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Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature

Introduction. Primary congenital lymphedema is a rare disorder associated with insufficient development of lymphatic vessels. Usually most patients present with lower extremity edema seen sonographically. Rarely primary congenital lymphedema may be associated with severe lymphatic dysfunction result...

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Detalles Bibliográficos
Autores principales: Singh, Paul, Connell, Matthew
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3600248/
https://www.ncbi.nlm.nih.gov/pubmed/23533860
http://dx.doi.org/10.1155/2013/186173
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author Singh, Paul
Connell, Matthew
author_facet Singh, Paul
Connell, Matthew
author_sort Singh, Paul
collection PubMed
description Introduction. Primary congenital lymphedema is a rare disorder associated with insufficient development of lymphatic vessels. Usually most patients present with lower extremity edema seen sonographically. Rarely primary congenital lymphedema may be associated with severe lymphatic dysfunction resulting in hydrops fetalis. Case. A 27-year-old primigravida with a family history of leg swelling throughout multiple generations was diagnosed early in the third trimester with hydrops fetalis. Delivery was undertaken at 32 weeks for nonreassuring fetal status and the infant expired at approximately 45 minutes of life. Primary congenital lymphedema was confirmed via molecular testing of the vascular endothelial growth factor receptor-3 gene. Discussion. The diagnosis of PCL is suspected prenatally when ultrasound findings coincide with a positive family history of chronic lower limb lymphedema. Isolated PCL is rarely associated with significant complications. Rarely, however, widespread lymphatic dysplasia may occur, possibly resulting in nonimmune hydrops fetalis.
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spelling pubmed-36002482013-03-26 Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature Singh, Paul Connell, Matthew Case Rep Obstet Gynecol Case Report Introduction. Primary congenital lymphedema is a rare disorder associated with insufficient development of lymphatic vessels. Usually most patients present with lower extremity edema seen sonographically. Rarely primary congenital lymphedema may be associated with severe lymphatic dysfunction resulting in hydrops fetalis. Case. A 27-year-old primigravida with a family history of leg swelling throughout multiple generations was diagnosed early in the third trimester with hydrops fetalis. Delivery was undertaken at 32 weeks for nonreassuring fetal status and the infant expired at approximately 45 minutes of life. Primary congenital lymphedema was confirmed via molecular testing of the vascular endothelial growth factor receptor-3 gene. Discussion. The diagnosis of PCL is suspected prenatally when ultrasound findings coincide with a positive family history of chronic lower limb lymphedema. Isolated PCL is rarely associated with significant complications. Rarely, however, widespread lymphatic dysplasia may occur, possibly resulting in nonimmune hydrops fetalis. Hindawi Publishing Corporation 2013 2013-02-28 /pmc/articles/PMC3600248/ /pubmed/23533860 http://dx.doi.org/10.1155/2013/186173 Text en Copyright © 2013 P. Singh and M. Connell. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Singh, Paul
Connell, Matthew
Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
title Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
title_full Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
title_fullStr Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
title_full_unstemmed Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
title_short Primary Congenital Lymphedema Complicated by Hydrops Fetalis: A Case Report and Review of the Literature
title_sort primary congenital lymphedema complicated by hydrops fetalis: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3600248/
https://www.ncbi.nlm.nih.gov/pubmed/23533860
http://dx.doi.org/10.1155/2013/186173
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