an unvaryingly fatal ending in all published reports, with most cases dying before birth, and the few born alive dying during the first days of life. We present a patient with primary congenital pulmonary lymphangiectasia complicated by hydrops fetalis, who was treated in utero, survived the neonatal period after intensive medical and surgical support, and was discharged home at the age of 2 months. She is currently 6 months old, and has minimal signs or symptoms of chronic lung disease. The different aspects of the management of congenital pulmonary lymphangiectasia are discussed in this report, together with a review of the literature.


Article / Publication Details

First-Page Preview
Abstract of Paper

Received: 4/25/2007
Accepted: 6/30/2007
Published online: 8/28/2008

Number of Print Pages: 5
Number of Figures: 4
Number of Tables: 0

ISSN: 1015-3837 (Print)
eISSN: 1421-9964 (Online)

For additional information: http://www.karger.com/FDT


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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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.

References

  1. Noonan JA, Walters LR, Reeves JT: Congenital pulmonary lymphangiectasias. Am J Dis Child 1970;120:314–319.
  2. Esther CR Jr, Barker PM: Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol 2004;38:308–313.
  3. Bellini C, Boccardo F, Campisi C, et al: Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis 2006;1:43.
  4. Wilson RD, Pawel B, Bebbington M, et al: Congenital pulmonary lymphangiectasias sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion. Prenat Diagn 2006;26:1058–1061.
  5. Fukunaga M: Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumors. Histopathology 2005;46:396–402.
  6. Kaiserling E: Immunohistochemical identification of lymph vessels with D2–40 in diagnostic pathology. Pathologe 2004;25:362–374.
  7. Siu SL, Lam DS: Spontaneous neonatal chylothorax treated with octreotide. J Pediatr Child Health 2006;42:65–67.

    External Resources

  8. Barker PM, Esther CR Jr, Fordham LA, et al: Primary pulmonary lymphangiectasia in infancy and childhood. Eur Respir J 2004;24:413–419.
  9. Scott C, Wallis C, Dinwiddie R, et al: Primary pulmonary lymphangiectasias in a premature infant: resolution following intensive care. Pediatr Pulmonol 2003;35:405–406.
  10. Dempsey EM, Sant’Anna GM, Williams RL, et al: Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: not uniformly fatal. Pediatr Pulmonol 2005;40:270–274.